Neuronotes Clinical Pocket Guide

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Electrophysiologic Studies Auditory-Evoked Potential (AER) uses sounds in the ear to determine the response of the brainstem and auditory regions of the cortex to the sound. AER is useful in diagnosing abnormalities of hearing, damage to the acoustic nerve and acoustic neuromas. Brainstem Auditory-Evoked Response (BAER) see Auditory-evoked potential. Electroencephalography (EEG) utilizes electrodes on the scalp to measure the electrical activity of the brain. It is used to analyze general brain function and for the diagnosis of different forms of epilepsy and seizures and metabolic and degenerative disorders of the brain as well as sleep disorders. EEG is age specific; in the normal aging process, the person may demonstrate a modest degree of slowing in the temporal regions. Electronystagmography (ENG) is used evaluate some brain functions and to assess the vestibular system including involuntary eye movement, dizziness and balance disorders. Electromyography (EMG) records the electrical discharges of the muscle in response to nerve stimulation. EMGs can be performed with surface electrodes measuring voltage on the skin or needle electrodes inserted into the skin. EMG is used for testing muscle and nerve disor- ders including amyotrophic lateral sclerosis, myasthenia gravis, the muscular dystrophies, peripheral neuropathies, and Guillain Barré syndrome. It is useful in differentiate between demyelinating and axonal pathology. Evoked Potentials examine the visual, auditory, and somatosensory sys- tems to identify sub-clinical lesions. Evoked potentials are essential to diagnose multiple sclerosis, stroke, visual acuity in children, optic neu- ropathy, demyelinating diseases, leukodystrophies, and lipidoses. Nerve Conduction Velocity (NCV) measures the speed of electrical impulse conduction along sensory and motor nerves that are superficial enough to be stimulated. NCV is used in nerve disorders such as carpal tunnel syndrome, diabetic neuropathy, peripheral nerve lesions, and Charcot-Marie-Tooth disease. 1 INTRO

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Neuronotes Clinical Pocket Guide

Transcript of Neuronotes Clinical Pocket Guide

Page 1: Neuronotes Clinical Pocket Guide

Electrophysiologic Studies

Auditory-Evoked Potential (AER) uses sounds in the ear to determine the

response of the brainstem and auditory regions of the cortex to the

sound. AER is useful in diagnosing abnormalities of hearing, damage to

the acoustic nerve and acoustic neuromas.

Brainstem Auditory-Evoked Response (BAER) see Auditory-evoked

potential.

Electroencephalography (EEG) utilizes electrodes on the scalp to

measure the electrical activity of the brain. It is used to analyze general

brain function and for the diagnosis of different forms of epilepsy and

seizures and metabolic and degenerative disorders of the brain as well

as sleep disorders. EEG is age specific; in the normal aging process, the

person may demonstrate a modest degree of slowing in the temporal

regions.

Electronystagmography (ENG) is used evaluate some brain functions

and to assess the vestibular system including involuntary eye movement,

dizziness and balance disorders.

Electromyography (EMG) records the electrical discharges of the

muscle in response to nerve stimulation. EMGs can be performed with

surface electrodes measuring voltage on the skin or needle electrodes

inserted into the skin. EMG is used for testing muscle and nerve disor-

ders including amyotrophic lateral sclerosis, myasthenia gravis, the

muscular dystrophies, peripheral neuropathies, and Guillain Barré

syndrome. It is useful in differentiate between demyelinating and

axonal pathology.

Evoked Potentials examine the visual, auditory, and somatosensory sys-

tems to identify sub-clinical lesions. Evoked potentials are essential to

diagnose multiple sclerosis, stroke, visual acuity in children, optic neu-

ropathy, demyelinating diseases, leukodystrophies, and lipidoses.

Nerve Conduction Velocity (NCV) measures the speed of electrical

impulse conduction along sensory and motor nerves that are superficial

enough to be stimulated. NCV is used in nerve disorders such as carpal

tunnel syndrome, diabetic neuropathy, peripheral nerve lesions, and

Charcot-Marie-Tooth disease.

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INTRO

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INTRO

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Somatosensory-Evoked Potential (SEP) uses EEG electrodes to record

the response of the brain to a sensory stimulus. It is useful in determining

determine if there is a delay in conduction to the brain or nerve or spinal

cord damage or nerve degeneration from multiple sclerosis and other

degenerating diseases.

Visual-Evoked Potential (VEP) involves the use of EEG electrodes and

visual stimuli, such as flashing lights. VEP is use to determine the brain’s

response to the stimuli and is useful in diagnosing optic nerve damage.

Genetic Testing

Genetic tests involve molecular, biochemical and/or cytogenetic analysis

to determine if a condition is genetic or inherited. They can involve DNA,

RNA, chromosome, and protein analysis.

Imaging Studies

Angiography utilizes a radio-opaque liquid that is injected into an artery or

vein to detect blockages by aneurysm, occlusion or displacement of blood

vessels by tumors. It is also useful in detecting vascular malformation.

Computed Tomography (CT) is the preferred initial imaging procedure

for patients with stroke, other intracranial abnormalities, brain tumors

and damage from head injury. CT is usually performed first without, and

then with intravenous contrast enhancement (for example, iodine) to

show vascular abnormalities, hematoma, or enhancement of lesions. CT

of the face and sinuses are performed to evaluate patients with suspected

trauma or infection. In CT, an x-ray beam and a detector system move

around the patient in an arc of 360 degrees. The images are then recon-

structed by specialized software.

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CT of the Spine

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Computed Tomographic Angiography is less sensitive than conventional

angiography but can provide important information for patients with

acute stroke. CTA – circle of Willis

CTA of the Brain

Magnetic Resonance Imaging (MRI) is used to determine intracranial abnor-

malities, such as tumors and multiple sclerosis (because it is capable to

detect small changes in soft tissue). Tissue with more water (high hydrogen

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INTRO

4

density) shows high MRI signals and the image appears to be white. MRI

angiography is usually performed alone or after intravenous contrast

enhancement, is to evaluate the carotid, vertebral, and cerebral arteries.

MRI of the Brain

Functional MRI (fMRI) is based on the increase of blood flow to specific

brain structures responsible for certain neural/functional activities. fMRI

allows us to examine not only brain structures but also to map brain func-

tions. fMRI is used primarily in research.

Magnetic Resonance Angiography is less sensitive than conventional

angiography. MR angiogram is useful to examine the carotid arteries and

proximal portions of the intracranial circulation to screen for stenosis,

occlusion, or large atheromatous lesions.

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MRA of the Neck

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Positron Emission Tomography (PET) is used to evaluate dynamic brain

activities by evaluating positrons emitting by glucose isotopes. These

scans can detect tumors, measure cellular and/or tissue metabolism, and

show blood flow.

*** PET Normal and PET of Patient with Alzheimer’sDisease

Source: From National Institute of Aging.

Radiography is used mostly to identify trauma to the skull and the spine

and metastatic diseases; for example: vertebral fracture caused by

metastatic breast cancer or prostate cancer.

Single Photon Emission Computed Tomography (SPECT) uses Gamma

rays to examine brain structures and is often used in fMRI.

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INTRO

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Transverse Slice of Brain of Person with MultipleConcussions

Source: Accessed August 17, 2007, from, http://www.drrobertkohn.com/BrainSpect/TBI/nk.htm

8/17/07, with permission.

Ultrasonography (US) utilizes high-frequency sound waves to obtain

images and is used with infants to examine intracranial hemorrhage,

hydrocephalus, and other abnormalities. Neurosonography is used to

analyze blood flow in the brain and is useful in diagnosing stroke, brain

tumors and hydrocephalus. Transcranial Doppler ultrasound is used to

assess blood flow in arteries and blood vessels in the neck to determine

the risk of stroke.

Laboratory Based Exams

Cerebrospinal Fluid Examination is useful to determine CNS infection,

neoplastic invasion of the subarachnoid space, multiple sclerosis, menin-

gitis, acute inflammatory demyelinating polyneuropathy (Guillain-Barré

syndrome). CSF exam is performed through lumbar puncture to deter-

mine the CSF pressure and CSF contents (cell counts, biochemical and

immunologic studies, and microbiologic analysis)

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INTRO

GlossaryUnless otherwise noted (*), definitions are from Taber’s Dictionary;

Copyright 2005 by F. A. Davis Company.

Agnosia – Inability to recognize or comprehend sights, sounds, words,

or other sensory information.

■ Auditory agnosia – Inability to interpret sounds.

■ Tactile agnosia – Inability to distinguish objects by sense of touch.

■ Visual agnosia – Loss of the ability to visually recognize objects

presented, even though some degree of ability to see is intact.

Allodynia – The condition in which an ordinarily painless stimulus, once

perceived, is experienced as being painful.

Amnesia – A loss of memory. The term is often applied to episodes during

which patients forget recent events, although they may conduct them-

selves appropriately, and following which no memory of the period

persists. Such episodes often are caused by strokes, seizures, trauma,

senility, alcoholism, or intoxication.

■ Anterograde amnesia – Amnesia for events that occurred after a

precipitating event or medication.

■ Post-traumatic amnesia (PTA) – A state of agitation, confusion, and

memory loss that the patient with traumatic brain injury (TBI)

enters soon after the injury or on awakening from coma. Edema,

hemorrhage, contusions, shearing of axons, and metabolic distur-

bances impair the brain’s ability to process information accurately,

resulting in unusual behaviors that often are difficult to manage.

Post-traumatic amnesia can last for months but usually resolves

within a few weeks.

■ Retrograde amnesia – Amnesia for events that occurred before the

precipitating trauma.

Anomia – Inability to remember names of objects.

Anosognosia – The apparent denial or unawareness of one’s own neuro-

logical defect.

Aphasia – Absence or impairment of the ability to communicate through

speech, writing, or signs because of brain dysfunction. It is considered

complete or total when both sensory and motor areas are involved.

■ Broca’s or executive aphasia – Aphasia in which patients know

what they want to say but cannot say it; inability to coordinate the

muscles controlling speech. It may be complete or partial.

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■ Wernicke’s aphasia – An inability to comprehend the spoken or

written word. Visual and auditory pathways are unaffected; however,

patients are unable to differentiate between words or interpret their

meaning.

■ Global aphasia – Total aphasia involving failure of all forms of

communication.

Apraxia – Inability to perform purposive movements although there is

no sensory or motor impairment. Inability to use objects properly.

■ Constructional apraxia – Inability to draw or construct two- or

three-dimensional forms or figures and impairment in the ability to

integrate perception into kinesthetic images.

■ Ideation apraxia – Misuse of objects due to inability to perceive

their correct use. SYN: sensory apraxia.

■ Motor apraxia – Inability to perform movements necessary to use

objects properly, although the names and purposes of the objects

are known and understood.

■ Verbal apraxia – The inability to form words or speak, despite the

ability to use oral and facial muscles to make sounds.

Arteriovenous malformation – Congenitally abnormal tangle of blood

vessels, which can produce seizures, neurological deficits and

headache.*

Astereognosis – Inability to recognize the form of an object or forms

by touch.

Ataxia – Defective muscular coordination, especially that manifested

when voluntary muscular movements are attempted.

Atherosclerosis – The most common form of arteriosclerosis, marked by

cholesterol-lipid-calcium deposits in the walls of arteries.

Autonomic dysreflexia – The state in which an individual with a spinal

cord injury at T–7 or above, experiences a life-threatening uninhibited

sympathetic response of the nervous system to a noxious stimulus.

Symptoms include sudden hypertension, bradycardia, sweating,

severe headache, and gooseflesh.

Axonotmesis – Nerve injury that damages the nerve tissue without actu-

ally severing the nerve.

Coma – A state of unconsciousness from which one cannot be aroused.

Coma is the most severe of the alterations of consciousness. It differs

from sleep in that comatose patients will not awaken with stimulation; it

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INTRO

differs from lethargy, drowsiness, or stupor (states in which patients are

slow to respond) in that comatose patients are completely unresponsive.

Confabulation – A behavioral reaction to memory loss in which the

patient fills in memory gaps with inappropriate words or fabricated

ideas, often in great detail.

Diadochokinesia – The ability to make antagonistic movements, such as

pronation and supination of the hands, in quick succession.

Dysesthesia – Uncomfortable, abnormal sensations, such as pins and

needles, burning, or crawling feelings that can occur spontaneously or

from external stimuli.*

Dysarthria – Impairments or clumsiness in the uttering of words due to

diseases that affect the oral, lingual, or pharyngeal muscles. The

patient’s speech may be difficult to understand, but there is no

evidence of aphasia.

Dyscalculia – An inability to make calculations. It may be found in child-

hood as a learning disability or may result from a stroke.

Dysgraphia – A persistent deficit in handwriting, usually the result of

developmental diseases (in children), and of brain injury, dementia, or

stroke (in adults).

Dysphagia – Inability to swallow or difficulty in swallowing.

Dyspraxia – A disturbance in the programming, control, and execution

of volitional movements. It cannot be explained by absence of com-

prehension, inadequate attention, or lack of cooperation; it is usually

associated with a stroke, head injury, or any condition affecting the

cerebral hemispheres.

Executive functions – The cognitive processes involving logic, planning,

analysis, and reasoning. These capacities enable us to solve problems

encountered in daily life that require considerations of goals, contexts,

options, and previous experiences to select an appropriate strategy.

Hemianopia, hemianopsia – Blindness in one-half of the visual field.

Intrathecal therapy – Injection of medications into the cerebral spinal

fluid via a lumbar puncture.*

Memory – The mental registration, retention, and recollection of past

experiences, sensations, or thoughts. This group of functions relies

on the coordinated activities of the association regions of the cerebral

cortex, specific sensory areas of the brain, subcortical centers, the

hypothalamus, the midbrain, and a wide array of neurochemicals and

neurotransmitters. Injury or damage to any of these regions of the

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brain (e.g., as a result of intoxication, stroke, atrophy, or infection)

impairs the ability to incorporate new memories or recall and use

prior ones.

■ Declarative – The conscious recollection of learned information—a

memory function that is improved by the association of learning

with highly charged emotional experiences.

■ Long-term – Recall of experiences, or of information gained, in the

distant past.

■ Procedural – The memory capability that permits an individual to

perform activities. This type of memory is usually preserved when

other memory functions are lost.

■ Short-term, immediate – Memory for events or information in the

immediate past. Brain damage that limits one’s ability to store new

information may impair immediate memory but have no effect on

memories of the distant past.

Muscle tone – The state of slight contraction usually present in muscles

that contributes to posture and coordination; the ability of a muscle to

resist a force for a considerable period without change in length.

Neurapraxia, neuropraxia – A temporary impairment in nerve conduc-

tion, typically caused by an injury that does not produce permanent

structural damage to the nerve.

Neurotmesis – Nerve injury with complete loss of function of the nerve

even though there is little apparent anatomic damage.

Oscillopsia – The visual perception that stationary objects are moving.

This perception is an illusion, and is usually associated with vestibular

dysfunction.

Paresthesia – An abnormal or unpleasant sensation that results from

injury to one or more nerves, often described by patients as numb-

ness or as a prickly, stinging, or burning feeling.

Perseveration – Abnormal, compulsive, and inappropriate repetition of

words or behaviors, a symptom observed, for example, in patients with

schizophrenia or diseases of the frontal lobes of the brain. The repeti-

tion of rhythmic but meaningless actions, behaviors, or movements.

Phonophobia – A morbid fear of sound or noise. A fear of speaking or

hearing one’s own voice.

Photophobia – Unusual intolerance of light, occurring in measles,

rubella, meningitis, and inflammation of the eyes.

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INTRO

Reflex sympathetic dystrophy – An abnormal response of the nerves

of the face or an extremity, marked by pain, autonomic dysfunction,

vasomotor instability, and tissue swelling. Although the precise

cause of the syndrome is unknown, it often follows trauma, stroke,

neuropathy, or radiculopathy. In about one third of all patients, the

onset is insidious. Affected patients often complain of burning pain

with any movement of an affected body part, excessive sensitivity to

light touch or minor stimulation, temperature changes (heat or cold)

in the affected limb, localized sweating, localized changes of skin

color, or atrophic changes in the skin, nails, or musculature.

Vertigo – The sensation of moving around in space (subjective vertigo)

or of having objects move about the person (objective vertigo).

Vertigo is sometimes inaccurately used as a synonym for dizziness,

lightheadedness, or giddiness.

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Medical Yellow Flags

Precautions: Yellow Flags indicate a medical precaution that warrants a

referral to an appropriate medical professional.

■ Bilateral edema

■ Chronic or persistent cough

■ Development of pain, sensory loss, weakness, paralysis, or sphincter

or sexual dysfunction (may be due to spinal cord compression)

■ Fever of unknown etiology

■ Pain on weight-bearing

■ Seizure

■ Shortness of breath at rest or with minimal exertion

■ Wheezing

Physical Therapy Examination for Patients withNeuromuscular Disorders

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Anthropometric Characteristics

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PT EXAM

24

52

20

in cm

Birth

Birth to 36 months: BoysHead circumference-for-age andWeight-for-length percentiles

WEIGHT

WEIGHT

HEAD

CIRCUMFERENCE

HEAD

CIRCUMFERENCE

3 6 9 12 15 18

AGE (MONTHS)

21 24 27 30 33 36

cm in

19

18

17

20

979075502510

3

97

9075502510

3

19

18

17

16

15

14

13

12

50

48

46

44

42

52

50

48

46

44

42

225048464442403836343230

282624222018161412

21

20

19

18

17

16

15

14

13

12

11

10

9

8

7

6

5

Date Age Weight Length Head Circ. Comment

64

26

50 60 62585654524846

18 19 20 21 22 23 24

27 28 29 30 31 32 33 34 35 36 37 38 39 40 41

68 70 72 74 76 78 80 82 84 86 88 90 92 94 96 98100

40

38

36

34

32

30

22201816141210

8642lb kg

cm

cm

in

in

lbkgLENGTH

11

10

9

8

7

6

5

4

3

21

NAME

RECORD #

66

Source: Available at: http://www.cdc.gov/nchs/data/nhanes/growthcharts/set1clinical/

cj41c017.pdf. Accessed October 1, 2007.

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WEIGHT

WEIGHT

STATURE

STATURE

incm

in cm

2 to 20 years: BoysStature-for-age and Weight-for-agepercentiles

12

2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20

13 14 15 16 17 18 19 20

AGE (YEARS)

AGE (YEARS)

97 190

160

155

150

145

140

135

130

125

120

115

110

105

100

95

90

85

80

35

30

25

20

15

10

185

180

175

170

165

160

155

150

105

100

95

90

85

80

75

70

65

60

55

50

45

40

35

30

25

20

15

10

Date

*To Calaculate BMI: Weight (kg) ÷ Stature (cm) ÷ Stature (cm) x 10,000or Weight (lb) ÷ Stature (in) ÷ Stature (in) x 703

Mother’s Stature Father’s StatureAge Weight Stature BMI*

lblb kgkg

NAME

RECORD #

3030

30

32

34

36

38

40

42

44

46

48

50

52

54

56

58

60

62

3 4 5 6 7 8 9 10 11

40

50

60

70

80

40

50

60

70

80

90

60

62

64

66

68

70

72

74

76

100

110

120

130

140

150

160

170

180

190

200

210

220

230

90

75

50

25

10

3

97

90

75

50

25

10

3

Source: Available at: http://www.cdc.gov/nchs/data/nhanes/growthcharts/set2clinical/

cj41c071.pdf. Accessed October 1, 2007.

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Assistive and Adaptive Devices

Wheelchair (WC) Checklist

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PT EXAM

Source: Adapted from O’Sullivan SB, Schmitz TJ. (2007). Physical Rehabilitation. 5th ed.

Philadelphia: F.A. Davis; 2007, page 1300, Figure 33.16 Foundation components of a prescriptive

wheelchair, with permission.

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Fittings/Category Components Recommendations Findings

Seat Width • Provide 1 in. of space to each

side of widest portion of thigh

& hip

Depth • Subtract 1 in. from distance

between posterior aspect of

buttock & popliteal fossa (for

adults)

Height • Provide 2 in. of space separating

(measure footplates from floor

with cushion • Allow patient to keep trunk

in place) erect & rest sound foot flat on

floor to facilitate propulsion

(in hemi-WC)

Cushion • Allow pressure relief for ischial

tuberosities

Frame Angle • Adjust according to patient’s

sitting tolerance

• Set at 90° for patients with

paraplegia

• Tilt backward for patients with

high level tetraplegia

Backrest Height • Adjust according to patient’s

(measure trunk stabilization ability

with cushion • Adjust top to below scapula’s

in place) inferior angle for patients with

tetraplegia

• Adjust lower for patients with

paraplegia

Straps Pelvic belts • Install for safety reasons

• Install additional trunk strap

for patients with limited trunk

stability

Leg rests Length • Have patient keep hips & knees

at 90° with foot in neutral &

adjust until a 2 in. space

separates floor from footplates

Types • Install swing-away leg rests to

ease transfer

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PT EXAM

Fittings/Category Components Recommendations Findings

Heel loops Types • Install heel loops & calf straps or

& calf straps pads for patients with severe

or pads spasticity

Armrests Height • Have patient hold trunk in

(measure comfortable sitting position with

with cushion arms at sides & elbows at 90° &

in place) adjust accordingly

Types • Install detachable or flip-up

armrests to ease transfer

Brakes Types • Install brake extensions for

extremity with limited upper

extremity strength or motion

Wheels Alignment • Install parallel wheels for standard

or lightweight adult WCs

• Install angled wheels for sports WCs

Tires Pressure • If WC has pneumatic tires, assess

pressure for leakage

Power WC Control • Install & position control mecha-

mechanism nism for easy control & access

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Muscle Performance (Including Strength, Power, and Endurance)

Apgar Scores

Sign Score � 0 Score � 1 Score � 2Heart rate Absent Below 100 per Above 100 per minute

minute

Respiratory effort Absent Weak, irregular, Good, crying

or gasping

Muscle tone Flaccid Some flexion Well flexed or

of arms and active movements of

legs extremities

Reflex/irritability No response Grimace or Good cry

weak cry

Color Blue all over, Body pink, hands Pink all over

or pale and feet blue

Total Score

Scores are taken at 1 minute, 5 minutes and also may be taken at 10 and 20 minutes post birth

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Pediatric Disorders

Cerebral Palsy (CP)

Description/Overview

The motor disorders of cerebral palsy are often accompanied by distur-

bances of sensation, cognition, communication, perception, and/or

behavior, and/or a seizure disorder.” While considered non-

progressive, the clinical expression of CP can change with maturation of

the brain, growth of the body, and increasing demands for mobility. Early

symptoms include developmental delay, lack of integration of primitive

reflexes, hyperreflexia and abnormal muscle tone.

■ Spasticity involves velocity-dependent resistance to passive

movement that results in increased muscle tone; movements

occur synergistically

■ Athetosis involves fluctuating muscle tone, involuntary and uncon-

trolled movements, and postural instability; its onset is usually

preceded by a period of hypotonia in infants

■ Ataxia is an infrequent finding in CP in which there is diminished

control of coordination and balance

■ Hypotonia involves a decreased ability to generate sufficient muscle

force for normal movement patterns

Special Tests

■ Radiograph used for assessment of scoliosis, bony deformities,

subluxed/dislocated joints, especially of the hips

■ Ultrasound used to detect hemorrhage or hypoxic-ischemic injury in

the neonatal period

■ Three-dimensional gait analysis used for baseline measure and prior

to introduction of new medication, Botox injection, and surgery.

■ Magnetic resonance imaging (MRI) used to detect congenital

malformations, intracranial hemorrhages, and periventricular

leukomalacia

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PEDS

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Tests and Measures

Work, Community, and Leisure Assessment

■ Pediatric Evaluation of Disability Index (PEDI)

■ Functional Independence Measure For Children (WeeFIM)

CognitionIndividuals with CP have a higher incidence of cognitive impairment and

learning disabilities than the non-disabled population. However, many

individuals with CP often have age-appropriate intelligence that may be

masked by difficulties in communication and motor control.

Differential Diagnosis

■ Genetic, metabolic, muscular, or neuronal tumor disorders that result

in abnormal tone

■ Rett syndrome, found primarily in girls

■ Progressive cerebellar degenerative disorders

■ Lesch-Nyhan syndrome, found in males, features self-mutilation and

hyperuricemia

■ Infantile spinal muscular atrophy, associated with hypotonia and

hyporeflexia

■ Tuberous sclerosis

Surgery

Common surgical procedures associated with CP include:

■ Dorsal rhizotomy to minimize spasticity

■ Obturator neurectomy to decrease the spastic influence of the hip

abductors

■ Soft-tissue releases/transfers to decrease the influence of spastic

muscles and improve gait

■ Major reconstructive femoral and/or pelvic osteotomy for prevention

and correction of hip subluxation and dislocation

■ Proximal femoral varus-producing osteotomy in combination with

appropriate soft tissue releases for treatment of subluxing hips

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■ Tibial derotation osteotomy

■ Achilles tendon, hip adductor, and hamstring lengthening

Prognosis

Maintaining independent sitting by the age of 2 years is a good prognos-

tic indicator of an eventual ability to ambulate. Individuals with CP gener-

ally have a life expectancy into adulthood. Issues limiting life span include

severe problems with sucking, swallowing, and feeding. Morbidity and

mortality are higher than for non-CP and are complicated by respiratory

and cardiac difficulties, cerebrovascular accident, and cancer.

Referral to Other Health-Care Providers

■ Speech-language pathologist

■ Audiologist for nerve deafness that may occurs in individuals with

athetoid CP

■ Ophthalmologist for visual problems such as strabismus, decreased

visual acuity secondary to retinopathy of prematurity, and visual field

abnormalities

■ Neurosurgery for consideration of an intrathecal baclofen pump,

stereotactic basal ganglia surgery to improve rigidity, choreoathetosis,

and tremor

■ Orthopedist for surgery for muscle and tendon release and transfer,

and correction of bony abnormalities

■ Nutritionist when feeding problems exist

■ Mental health workers for issues involved with disability and depression

Resources

Easter Seals www.easterseals.com

United Cerebral Palsy (UCP)/UCP Research & Educational Foundationwww.ucp.org

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PEDS

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Developmental Coordination Disorder

Description/Overview

DCD is associated with speech delays, visual-perceptual, visual-spatial and

visual-motor impairments and can interfere with academic achievement and

activities of daily living. It is estimated that 6% of the U.S. children between

the ages of 5 and 11 have DCD.

Tests and Measures

Arousal, Attention, and CognitionPotential findings

■ Individuals with DCD generally have age-appropriate intelligence

but poor academic achievement due to impairments that limit their

coordination

Differential Diagnosis

■ Autism

■ Attention-deficit hyperactivity disorder

■ Developmental motor delay

■ Mild cerebral palsy

■ Pervasive developmental disorder

Prognosis

DCD can continue into adulthood. Individuals can learn strategies to

develop skills they need for ADLs and other tasks. However, they often

continue to lack the abilities of age-matched peers.

Referral to Other Health-Care Providers

■ Occupational therapist for fine motor and visual-perceptual issues

■ Speech-language pathologist

■ Ophthalmologist if visual problems detected or suspected

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■ Mental health workers as DCD can significantly impact social and

emotional well-being and result in low self-esteem, diminished

self-image, and depression

Resources

Can Child Centre for Childhood Disability Research

Epilepsy

Description/Overview

In epilepsy, the normal pattern of neuronal activity becomes disturbed,

causing strange sensations, emotions, and behavior or sometimes con-

vulsions, muscle spasms, and loss of consciousness.”

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Other Seizure Activity Requiring Immediate MedicalAttention

Symptoms/conditions Management• The first occurrence of a seizure • Seek immediate medical attention

• Seizure occurring after an injury or • Follow safety procedures for

illness seizures (see following)

• During pregnancy

• With a diagnosis of diabetes

For individuals with a seizure history:

• Recovery from the seizure is slower

than usual

• Change in frequency and severity

• Seizure is preceded by a severe

headache or neurological

symptoms such as numbness,

weakness, speech disturbances, etc.

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Special Tests

■ Electroencephalogram (EEG) and 24-hour EEG monitoring to deter-

mine seizure pattern and areas in which the seizure originates

■ Magnetic resonance imaging (MRI) to determine the source of

seizures

■ Positive emission tomography (PET) used during epilepsy surgery to

determine how areas of the brain metabolize glucose

■ Single photon emission computed tomography (SPECT) to localize

the area of the brain responsible for seizures

■ Wada test (also known as Intracarotid Amobarbital Procedure) is

used before epilepsy surgery and determines which side of the brain

controls language and memory function

Surgery

■ Resective surgery involves removal of the section of the brain respon-

sible for the seizure.

■ Corpus callosotomy prevents a focal event to cross over and become

a generalized seizure and is most effective for generalized atonic

seizures

■ Hemispherectomy is used for patients with congenital syndromes

that result in severe uncontrollable symptoms.

■ Vagus nerve stimulation is used to limit the severity of seizures.

Prognosis

Life expectancy is minimally reduced for individuals with idiopathic

epilepsy. However, symptomatic epilepsy (having an identifiable cause)

has a negative impact on life expectancy which can be shortened by

several years.

Resources

Epilepsy Foundation http://www.epilepsyfoundation.org/

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Hydrocephalus

Description/Overview

Hydrocephalus is a pathologic accumulation of cerebral spinal fluid (CSF)

resulting from an imbalance between the formation of CSF and its

absorption. Common causes include excessive secretion of CSF; block-

age of CSF flow within the ventricles (non-communicating hydro-

cephalus); obstruction of CSF distal to the fourth ventricle foramina, in

the cisterns or the cerebral subarachnoid space itself (communicating

hydrocephalus); tumor; and traumatic brain injury. Hydrocephalus is a

common occurrence in Chiari and Dandy-Walker malformations and in

meningocele and myelomeningocele.

Special Tests

■ CT scan – allows prenatal diagnosis, treated with intrauterine

shunting.

■ MRI, including fetal MRI

■ Ultrasonography

■ Lumbar puncture

■ Intracranial pressure monitoring

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Setting Sun SignHydrocephalus

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Tests and Measures

Communication Ability (Affect, Cognition, Language,Learning)“Cocktail party syndrome” is associated with hydrocephalus. It involves

being verbose and engaging in superficial social language that may lack

depth and not be appropriate to the situation.

Surgery

Hydrocephalus is treated by:

■ Extracranial shunts including ventriculoperitoneal (most common),

ventriculoatrial, and ventriculopleural types are used to divert the

CSF to a compartment of the body that can absorb the fluid

■ Surgical correction of CSF obstruction, such as a tumor or cyst,

ventricular bypass via shunt to an extracranial compartment

Prognosis

The prognosis varies and is dependent on many factors including the

timeliness of treatment/surgery and associated disorders that accompany

hydrocephalus, including head injury, infection, and SB. Because of

the likeliness of comorbidities, hydrocephalus is often associated with

physical, perceptual, and cognitive deficits.

Referral to Other Health-Care Providers

■ Ophthalmologist as hydrocephalus may result in decreased visual

acuity due to pressure on the optic nerve

■ Speech-language pathologist if there are issues with sucking, feeding

and swallowing

Resources

Hydrocephalus Association www.hydroassoc.org

National Hydrocephalus Foundation http://nhfonline.org

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Muscular Dystrophy (MD)

Description/Overview

Duchenne MD is the most common form, affecting primarily boys and

results from an absence of dystrophin, whereas Becker MD is caused by

insufficient dystrophin.

Special Tests

■ Blood tests for the presence of creatine kinase (suggests muscle

disease)

■ Electromyography

■ Ultrasonography can detect certain muscle abnormalities

■ Muscle biopsy to detect markers associated with specific types of MD

and the presence of dystrophin

■ Genetic testing to detect gene mutations that produce dystrophin

Tests and Measures

Neuromotor Development and Sensory Integration (Refer to Tab 2)Potential findings

■ DMD—difficulty rising to stand from sitting or lying and with stair

climbing; frequent falls

Work, Community, and Leisure (See Tab 2)Assessment

■ Pediatric Evaluation of Disability Index

■ Functional Independence Measure For Children (WeeFIM)

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Disease-Specific Tests and Measures

Surgery

■ Achilles tendon lengthening

■ Spinal stabilization for scoliosis and other spinal deformities

Differential Diagnosis

■ Must distinguish among the types of MD

■ Kugelberg-Welander spinal muscular atrophy

■ Werdnig-Hoffmann spinal muscular atrophy

Prognosis

Refer to earlier table for life expectancy for each variation of MD; death is

usually associated with respiratory or cardiac complications

Referral to Other Health-Care Providers

■ Speech-language pathologist for assessment of language and dysphagia

■ Occupational therapy for ADLs and environmental adaptations

■ Mental health workers for issues involved with disability, decreased

function and independence, and depression

Medications

Indications Generic Name Brand Name Common Side EffectsWeight loss; oxandrolone Oxandrin Insomnia, depression,

side effects anxiety

caused by

corticosteroids

Source: Vignos PJ, Spencer, GE, Archibald, KC. Management of progressive muscular dystrophy.

JAMA. 2963;184:103–112.

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Resources

Muscular Dystrophy Association [email protected]

Neonatal Neurological Conditions

Hypoxic Ischemic Encephalopathy (HIE)

Considered the most common cause of severe, non-progressive neuro-

logic defect caused by perinatal events, it can result from respiratory or

cardiac insufficiency. Hypoxic-ischemic events can also be caused by

emboli, thrombosis, or clot formation.

HIE is diagnosed based on umbilical arterial blood pH, the persistence

of an Apgar score of less than 3 at 5 minutes, neurological signs includ-

ing seizures and hypotonia and involvement of several organs, including

but not limited to, the lungs, heart, kidneys, and liver.

Severe HIE is characterized by the need for ventilatory support, flaccid-

ity, and absent reflexes. Respiratory and cardiac functions may be

depressed and can fail.

Low Birth Weight

Infants with low birth weights often develop periventricular leukomalacia,

intraventricular hemorrhage, and respiratory problems, including respiratory

distress syndrome. These complications worsen for those in the VLBW and

ELBW categories and they have a greater chance of developing hypother-

mia, perinatal asphyxia, hyperbilirubinemia, infection, and other medical

complications.

Prematurity

Infants born earlier than 37 weeks’ gestation are considered premature.

Special Tests

■ Electroencephalogram for diagnosing neonatal seizures

■ Somatosensory evoked potentials (SSEPs)

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■ Visual evoked potentials (VEPs)

■ MRI is the imaging modality of choice for HEI and PVL

■ Ultrasonography used to diagnosis IVH and PVL

■ Near-infrared spectroscopy for monitoring CNS ischemic events.

Physical Therapy Examination - Tests and Measures

Reflex IntegrityReflex Integrity is often accomplished as part of assessment tools listed in

section “Neuromotor Development and Sensory Integration.”

Assessment

■ Deep tendon reflexes

■ Postural responses, including righting, equilibrium, and protective

reactions

■ Primitive reflexes

■ Muscle tone

Prognosis

Hypoxic Ischemic Encephalopathy is associated with significantly high

rates of mortality and morbidity. HIE often results in CP, especially when

abnormal muscle tone persists past the first week of life. Ongoing seizures

and cognitive impairment are common neurological sequelae. HIE can

also result in visual impairment, hearing impairment, and chronic lung

disease.

Intraventricular Hemorrhage: Outcome is dependent on the severity of

hemorrhage. IVH grades of I and II are associated with minimal risk of

sustaining long-term disability. However, grades III and IV represent a

worsening prognosis that is more significant when accompanied by other

medical complications. Grades III and IV are associated with a high risk of

developing mental retardation, hydrocephalus, seizures, and CP.

Low Birth Weight: Infants who have a very low birth weight or extremely

low birth weight are at high risk for infant mortality and morbidity.

Neurological consequences include CP, learning disorders, and cognitive

impairment. Other complications related to VLBW and ELBW include sen-

sorineural hearing loss, seizures, and attention-deficit disorder.

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Periventricular Leukomalacia represents an increased risk for CP, cogni-

tive impairment, coordination difficulties, and vision and hearing impair-

ments. The prognosis is dependent on the severity of brain damage and

can range from very mild symptoms to significant disability.

Prematurity: The prognosis of premature infants varies and is linked to

accompanying medical complications. While some very premature

infants have no long-term complications others have neurological seque-

lae including CP, learning disabilities, attention-deficit hyperactivity disor-

der, sensorineural hearing loss, feeding difficulties, and retinopathy of

prematurity.

Referral to Other Health-Care Providers

■ Speech language pathologist for language delays and feeding

difficulties

■ Early intervention/speech education professional for cognitive delays,

attention deficit/hyperactivity disorder and learning disability.

■ Ophthalmologist for suspected vision impairments

■ Audiologist for hearing difficulties

Resources

American Association of Premature Infants www.aapi-online.org

Obstetrical Brachial Plexus Palsy

Description/Overview

Injury can result from neurapraxia, neurotmesis, and axonotmesis. It can

also be caused by hemorrhage and edema that compress the nerves in

the plexus, and pressure on the nerve from neuromas that develop

during the healing process.

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Special Tests

■ EMG and fluoroscopy to determine diaphragmatic function

■ High-resolution MRI

■ Radiographs to assess possible fractured clavicle and humerus

Tests and Measures

Anthropometric Characteristics Assessment

■ Girth measurements

■ Limb length

Assistive and Adaptive DevicesConsiderations

■ Assistive and positioning devices, splints, etc., should be appropriate

for child’s age; some positioning can be done with stuffed animals,

infant pillows, etc.

Work, Community, and Leisure Assessment (Refer to Tab 2)

■ Pediatric Evaluation of Disability Index

■ Functional Independence Measure For Children (WeeFIM )

Differential Diagnosis

■ Fractures of the clavicle and humerus

■ Torticollis

■ Facial nerve palsy

Surgery

In infancy:

■ Excision of neuromas or hematomas

■ Neurolysis to remove scar tissue that develops around the injured

nerve

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■ Repair by nerve graft

In older child:

■ Tendon transfers for improved flexibility, function and cosmesis;

common procedures include transfer of:

■ Latissimus dorsi and/or teres major to the posterior aspect of the

upper humerus for improved external rotation and abduction

■ Triceps to biceps

■ Contralateral trapezium and/or rhomboids for stabilization of the

affected scapula

■ Soft tissue releases of contractures

■ Osteotomy for external rotation of the humerus

Prognosis

Prognosis is dependent on the site, nature, and severity of the injury. The

prognosis is best when significant recovery occurs in the first few months

of age. If there is a lack of recovery within the first few weeks, long-term

disability is likely to occur. Prognosis based on the type of injury is:

■ Avulsion injuries Permanent injury unless surgically repaired

■ Erb’s and Klumpke’s Spontaneous recovery in 70% to 80% of

palsy cases

■ Total plexus palsy Spontaneous recovery rate – less than 50%.

Resources

Brachial Plexus Palsy Foundation www.brachialplexuspalsyfoundation.org

United Brachial Plexus Network www.ubpn.org

Rett Syndrome

PrecautionsThere is an increased risk of osteoporosis associated with RS.

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Special Tests

■ Genetic testing—positive MECP2 mutation gene analysis indicative

of RS

■ EEG to identify seizure activity

■ Swallowing studies to determine risk of aspiration

■ Respiratory studies of disorganized breathing patterns, clinical

cyanosis, sleep apnea

Tests and Measures

Communication Ability (Affect, Cognition, Language,Learning) Speech apraxia is present in RS but receptive speech may remain.

Patients may attempt to communicate with body language and

expression. Communication may be possible through the use of eye gaze

systems, picture boards, and voice output systems.

Differential Diagnosis

■ Angelman’s syndrome—but no loss of hand function

■ Spinocerebellar degenerations—but no loss of hand function

■ Benign congenital hypotonia

■ Cerebral palsy

■ Autism

Surgery

■ Vagal nerve stimulation has been successful in increasing alertness

and social functioning

■ Gastrostomy is used if there are significant feeding problems or aspi-

ration pneumonia

■ Surgical correction of scoliosis should be considered when curves are

greater than 40 to 45 degrees

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Prognosis

Patients often stabilize by the second decade when seizures may lessen

and an increase in hand function and social interaction may occur. Gait

may improve in some cases. Active PT and OT are important in preventing

contractures and maintaining strength and function. Patients with RS may

live into their fifth or sixth decades.

Referral to Other Health-Care Providers

■ Speech-language pathologist for assessment of oral motor status and

communication

■ Psychologist for IQ and autism testing

■ Dietician as a high calorie diet is associated with improved weight

gain and seizure control

■ Assistive technology specialist for eye gaze system or voice output

communication

Resources

International Rett Syndrome Association www.rettsyndrome.org

Rett Syndrome Research Foundation www.rsrf.org

Shaken Baby Syndrome

Description/Overview

Due to the weakness of an infant’s neck muscles and the size of the head,

shaking results in multiple forces of the fragile brain against the skull. This

causes direct trauma to the brain resulting in a classic triad of symptoms

that include brain swelling, subdural hematoma, and retinal hemorrhage.

Fractures of the long bones and ribs may also occur.

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Special Tests

■ CT scan is used if SBS suspected and can demonstrate subarachnoid

hemorrhage and diffuse hypoxic-ischemic changes

■ Magnetic resonance imaging is used to demonstrate changes in the

brain tissue

■ Radiographs are used for diagnosing skull fractures and other fractures

Surgery

Surgery may be indicated to stop bleeding in the brain or to relieve pres-

sure from bleeding.

Differential Diagnosis

■ SBS syndrome results in serious brain damage that is not caused by

an accidental fall, bouncing, baby swings, etc.

■ Initially SBS may appear to be related to the flu or meningitis

Prognosis

SBS is the most common cause of mortality from child abuse occurring in

infants. Resultant disability ranges from mild learning disability to pro-

found mental retardation and permanent vegetative state.

Referral

■ In all states, therapists are mandated reporters, who are required by law

to report suspected child abuse or maltreatment. Every state has a Child

Abuse Hotline. The National Child Abuse Hotline is 1-800-4-A-CHILD.

Resources

Think First Foundation National Injury Prevention Programhttp://www.thinkfirst.org

National Center on Shaken Baby Syndrome E-mail: [email protected]

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Spina Bifida (SB)

Description/Overview

■ Spina bifida occulta: often accompanied by neurocutaneous stigmata

such as a dimpling, small hole, a lipoma or tuft of hair on the back, it

is usually asymptomatic

■ Myelomeningocele: there may not be a clear delineation of involve-

ment associated with a particular spinal level; orthopedic deformities

are common due to bony malformations, residual paralysis, weak-

ness, and the uneven muscle activity around affected joints

■ Tethered Cord Syndrome: can result from spina bifida occulta or scar-

ring after surgical repair of SB; complications arise during periods of

growth when blood vessels stretch, resulting in decreased blood flow

to the spinal cord; the cord may stretch abnormally and resultant ten-

sion can cause permanent damage to the nerves; Hydrocephalus

occurs in more than 90% of those with lumbrosacral myelomeningo-

cele. Chiari malformation is a common occurrence in myelomeningo-

cele and involves structural defects in which medulla, pons, fourth

ventricle and lower portion of the cerebellum herniate into the upper

cervical canal. The flow of the CSF is blocked resulting in symptoms

that include dizziness, numbness, visual defects, headache and dimin-

ished balance and coordination. There may be complications with

respiration, swallowing, gagging and aspiration.

Medical Yellow Flags

Dislocation of the Hips

Symptoms Possible Causes Management• Decreased passive range • Muscle imbalance Referral to physician

of motion, typically limiting around the joint

hip adduction and internal • Joint contractures

rotation

• Leg length discrepancy

• Pain

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Special Tests

■ Urinalysis, urine culture, and serum urea nitrogen creatinine test are

assessed at birth to evaluate renal function and for signs and symp-

toms of urinary tract infection

■ Voiding cystogram is used to assess the lower urinary tract

■ X-Rays are used for determination of the level of lesion; spinal

deformities, deformities of the foot, ankle, and other joints, and

subluxation/dislocation of hips

■ EMG and nerve conduction velocities may be done in the LEs of

infants with SB

■ Myelogram to detect a tethered cord or spinal cord abnormality

■ Neuro-imaging

■ CT scans for hydrocephalus

■ MRI of the brain and spine to determine the level and extent of the

SB and to detect any abnormalities of the spinal cord and column

Tests and Measures

Anthropometric CharacteristicsConsideration

■ Obesity is a common problem in SB

Assessment

■ Limb length and circumference

Worsening of Neurological Signs

Symptoms Possible Causes Management• A decrease in muscle • Tethered cord Referral to physician

function • Malfunctioning

• Decreasing bowel and shunt

bladder function

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Arousal, Attention, and CognitionConsiderations

■ The majority of individuals with myelomeningocele have intellectual

scores within age-appropriate ranges; lower intellectual abilities

and cognitive impairments are associated with higher-level lesions

(thoracic and cervical), hydrocephalus, and CNS infections

Assessment Refer to “Neuromotor Development and Sensory Integration”

Bowel and Bladder Control Potential findings

■ Anorectal sensation is usually absent

■ Kidney infections are common because of retrograde flow of urine

and difficulties emptying the bladder

Integumentary Integrity■ Orthotics should be modified if their use results in redness that per-

sists for more than 20 minutes

■ Instruction in patient/family assessment and reporting is important

due to sensory deficits

Muscle PerformanceConsiderations

■ It is necessary to determine the motor lesion level as it is a useful

predictor of potential contractures and deformities as functional

abilities that may be attained; lesions may resemble complete cord

transection or may be incomplete, with mixed representation of

muscle use, spasticity, and paralysis in muscles below the lesion

Assessment

■ MMT should be performed if appropriate considering age and

cognition

■ Dynamometry can be used for muscle force and grip and pinch

strength

Neuromotor Development and Sensory IntegrationConsiderations

■ Testing should be done with and without adaptive/assistive equip-

ment and orthoses

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Orthotic, Protective, and Supportive DevicesConsiderations

■ Braces/orthosis are often required to prevent contractures/deformities

and aid mobility

■ Bracing for spinal deformities is difficult because the decreased sen-

sation makes breakdown more likely

Range of Motion Considerations

■ Clubfoot and rocker-bottom foot deformities may occur secondary to

the unopposed pull of intrinsic foot muscles or muscles at the ankle

joint and as the result of intrauterine positioning

Ventilation and Respiration

■ Cough—determine strength of cough and ability to clear secretions

LanguageLanguage is often impaired in those individuals who have hydrocephalus,

who often demonstrate a “cocktail personality.” They may be verbose

and use clichés and social conversation, which has little depth.

Surgery

■ In myelomeningocele, surgical closure and repair is ideally performed

within 12 to 48 hours to minimize the risk of infection and further

damage to the spinal cord; if there is CSF leakage, immediate surgery

is performed

■ Surgical correction of a Chiari malformation and tethered cord may

be required if neurological signs worsen

■ Other surgical management includes correction of:

■ Contractures of hip and knee musculature

■ Subluxation and dislocation (controversy exists about the need to

relocate hip dislocations in non-ambulatory patients)

■ Club feet and other ankle and foot deformities including

equinovalgus, pes cavus, calcaneovarus and calcaneovalgus.

■ Contractures resulting in functional deficits, including tenotomy of

knee flexors

■ Spinal stabilization is used for correction of kyphosis and scoliosis.

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Prognosis

Prognosis is dependent on the level of the lesion and medical complica-

tions. Generally survival rate is lower, the higher the sensory level of

deficit. Adults with lower level lesions can be employed and self suffi-

cient. With aggressive attention to bowel and bladder management

survival well into adulthood can be expected.

Referral to Other Health-Care Providers

■ Orthopedist for correction of contractures and deformities

■ Neurologist for changes in neurological signs and symptoms

■ Psychological or early childhood specialist for assessment of

cognitive status

■ Speech-language pathologist for feeding issues, language delays

■ Social work for psychosocial issues

■ Orthotist for bracing fabrication and adjustment

Resources

Spina Bifida Association of America http://www.sbaa.org

March of Dimes Birth Defects Foundation http://www.marchofdimes.com

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Nonprogressive Disorders of the Central NervousSystem

Central Vestibular Dysfunction

Vertigo of Central Origin

Description/Overview

Central vestibular dysfunction (CVD) can result from atherosclerosis of the

vertebral and basilar arteries; infarcts to the anterior-inferior cerebellar

artery, posterior-inferior cerebellar or vertebral artery; neoplasms; or

degenerative diseases.

Special Tests

■ Electronystagmography (ENG)

■ Caloric testing

■ Rotational testing

■ Neuroradiologic imaging for diagnosis of a central cause

Differential Diagnosis

■ Orthostatic hypotension

■ Cardiac arrhythmia

■ Psychological dysfunction

■ Peripheral vestibular disorders (Refer to Tab 6)

Surgery

Surgical incision may be used for any tumors affecting the central

vestibular system.

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Prognosis

Prognosis for recovery of CVD varies depending on the underlying cause.

Recovery is typically slower in response to treatment (versus PVD).

Referral to Other Health-Care Providers

■ Neuro-otologic examination

■ Neurologist

Resources

Brown KE, Whitney SL, et al. Physical therapy for central vestibular dys-

function. Arch Phys Med Rehabil. 2006; 87(1):76–81.

Vestibular Disorders Association http://www.vestibular.org

Cerebrovascular Accident

Description/Overview

Cerebrovascular accident (CVA), or stroke, is an interruption of blood flow

to the brain resulting in transient or permanent neurological deficit.

■ Ischemic stroke results when a thrombus or embolus causes a block-

age of cerebral blood flow

■ Hemorrhagic stroke, caused by an intracerebral or subarachnoid hem-

orrhage, can result from an aneurysm, arteriovenous malformation,

or traumatic brain injury

■ Transient ischemic attack occurs from an interruption of arterial blood

flow in the brain that resolves spontaneously without tissue damage

Common syndromes related to disrupted blood flow include:

Ischemic stroke

■ Ideomotor apraxia (inability to pantomime the use of tools, e.g.. pre-

tending to brush hair)

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■ Right hemisphere infarction

■ Motor impersistence

■ Visual field neglect (attentional, representational, and intentional

neglect)

■ Visuospatial impairment

■ Contralateral neglect

■ Behavioral impairment including agitation and impulsiveness

■ Cognitive impairment including confusion, delusion, and

anosognosia

Basilar artery (lateralized deficits relate to branches of the basilar artery;

ischemia of the entire basilar artery results in bilateral deficits)

■ Nystagmus

■ Ipsilateral decrease in facial sensation

Medical Yellow Flags

PrecautionsSeizures often occur within first week after onset of ischemic stroke; later

onset with hemorrhagic stroke.

■ Stroke is often associated with vascular disease so physician clear-

ance should be obtained before initiating therapy

Symptoms Causes Management• Loss of consciousness,

followed by stiffening and then jerking of extremities; May bite tongue, cheek or lip; drool; and have bladder and bowel incontinence

• Post-strokehypoxia andimpaired bloodflow; metabolicimbalance

• Protect the patientfrom injury

• Contact a physicianfor first occurrenceor change in seizurepattern

Special Tests

■ Thyroid function to test for accelerated atherosclerosis secondary to

hypothyroidism

■ CT scan to diagnose hemorrhage and subdural hematomas

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■ MRI to diagnose ischemic stroke

■ Echocardiogram including transesophageal echocardiography (TEE)

to assess possible cardiogenic sources of stroke

■ Carotid duplex scanning to determine the cause of the stroke and

need for carotid endarterectomy

■ Transcranial and carotid Doppler ultrasonography

Tests and Measures

Anthropometric CharacteristicsAssessment – Assess

■ Girth measurements if edema is present

Motor FunctionAssessment

■ Examine components of motor control (Refer to Tab 2)

■ Describe accuracy (or error) in reaching a target; ability to correct

movement in mid-course

Muscle PerformanceAssessment – Assess

■ Any loss of muscle bulk

■ Substitutions used in movement

PainPotential findings

■ Subthalamic lesions can result in spontaneous pain on the contralat-

eral side that may be triggered by light touch, pressure, or contact

with heat or cold

PostureAssessmen – Assess

Potential findings

■ Persistent posturing may be observed

■ Secondary to pusher syndrome, the patient may leans toward hemi-

plegic side and resists upright positioning

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Reflex IntegrityAssessment

■ Resting posture and position of limbs, and resistance to passive

movement

■ Response to perturbation

Sensory Integrity

Potential findings

■ Sensory deficits may be contralateral or ipsilateral, depending on

location of lesion

Differential Diagnosis

■ Transient ischemic attack

■ Encephalitis

■ Migraine headache

■ Hypernatremia

■ Meningitis

■ Neoplasms of the brain

Surgery

■ Hemorrhagic stroke usually requires surgical evacuation to decrease

intracranial pressure and/or repair defective blood vessels

■ Endovascular coil embolization involves inserting stents into the

defective blood vessel to prevent further damage

■ Carotid endarterectomy is used to remove atherosclerotic deposits in

the carotid artery

■ Arteriovenous malformations require surgery to repair abnormal

blood vessels

Prognosis

Prognosis varies depending on the type of stroke and duration and extent

of obstruction or hemorrhage. Disability can range from minimal loss of

sensory and motor function to loss of motor and sensory functions,

speech, understanding, and reasoning. Prognosis affected by age, pre-

morbid status and social supports.

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Postural Assessment Scale for Stroke (PASS)Items and Criteria for Scoring

Maintaining Date/Pt a Posture Score Score

1. Sitting without

support (sitting

on edge of a

50-cm-high

examination

table with

feet touching

the floor)

2. Standing with

support (feet

position free,

no other

constraints)

3. Standing without

support (feet

position free,

no other

constraints)

Cannot sit

Can sit with slight support, for

example, by 1 hand

Can sit for more than 10 sec

without support

Can sit for 5 min without support

Cannot stand, even with support

Can stand with strong support

of 2 people

Can stand with moderate sup-

port of 1 person

Can stand with support of only

1 hand

Cannot stand without support

Can stand without support for

10 sec or leans heavily on

1 leg

Can stand without support for 1

min or stands slightly

asymmetrically

Can stand without support for

more than 1 min and at the

same time perform arm

movements above the

shoulder level

0

1

2

3

0

1

2

3

0

1

2

3

Referral to Other Health-Care Providers

■ Mental health workers as depression is a common occurrence

■ Speech language pathologist if language is involved

■ Orthotist if splints or orthotics needed

Disease-Specific Tests and Measures

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Postural Assessment Scale for Stroke (PASS)—Cont’dItems and Criteria for Scoring

Maintaining Date/Pt a Posture Score Score

4. Standing on

nonparetic leg

(no other

constraints)

5. Standing on

paretic leg

(no other

constraints)

Changing Posture

6. Supine to

affected

side lateral

7. Supine to

nonaffected

side lateral

Cannot stand on nonparetic leg

Can stand on nonparetic leg for

a few seconds

Can stand on nonparetic leg for

more than 5 sec

Can stand on nonparetic leg for

more than 10 sec

Cannot stand on nonparetic leg

Can stand on nonparetic leg

for a few seconds

Can stand on nonparetic leg

for more than 5 sec

Can stand on nonparetic leg

for more than 10 sec

Scoring of items 6–12 is as fol-

lows: items 6–11 are to be per-

formed with a 50-cm-high

examination table; items 10–12

are to be performed without any

support; no other constraints

Cannot perform the activity

Can perform the activity with

much help

Can perform the activity

with little help

Can perform the activity

without help

Cannot perform the activity

Can perform the activity with

much help

Can perform the activity with

little help

Can perform the activity

without help

0

1

2

3

0

1

2

3

0

1

2

3

0

1

2

3

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Postural Assessment Scale for Stroke (PASS)—Cont’dItems and Criteria for Scoring

Maintaining Date/Pt a Posture Score Score

8. Supine to sitting

up on the edge

of the table

9. Sitting on the

edge of the table

to supine

10. Sitting to

standing up

11. Standing up to

sitting down

12. Standing,

picking up

a pencil

from the floor

Cannot perform the activity

Can perform the activity

with much help

Can perform the activity

with little help

Can perform the activity

without help

Cannot perform the activity

Can perform the activity with

much help

Can perform the activity

with little help

Can perform the activity

without help

Cannot perform the activity

Can perform the activity with

much help

Can perform the activity

with little help

Can perform the activity

without help

Cannot perform the activity

Can perform the activity with

much help

Can perform the activity with

little help

Can perform the activity

without help

Cannot perform the activity

Can perform the activity

with much help

Can perform the activity

with little help

Can perform the activity

without help

0

1

2

3

0

1

2

3

0

1

2

3

0

1

2

3

0

1

2

3

Source: Benaim C, et al. Validation of a standardized assessment of postural control in stroke

patients. The Postural Assessment Scale for Stroke Patients. Stroke. 1999;30:1862–1868,

with permission.

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Resources

American Stroke Association www.strokeassociation.org

National Stroke Association www.stroke.org

Traumatic Brain Injury (TBI)

Description/Overview

It can result from a closed head injury (acceleration, deceleration, and

rotational forces resulting in compression and/or shearing of brain tissue)

or an open head injury, in which there is disruption to the skull.

Complex concussion also includes cases of repeated concussions,

including those that result from progressively less impact.

Mild traumatic brain injury or concussion can result in post concussive

syndrome in which the following symptoms can last more than 1 year:

Confusion Visual disturbances Disorientation

Dizziness Headache Balance disturbances

Memory deficits Fatigue Mood swings/irritability

Seizures Poor attention/concentration

Moderate to severe TBI can include the symptoms previously listed but

also may initially result in nausea and vomiting, a worsening headache,

seizures, lethargy, pupil dilation, loss of coordination, weakness of the

extremities and agitation.

Special Tests

Refer to Tab 1 Bonus Content imaging techniques related to skull frac-

tures, intracranial hemorrhage, and hematomas.

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CNS-NP

Tests and Measures

Integumentary IntegrityConsiderations

■ Due to deficits in sensation and language, potential areas of break-

down should be monitored

■ Patient/family should be trained to recognize early stages of skin

breakdown

Motor FunctionAssessment - Provide narrative description of

■ Ability to initiate, maintain, and terminate movement

■ Ability to shift weight

Medications

Generic Brand Common Indications Name Name Side EffectsSpasticity— diazepam Valium Dizziness, drowsiness,

overall ataxia, nausea, blurred

vision, slurred speech,

confusion, impaired

memory

Seizures phenytoin Dilantin, Slurred speech, dizziness,

Diphenylan nausea, incoordination,

diplopia, nystagmus

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Surgery

Surgery may be needed to evacuate hematomas and decompress the

injured brain.

Prognosis

The prognosis is dependent on the:

■ Severity and location of the injury

■ Duration and severity of the coma

■ Duration of post-traumatic amnesia

■ Age and general health of patient

Referral To Other Health-Care Providers

■ Neuropsychologist—behavioral/cognitive testing, depression, and

anxiety

■ Speech-language pathologist for language and dysphagia

■ Mental health worker to work with multiple needs that occur with

recovery

Resources

Brain Injury Association of America www.biausa.org

Brain Trauma Foundation www.braintrauma.org

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Progressive Disorders of the Central Nervous System(CNS-P)

Alzheimer’s Disease

Description/Overview

Dementia is an acquired, generalized, & usually progressive impairment

of cognitive function without changes in consciousness. Dementia is not

a part of normal aging. Many diseases may cause dementia, with

Alzheimer’s disease as the leading one.

According to the National Institute of Health (NIH), Alzheimer’s disease

(AD) initially affects cognitive function & can be divided into 4 stages: pre-

clinical, mild, moderate, & severe. In the preclinical stage, patients often

demonstrate minimal cognitive impairment with memory loss usually

being the first visible sign. AD diagnosis usually occurs during the mild

stage in which symptoms include memory loss, confusion (feeling lost

despite being in a familiar place), taking more time to accomplish daily

tasks, problems with money-handling & bill-paying, poor judgment lead-

ing to bad decisions, loss of spontaneity & initiative, mood & personality

changes, & increased anxiety. In AD’s moderate stage, patients may

demonstrate more severe cognitive, perceptual and/or motor problems

such as hallucination, delusion or paranoia, making repetitive statements

or movements, lack of impulse control, difficulty rising from a chair, etc.

Finally, patients with severe AD may no longer recognize family members

or loved ones & often are completely ADL-dependent.

Special Tests

■ No definitive laboratory & imaging tests to diagnose AD

■ MRI, blood test, CSF exam to rule out other diseases

Differential Diagnosis

■ Dementia associated with mini stroke or Lewy bodies

■ Brain tumors (mass lesions)

■ Delirium

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■ Frontotemporal dementia (Pick’s disease)

■ Prion diseases (Creutzfeldt-Jacob’s disease)

■ Depression

■ Hydrocephalus

■ B12 deficiency

■ Hypothyroidism

■ Demyelinating diseases

■ Infectious diseases (meningitis, syphilis, HIV/AIDS, etc.)

■ Lead, mercury, arsenic, or copper poisoning

■ Head trauma

■ Wilson’s disease

Prognosis

According to NIH, patients with AD generally live between 5 & 20 years

post diagnosis with the average life expectancy post diagnosis being

8 years.

Referral to Other Health-Care Providers

■ Dieticians

■ Neurologists

■ Psychiatrists

■ Social workers

Amyotrophic Lateral Sclerosis(ALS or “Lou Gehrig’s Disease”)

Description/Overview

Amyotrophic lateral sclerosis (ALS) is characterized by a progressive

degeneration of motor neurons in the spinal cord & cranial nerves. ALS

generally does not involve sensory or autonomic nervous systems.

Patients with ALS may demonstrate both lower motor neuron & upper

motor neuron symptoms, & recent research shows that a small percent-

age of patients with ALS may experience cognitive problems including

memory loss, decision-making difficulty, & even dementia.

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For 70% to 80% of the ALS population, initial symptoms involve the

limbs while the remaining population experiences initial symptoms

associated with bulbar signs (difficulty swallowing, slurred speech,

hoarseness, or low speech volume).

Special Tests

■ Neurological exam should only show muscular or motor involve-

ment; sensory tests should be normal

■ NCV & EMG (with fasciculation potentials) to verify ALS diagnosis;

sensory nerve conduction velocity (NCV) may appear normal but the

motor nerve is affected

■ Blood test to detect creatine kinase presence & to confirm familial ALS

■ Respiratory system test to determine if respiratory muscles are

affected

■ MRI & CSF exam to rule out other neurological diseases

Differential Diagnosis

■ Other noninfectious disorders of anterior horn cells (motor neurons)

■ Brain stem tumors

■ Cervical spondylosis

■ Dermatomyositis or polymyositis

■ HIV-associated progressive neuropathy & myopathy

■ Lyme disease

■ Multifocal motor neuropathy (diabetic neuropathy)

■ Myasthenia gravis

■ Spinal muscular atrophy

Prognosis

Most patients with ALS die from respiratory failure 3 to 5 years following

the onset of symptoms. Only about 10% of patients with ALS survive for

10� years.

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Referral to Other Health-Care Providers

■ Dietician (patients with advanced illness may have difficulty swallow-

ing & may need tube feeding)

■ Neurologist

■ Occupational therapist

■ Orthotist

■ Pulmonary specialist

■ Speech & language pathologist (for swallowing & feeding & assistive

technology for communication)

■ Social worker

Brain Tumor

Description/Overview

According to NIH, brain tumors (BT) primarily affect two groups of

patients: children 0 to 15 years of age & adults 40 to 60 years of age. The

most prevalent BT types are anaplastic astrocytoma & glioblastoma

(38%) & meningiomas & other mesenchymal tumors (27%). BT can be

malignant or benign & can produce symptoms primarily by pressing

against or destroying functioning brain tissue. Symptoms directly corre-

late to BT location (refer to CVA for specific cerebral lesion symptoms,

CNS-NP tab).

Special Tests

■ MRI (more sensitive than CT) to confirm tumor existence

■ Biopsy to determine brain tumor cell type

■ EEG to monitor brain activity & detect seizures

■ Endocrine assessment to determine if tumor is located in either

pituitary gland or hypothalamus

■ CSF exam & blood & urine tests to rule out other diseases

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Differential Diagnosis

■ Brain abscess

■ CNS infections

■ Stroke

Surgery

Perform tumor removal surgery if possible

Prognosis

Prognosis of primary BT depends on type of tumor. From 1997–2002,

the 5-year survival rate for brain & other nervous system cancer was

approximately 33%.

Referral to Other Health-Care Providers

■ Neurologists

■ Neurosurgeons

■ Occupational therapists

■ Oncologists

■ Respiratory therapists

■ Wheelchair/Assistive Technology specialists

Cerebellar Degeneration

Description/Overview

Unilateral damage to a cerebellar hemisphere (vascular occlusion, tumor,

or white matter demyelination in one or more cerebellar peduncles)

results in symptoms affecting the same side of the body as the damaged

hemisphere. Patients with middle cerebellum lesions or patients with

multiple sclerosis (demyelination throughout the brain & spinal cord)

show bilateral symptoms such as ataxic gait.

Types of cerebellar degeneration (CD) include: alcoholic cerebellar

degeneration (occurs among chronic alcoholics & may be caused by

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58

nutritional deficiency); phenytoin-induced cerebellar degeneration

(caused by high dose of phenytoin therapy); paraneoplastic cerebellar

degeneration (caused by lung cancer, Hodgkin’s disease, or breast cancer);

autosomal dominant spinocerebellar ataxias (an inherited, mutated gene);

& Friedreich ataxia (an inherited, mutated gene that first shows signs in

childhood).

Special Tests

■ Genetic testing to identify & verify mutated gene(s)

■ CT & MRI to rule out posterior fossa tumor, lung & breast cancer &

Hodgkin’s disease

■ Blood test & CSF exam to rule out infection

Differential Diagnosis

■ Ataxia - Telangiectasia

■ Wilson’s disease

■ Prion disease (Creutzfeldt-Jakob’s disease)

■ Posterior fossa tumor

■ Posterior fossa malformation

Prognosis

Prognosis for patients with CD varies depending on underlying cause.

Patients with Friedreich ataxia usually become wheel-dependent 15 to

20 years after the appearance of symptoms.

HIV Infection

Description/Overview

The U.S. Center for Disease Control & Prevention (CDC) estimates that HIV

infects 40,000 Americans each year. Acquired Immune Deficiency

Syndrome (AIDS), a condition that occurs in the most advanced stages of

HIV, may take many years to develop following the initial infection. In the

U.S., approximately 40% of adults with AIDS experience neurological

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CNS-P

complications, affecting CNS, PNS &/or ANS. Refer to Tab 6 (page xx) for

adults with AIDS and PNS involvement.

There are a number of CNS opportunistic infections found in patients

with HIV/AIDS, the most common of which is cerebral toxoplasmosis

(10-20%). Initial symptoms include malaise, fever, neck stiffness &

hemiparesis while less common symptoms include confusion, aphasia &

vision changes. Cryptococcal meningitis, the most common mycotic

infection in patients with AIDS, may take either an acute course (including

severe headache, mental status changes, fever, nuchal rigidity & focal

signs) or a subacute course (including malaise & headache without neck

stiffness) over several weeks. Cytomegalovirus (CMV) encephalitis usually

results in mental status changes evolving over several weeks & often

causes death. Other common CNS disorders found in patients with

advanced HIV/AIDS illness are AIDS-related dementia, CNS lymphoma &

progressive multi-focal leukoencephalopathy.

Special Tests

■ Laboratory tests to confirm diagnosis & determine viral load &

opportunistic infection type

■ CSF test (by lumbar puncture) to determine CNS opportunistic

infection type & rule out other diseases

■ MRI & CT brain scan to examine infection-related lesions

Differential Diagnosis

■ Classical bacterial cerebral abscess

■ Dementia

■ Other types of meningitis

■ Primary CNS lymphoma

Prognosis

90% of patients with cerebral toxoplasmosis respond to medications

within 2 to 4 weeks. CMV encephalitis often causes death in patients with

HIV/AIDS within weeks to months.

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Referral to Other Health-Care Providers

■ Infectious diseases specialists

Huntington’s Disease/Huntington’s Chorea

Special Tests

■ Genetic testing to confirm diagnosis & identify mutated gene

■ CT & MRI to verify atrophy of cerebral cortex & caudate nucleus

Differential Diagnosis

■ Benign hereditary chorea

■ Alzheimer’s disease

■ Wilson’s disease

■ Basal ganglion or subthalamic infarction

■ Multiple sclerosis

■ Parkinson’s disease treated with levodopa

■ Other drug-induced chorea

Prognosis

There is no cure for Huntington’s disease. Patients with HD usually die 10

to 30 years after the initial onset of clinical symptoms.

Referral to Other Health-Care Providers

■ Dietitians

■ Psychiatrists

■ Speech & language pathologists

■ Specialists for genetic counseling

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Lyme Disease

Description/Overview

Lyme disease (LD) is a tick-borne disorder resulting from a systemic

infection with spirochete Borrelia burgdorferi & causing flu-like symp-

toms including fever, headache, malaise, muscle & joint pain, & swollen

lymph nodes. About 70% to 80% of patients develop a “bull’s eye” rash

characteristic at the site of the tick bite after a delay of 3 to 30 days. If

untreated, patients may develop Bell’s palsy, meningitis, shooting pains,

& heart palpitations, though many symptoms resolve without treatment.

Dark-skinned people may develop a rash that resembles a bruise.

If detected early, most patients can be treated successfully with

antibiotics. If untreated, approximately 60% of patients with LD develop

intermittent bouts of arthritis (severe joint pain & swelling) several

months after infection whereas approximately 5% of untreated patients

with LD develop chronic neurological symptoms months to years after

infection. Chronic neurological symptoms include:

■ Brain dysfunction resulting in memory loss

■ Cranial nerve damage

■ Brain & spinal cord inflammation or meningitis

■ Corneal inflammation causing vision impairment & eye pain

■ Rapidly progressive motor neuron paralysis involving peripheral

nerve inflammation

Special Tests

■ Blood test to confirm B. burgdorferi existence

■ CSF examination (polymerase chain reaction) for patients demon-

strating neurological signs to determine lymphocytic pleocytosis &

slightly elevated proteins

■ Synovial fluid examination (from an affected joint) to confirm

spirochete presence

■ CT & MRI to rule out other diseases

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Differential Diagnosis

■ Alzheimer’s disease

■ Bacterial or viral meningitis

■ Chronic fatigue syndrome

■ Dementia

■ Gout or pseudogout

■ Multiple sclerosis

■ Prion-related diseases

■ Radiculopathy

■ Rheumatoid arthritis

Prognosis

Most patients with LD can be treated successfully with a few weeks of

antibiotics, though patients with chronic, late-stage LD may develop

permanent neurological problems in varying degrees.

Referral to Other Health-Care Providers

■ Infectious diseases specialists

■ Neurologists

■ Orthopedists

Multiple Sclerosis

Special Tests

■ MRI to detect acute & chronic lesions regardless of size

■ Visual evoked potentials to assess visual system

■ CSF analysis to rule out infections, neoplasm, & to confirm

immunoglobulin presence

■ Blood test to rule out other diseases

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Kurtzke Expanded Disability Status Scale for Patientswith Multiple Sclerosis

Functional Systems Findings

Pyramidal functions

Cerebellar functions

Brainstemfunctions

• 0 Normal

• 1 Abnormal signs without disability

• 2 Minimal disability

• 3 Mild or moderate paraparesis or hemiparesis;

severe monoparesis

• 4 Marked paraparesis or hemiparesis; moderate

quadriparesis; or monoplegia

• 5 Paraplegia, hemiplegia, or marked quadriparesis

• 6 Quadriplegia

• V Unknown

• 0 Normal

• 1 Abnormal signs without disability

• 2 Mild ataxia

• 3 Moderate truncal or limb ataxia

• 4 Severe ataxia, all limbs

• 5 Unable to perform coordinated movements due

to ataxia

• V Unknown

• X Is used throughout after each number when

weakness (grade 3 or more on pyramidal) inter-

feres with testing

• 0 Normal

• 1 Signs only

• 2 Moderate nystagmus or other mild disability

• 3 Severe nystagmus, marked extra-ocular weak-

ness, or moderate disability of other cranial

nerves

• 4 Marked dysarthria or other marked disability

• 5 Inability to swallow or speak

• V Unknown

Disease-Specific Tests and Measures

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64

Kurtzke Expanded Disability Status Scale for Patientswith Multiple Sclerosis—Cont’d

Functional Systems Findings

Sensory functions

Bowel & bladder functions

• 0 Normal

• 1 Vibration of figure-writing decrease only, in one

or two limbs

• 2 Mild decrease in touch or pain or position

sense, and/or moderate decrease in vibration in

one or two limbs; or vibratory decrease alone in

three or four limbs

• 3 Moderate decrease in touch or pain or position

sense, and/or essentially lost vibration in one or

two limbs; or mild decrease in touch or pain

and/or moderate decrease in all proprioceptive

tests in three or four limbs

• 4 Marked decrease in touch or pain or loss of

proprioception, alone or combined, in one or two

limbs; or moderate decrease in touch or pain

and/or severe proprioceptive decrease in more

than two limbs

• 5 Loss (essentially) of sensation in one or two

limbs; or moderate decrease in touch or pain

and/or loss of proprioception for most of the body

below the head

• 6 Sensation essentially lost below the head

• V Unknown

• 0 Normal

• 1 Mild urinary hesitancy, urgency, or retention

• 2 Moderate hesitancy, urgency, retention of bowel

or bladder, or rare urinary incontinence

• 3 Frequent urinary incontinence

• 4 In need of almost constant catheterization

• 5 Loss of bladder function

• 6 Loss of bowel & bladder function

• V Unknown

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Kurtzke Expanded Disability Status Scale for Patientswith Multiple Sclerosis—Cont’d

Functional Systems Findings

Visual (optic) functions

Cerebral (mental) functions

Other functions

• 0 Normal

• 1 Scotoma with visual acuity (corrected) better

than 20/30

• 2 Worse eye with scotoma with maximal visual

acuity (corrected) of 20/30 to 20/59

• 3 Worse eye with large scotoma, or moderate

decrease in fields, but with maximal visual acuity

(corrected) of 20/60 to 20/99

• 4 Worse eye with marked decrease of fields &

maximal visual acuity (corrected) of 20/100 to

20/200; grade 3 plus maximal acuity of better eye

of 20/60 or less

• 5 Worse eye with maximal visual acuity (corrected)

les than 20/200; grade 4 plus maximal acuity of

better eye of 20/60 or less

• 6 Grade 5 plus maximal visual acuity of better eye

of 20/60 or less

• V Unknown

• X Is added to grades 0 to 6 for presence of temporal

pallor

• 0 Normal

• 1 Mood alteration only

• 2 Mild decrease in mentation

• 3 Moderate decrease in mentation

• 4 Marked decrease in mentation; chronic brain

syndrome; moderate

• 5 Dementia or chronic brain syndrome; severe or

incompetent

• V Unknown

• 0 None

• 1 Any other neurologic findings attributed to MS

• V Unknown

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66

Kurtzke Expanded Disability Status Scale for Patientswith Multiple Sclerosis—Cont’d

Functional Systems Findings

Expanded Disability Status Scale (EDSS)

• 0 – Normal neurologic examination (all grade 0

in functional systems [FS]; cerebral grade 1

acceptable)

• 1–No disability, minimal signs in one FS (i.e., one

grade 1 excluding cerebral grade 1)

• 1.5–No disability, minimal signs in more than one

FS (more than one grade 1 excluding cerebral

grade 1)

• 2.0–Minimal disability in one FS (one FS grade 2,

others 0 or 1)

• 2.5–Minimal disability in two FS (two FS grade 2,

others 0 or 1)

• 3.0–Moderate disability in one FS (one FS grade 3,

others 0 or 1), or mild disability in three or four FS

(three or four FS grade 2, others 0 or 1)

• 3.5–Fully ambulatory but with moderate disability

in one FS (one grade 3 & one or two FS grade 2)

or two FS grade 3, others 0 or 1, or five FS grade

2, others 0 or 1

• 4.0–Fully ambulatory without aid, self-sufficient,

up & about some 12 hours a day despite relatively

severe disability consisting of one FS grade 4

(others 0 or 1), or combinations of lesser grades

exceeding limits of previous steps; able to walk

without aid or rest some 500 meters (0.3 miles)

• 4.5–Fully ambulatory without aid, up & about

much of the day, able to work a full day, may

otherwise have some limitation of full activity or

require minimal assistance; characterized by rela-

tively severe disability, usually consisting of one

FS grade 4 (others 0 or 1) or combinations of lesser

grades exceeding limits of previous steps; able to

walk without aid or rest for some 300 meters

(975 ft)

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Kurtzke Expanded Disability Status Scale for Patientswith Multiple Sclerosis—Cont’d

Functional Systems Findings

• 5.0–Ambulatory without aid or rest for about

200 meters (650 feet); disability severe enough to

impair full daily activities (e.g., to work a full day

without special provisions); usual FS equivalents

are one grade 5 alone, others 0 or 1, or combina-

tions of lesser grades usually exceeding specifica-

tions for step 4.0

• 5.5–Ambulatory without aid or rest for about

100 meters (325 ft); disability severe enough to

impair full daily activities; usual FS equivalents

are one grade 5 alone, others 0 or 1, or combina-

tions of lesser grades usually exceeding specifica-

tions for step 4.0

• 6.0–Intermittent or constant unilateral assistance

(cane, crutch, brace) required to walk about

100 meters (325 ft) with or without resting; usual

FS equivalents are combinations with more than

two FS grade 3�• 6.5–Constant bilateral assistance (canes, crutches,

braces) required to walk about 20 meters (65 ft);

usual FS equivalents are combinations with more

than two FS grade 3�• 7.0–Unable to walk beyond about 5 meters (16 ft)

even with aid, essentially restricted to wheelchair;

wheels self in standard wheelchair a full day &

transfers alone; up & about in wheelchair some

12 hours a day; usual FS equivalents are combina-

tions with more than one FS grade 4�; very rarely

pyramidal grade 5 alone

• 7.5–Unable to take more than a few steps; restricted

to wheelchair; may need aid in transfers, wheels

self but cannot carry on in standard wheelchair a

full day; may require motorized wheelchair; usual

FS equivalents are combinations with more than

one FS grade 4�

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68

Kurtzke Expanded Disability Status Scale for Patientswith Multiple Sclerosis—Cont’d

Functional Systems Findings

• 8.0–Essentially restricted to bed or chair or peram-

bulated in wheelchair; but may be out of bed

much of the day; retains many self-care functions;

generally has effective use of arms; usual FS

equivalents are combinations, generally grade 4�in several systems

• 8.5–Essentially restricted to bed for much of the

day; has some effective use of arm(s); retains

some self-care functions; usual FS equivalents

are combinations, generally grade 4� in several

systems

• 9.0–Helpless bed patient; can communicate & eat;

usual FS equivalents are combinations, mostly

grade 4

• 9.5–Totally helpless bed patient; unable to com-

municate effectively or eat/swallow; usual FS

equivalents are combinations, almost all grade 4�• 10–Death due to MS

Source: Kurtzke JF. On the evaluation of disability in multiple sclerosis. Neurology. 1961

Aug;11;686–694, with permission.

Differential Diagnosis

■ Small-vessel cerebrovascular disease

■ Spinal cord tumor & cervical spondylosis

■ Spinocerebellar degeneration

■ Lyme disease

■ Guillain-Barré syndrome

■ Systemic lupus erythematosus

■ HIV/AIDS

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Prognosis

The life expectancy for patients with MS is about 6 to 7 years less than

that for a control population without MS.

Referral to Other Health Care Providers

■ Neurologists

■ Ophthalmologists

■ Psychiatrist or psychologists

Parkinson’s Disease

Special Tests

■ No laboratory or imaging tests can definitively diagnose PD

■ MRI, CT scan, & CSF examination to rule out other diseases

Differential Diagnosis

■ Medication-induced parkinsonism (usually from anti-psychotic drugs)

■ Normal pressure hydrocephalus

■ Progressive supranuclear palsy (rigid in neck extension & loss of

vertical eye control)

■ Corticobasal degeneration

■ Multiple system atrophy

■ Vascular parkinsonism from multiple strokes

■ Wilson’s disease

■ Essential (benign, familial) tremor

Surgery

■ Deep brain stimulation

■ Thalamotomy or pallidotomy

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70

Prognosis

There is no cure for PD at this time. PD is chronic & progressive. Some

patients with PD become severely disabled while others experience

onlyminor motor problems. Patients with PD often die from pulmonary

complications.

Referral to Other Health-Care Providers

■ Dietitians

■ Neurologists

■ Neurosurgeons

■ Occupational therapists

■ Social workers

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PNSINJURY

Peripheral Nerve Injury (PNI)

Bell’s Palsy

Description/Overview

According to NIH, Bell’s palsy (BP) is facial muscle weakness or paralysis

resulting from injury to one of the two facial nerves (LMN type). Facial

palsy resulting from facial nerve injury (LMN) differs from facial palsy

resulting from facial motor nucleus or corticobulbar fibers lesions (UMN

type). Generally, BP affects only one side of the face with the frontalis

muscle of the same side being affected, though in UMN types of facial

palsy, the frontalis muscle on both sides of the face remains intact.

BP symptoms usually occur suddenly & may include: inability to close

eye, drooping eyelid or corner of the mouth, drooling, dry eye or mouth,

taste impairment, & excessive tearing in the eye.

Special Tests

■ NCV & EMG 7 to 10 days after symptom onset to confirm diagnosis

■ Laboratory exams, MRI & CT scan to rule out other diseases

Differential Diagnosis

■ Guillain-Barré syndrome

■ Lyme disease

■ Meningitis

■ Facial nerve or cerebellopontine tumors

■ UMN type of facial palsy (bilateral frontalis muscles are intact)

■ Amyloid angiopathy

■ Multiple sclerosis

■ Basal skull fracture

Prognosis

According to NIH, most patients with BP begin to recover within 2 weeks

after the initial onset of symptoms & recover completely within 3 to

6 months. Some patients may never completely recover. The extent of

nerve damage determines recovery timing & process.

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72

HIV Infection

Description/Overview

The U.S. Centers for Disease Control & Prevention (CDC) estimate that HIV

infects 40,000 Americans each year. Acquired Immune Deficiency

Syndrome (AIDS), a condition that occurs in the most advanced stages of

HIV infection, may take many years to develop following initial infection.

In the United States, approximately 40% of adults with AIDS experience

neurological complications.

Peripheral neuropathy observed among patients with HIV/AIDS can be

divided into: HIV-associated sensory or toxic neuropathy, inflammatory

demyelinating polyneuropathy, & autonomic neuropathy.

■ Sensory neuropathies, particularly distal sensory polyneuropathy &

antiretroviral drug toxic neuropathy, are the most common types of

HIV-related peripheral neuropathies & affect approximately 30% of

patients with HIV/AIDS. Characterized by painful feet & lower limb

dysesthesia, sensory neuropathy symptoms generally worsen at

night & can be aggravated by innocuous stimuli, such as bed sheets

or shoes

■ Patients with advanced HIV/AIDS generally experience inflammatory

demyelinating polyneuropathies with Guillain-Barré syndrome–like

symptoms: progressive weakness, decreased or absent deep tendon

reflexes, & minor sensory involvement

■ Symptoms of autonomic neuropathy, a rare condition occurring in

patients with advanced HIV/AIDS, include postural hypotension,

bowel & bladder dysfunction, impotence, sweating abnormalities,

presyncope, & arrhythmia

Special Tests

■ Blood tests to confirm HIV infection & to determine viral load

■ CSF to rule out Guillain-Barré syndrome

■ EMG & NCV to determine affected peripheral (motor & sensory)

nerves

■ Nerve biopsy to assess peripheral nerve inflammation

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Differential Diagnosis

■ Diabetic neuropathy

■ Guillain-Barré syndrome

■ Myopathy

■ Infection-related neuropathy (e.g., cytomegalovirus)

■ Syphilis

■ Vitamin B6 toxicity

Prognosis

According to NIH, the prognosis for patients with HIV/AIDS has improved

significantly in recent years due to new drugs & treatments. However, the

prognosis for patients with HIV/AIDS & peripheral neuropathy worsens

unless the infection is adequately controlled.

Referral to Other Health-Care Providers

■ Neurologist

■ Occupational therapist

■ Orthotist

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Nerve Muscles Innervated Sensory InnervationSciatic nerve Semitendinosus, See common peroneal

(L4–5, S1–3) semimembranosus, biceps nerve & tibial nerve

femoris, adductor magnus

Common Tibialis anterior, peroneus Lateral side of lower

peroneal longus, peroneus brevis, leg, dorsum of foot

nerve (L4–5, peroneus tertius, extensor

S1–2, branch hallucis longus & brevis,

of Sciatic extensor digitorum longus &

nerve) brevis

Tibial nerve Soleus, gastrocnemius, Posterolateral half of

(L5, S1–2, plantaris, politeus, tibialis calf, most of sole

branch of posterior, flexor hallucis

Sciatic nerve) longus, flexor digitorum

longus, all foot muscles in

the sole

Peripheral Nerve Injuries of Lower Limbs

Sciatica

Overview/Description

Emerging from the lumbosacral plexus & branching into the common

peroneal nerves (L4, L5, S1, S2) & tibial nerves (L4, L5, S1, S2, S3), the

sciatic nerve may experience pressure caused by disc herniation, degen-

erative lumbar disc disorders, piriformis syndrome, spinal stenosis or

spondylolisthesis leading to pain radiating down the lower back & into

the back of the lower limbs. Patients with sciatica may also experience

motor problems, including weakened hip extensors & adductors, knee

flexors, ankle dorsiflexors, plantar flexors, evertors, invertors & big toe

extensors; sensory problems such as lower limb & buttock paresthesia or

numbness; & bowel & bladder control problems.

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Special Tests

■ EMG & NCV to confirm sciatic nerve compression level & extent

■ Radiograph, CT scan, & MRI to examine lumbar spine & surrounding

soft tissue & to rule out other diseases

Differential Diagnosis

■ Aortic aneurysm

■ Lumbar spine fracture

■ Sciatic nerve tumor

■ Spinal tumor

■ Tarlov cysts

Surgery

Diskectomy or microdiskectomy can be effective if sciatica is caused by

disc herniation. Repeated epidural injections may temporarily decrease

sciatica.

Prognosis

According to Peul and associates, 95% of patients with sciatica show func-

tional improvement with either conservative treatment (medication &

physical therapy) or surgery at 1-year follow-up.

Referral to Other Health-Care Providers

■ Neurologists

■ Orthopedists

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Peripheral Nerve Injuries of Upper Limbs

Trunk of Division Cord of Brachial of Brachial Brachial Muscles Sensory

Nerve Plexus Plexus Plexus Innervated InnervationMedian nerve(C5–8, T1)

Anterior interosse- ous nerve(branch

of median

nerve)

Upper,

middle,

lower

Upper,

middle,

lower

Anterior

divisions

of the

upper,

middle,

& lower

trunk

Anterior

divisions

of the

upper,

middle,

& lower

trunk

Lateral &

medial

Lateral &

medial

Pronator

teres, flexor

carpi radialis,

palmaris

longus, flexor

digitorum

superficialis,

flexor pollicis

longus,

pronator

quadratus,

thenar mus-

cles, radial

half of two

lumbricals

Flexor pollicis

longus, radial

half of flexor

digitorum

profundus,

pronator

quadratus,

thenar

muscles,

radial two

lumbricals

Radial half

of palm;

palmar side

of thumb,

2nd, 3rd, &

radial half of

4th finger;

dorsal side

of distal

third of 2nd,

3rd, & radial

half of 4th

finger

None

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Peripheral Nerve Injuries of Upper Limbs—Cont’d

Trunk of Division Cord of Brachial of Brachial Brachial Muscles Sensory

Nerve Plexus Plexus Plexus Innervated InnervationRadial nerve(C5–8, T1)

Posterior interosse-ous nerve(branch

of radial

nerve)

Upper,

middle,

lower

Upper,

middle,

lower

Posterior

divisions

of the

upper,

middle,

& lower

trunk

Posterior

divisions

of the

upper,

middle,

& lower

trunk

Posterior

Posterior

Triceps,

brachioradialis.

extensor carpi

radialis longus

& brevis,

supinator,

extensor carpi

ulnaris,

extensor

digitorum,

extensor

pollicis longus

& brevis,

abductor

pollicis longus,

extensor

indices,

extensor digiti

minimi

Extensor carpi

radialis brevis,

extensor

digitorum,

extensor

pollicis longus

& brevis,

abductor

pollicis longus,

extensor carpi

ulnaris,

extensor

indices,

extensor digiti

minimi

Radial 2/3

dorsum of

the hand,

dorsum &

lateral half

aspect of

the thumb,

proximal

1/3 dorsum

of the 2nd &

3rd fingers

& radial

half of the

4th finger

None

Continued

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Peripheral Nerve Injuries of Upper Limbs—Cont’d

Trunk of Division Cord of Brachial of Brachial Brachial Muscles Sensory

Nerve Plexus Plexus Plexus Innervated InnervationUlnar nerve(C7–8, T1)

Lower Anterior

division

of the

lower

trunk

Medial Flexor carpi

ulnaris, ulnar

half of flexor

digitorum

profundus,

palmaris

brevis,

hypothenar

muscles,

adductor

pollicis,

ulnar two

lumbricals,

all interossei

muscles

Ulnar half of

palm and

dorsum of

hand, dorsal

& palmar

side of the

5th finger &

the ulnar

half of the

4th finger

Carpal Tunnel Syndrome

Description/Overview

Carpal tunnel syndrome (CTS) occurs when the median nerve (C5–8, T1)

becomes compressed at the wrist. Sensory problems (tingling or

numbness of thumb, 2nd, & 3rd fingers, radial half of 4th finger, & radial

half of palm) normally are the first indicators of CTS with motor prob-

lems (weak grip or pinch & weak or no thumb flexion & opposition)

appearing later. CTS usually affects the dominant hand first. Patients

with CTS sometimes drop objects from the affected hand when distract-

ed due to sensory problems & weakness. Women are three times more

likely than men to develop CTS.

Median nerve injury at the elbow may cause pronator & wrist flexor

weakness & limited wrist radial deviation in addition to the above func-

tional impairments.

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Special Tests

■ EMG & NCV to determine entrapment level & nerve injury extent

■ Radiographs, CT scan,& MRI of neck & upper limbs to rule out other

pathology

Surgery

Carpal tunnel release surgery (open release or endoscopic surgery) to

reduce compression of median nerve.

Differential Diagnosis

■ Pronator teres syndrome

■ To determine if the patient has pronator teres syndrome

• Patient fully flexes elbow to 90˚

• Examiner resists pronation as patient extends elbow

• Tingling or paresthesia along median nerve distribution in

forearm or hand indicates pronator teres syndrome

■ Tendonitis

■ Tenosynovitis

■ Compressive neuropathies of cervical spine nerve roots & brachial

plexus

■ Proximal median neuropathy

■ Polyneuropathy

Prognosis

According to NIH, most patients with CTS respond well to treatment. The

less severe the symptoms, the better the prognosis.

Referral to Other Health-Care Providers

■ Ergonomists

■ Occupational therapists or hand therapists

■ Orthopedists

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Radial Nerve Injury

Description/Overview

Radial nerve (C5–8, T1) compression in the spiral groove of the humerus

is the most common cause of radial nerve injury (RNI).

Forearm RNI can lead to grasping, finger extension & thumb abduction

problems as well as sensory loss at the radial 2/3 dorsum of the hand,

dorsum, & lateral half aspect of the thumb, proximal 1/3 dorsum of the

2nd & 3rd fingers, & radial half of the 4th finger.

Elbow RNI can lead to difficulty with forearm supination & wrist exten-

sion and above-elbow RNI can cause elbow extension problems in addi-

tion to the aforementioned functional impairments.

Special Tests

■ EMG & NCV to confirm RNI level & extent

■ Radiograph to rule out mid-humeral fracture

■ MRI to rule out nerve tumor, aneurysm, & rheumatoid synovitis

Differential Diagnosis

■ Brachial plexopathy

■ Peripheral neuropathy from diabetes mellitus, renal failure, HIV/AIDS,

Lyme disease, systemic lupus erythematosus, vitamin deficiency, or

toxin exposure

■ Stroke

■ Lateral epicondylitis

Surgery

Depending on the site of compression, several types of decompression

surgery may relieve pressure on radial nerve.

Prognosis

Depends upon RNI type (refer to Tab 1).

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Referral to Other Health-Care Providers

■ Neurologists

■ Occupational or hand therapists

■ Orthopedists

Ulnar Nerve Injury (Entrapment)

Description/Overview

Ulnar nerve (C7–8, T1) injury (UNI), the second most common upper

limb nerve entrapment syndrome, can occur either at the elbow or

wrist. Wrist UNI can lead to inability to: flex the 5th finger; abduct &

adduct fingers; extend the proximal & distal interphalangeal joints of

the 4th & 5th fingers; sense light touch, pain, & temperature on the

ulnar half of palm and dorsum of hand, dorsal & palmar side of the

5th finger, & the ulnar half of the 4th finger.

Elbow UNI can lead to wrist flexor weakness & ulnar deviation loss in

addition to the aforementioned functional impairments.

Special Tests

■ EMG & NCV tests to determine the location of ulnar nerve

entrapment

■ Radiograph to rule out upper limb fractures

■ MRI & CT scan to rule out cervical spine & disc problems

■ Blood & urine tests to rule out other diseases

Differential Diagnosis

■ Cervical disc disease

■ Brachial plexus abnormalities

■ Thoracic outlet syndrome

■ Elbow abnormalities (e.g., epicondylitis, previous medial humeral

epicondyle fractures, etc.)

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■ Neuropathy resulting from infections, tumors, diabetes mellitus,

hypothyroidism, or alcoholism

■ Other systematic diseases (e.g., ALS)

■ Wrist fractures

■ Ulnar artery aneurysms or thrombosis at the wrist

Surgery

May be necessary to decompress ulnar nerve.

Prognosis

Depends on injury severity

Referral to Other Health-Care Providers

■ Ergonomist

■ Occupational or hand therapist

■ Orthopedist

Peripheral Vestibular Diseases

Benign Paroxysmal Positional Vertigo (BPPV)

Special Tests

■ Electronystagmography (ENG) to objectively quantify the velocity,

frequency, & amplitude of spontaneous-induced nystagmus

■ CT scan, evoked auditory potential studies, MRI to rule out acoustic

neuroma & other brain lesions

Differential Diagnosis

■ Central vestibular disorders (refer to Tab 4)

■ Cerebellar tumor or stroke involving cerebellar arteries

■ Acoustic neuroma

■ Vertebrobasilar vascular disease

■ Posterior cerebral vascular disease

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■ Multiple sclerosis

■ Temporal lobe seizure

■ Perilymph fistula syndrome

■ Migraine

Surgery

If conservative treatment (canalith repositioning procedure) or habitua-

tion exercise are unsuccessful, consider bone plug implantation surgery

to block part of affected inner ear.

Prognosis

Most patients with BPPV respond well to the Epley/canalith repositioning

maneuver.

Referral to Other Health-Care Providers

■ Ear, nose, & throat specialists

Meniere’s Disease

Description/Overview

Meniere’s disease (MD), a vestibular disorder with an unknown cause, is

associated with a recurring symptom set that includes sudden onset of

severe vertigo, tinnitus, hearing loss, & pain & pressure in the affected

ear. MD attacks may last from 20 minutes to 24 hours & the duration

between attacks varies, ranging from less than a day to a number of

years. MD symptoms generally involve excessive endolymph in the inner

ear with progressive, fluctuating hearing loss being the most significant

(unlike other peripheral vestibular disorders). MD may affect both ears,

but generally, it affects only one.

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Special Tests

■ Serial audiograms to determine the extent & progression of hearing

loss

■ Brain stem auditory evoked potentials

■ Caloric test

■ Blood test, urine test, ENG, & MRI to rule out other diseases

Differential Diagnosis

■ Brain tumor

■ BPPV

■ Perilymph fistula syndrome

■ Central vestibular disorders (refer to Tab 4)

■ Labyrinthitis

■ Transient ischemic attacks

■ Stroke

Surgery

If medications are ineffective, surgery can be performed to relieve inner

ear pressure (endolymphatic shunt or endolymphatic sac decompression)

or destroy either the inner ear (labyrinthectomy) or vestibular nerve

(vestibular nerve section).

Prognosis

MD is progressive & incurable, but can be controlled through medication.

Referral to Other Health-Care Providers

■ Audiologists

■ Ear, nose, & throat specialists

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Trigeminal Neuralagia (Tic Douloureux)

Description/Overview

Trigeminal neuralgia (TN), also known as tic douloureux due to associated

facial tics, is a chronic pain condition causing severe, sudden burning

episodes or shock-like facial pain lasting between seconds & minutes.

Although TN can affect anyone at any age, it most often occurs in patients

over age 50 & is more common in women. One potential cause of TN is a

blood vessel pressing on the trigeminal nerve as it exits the brain stem. TN

is also associated with demyelinating diseases, such as multiple sclerosis.

Pain See the photo for Trigeminal Nerves in the Cranial/Peripheral Nerve

Integrity section of Tab 2.

Special Tests

■ CT scan & MRI to rule out other diseases

Differential Diagnosis

■ Atypical facial pain

■ Temporomandibular joint syndrome

■ Glossopharyngeal neuralgia

■ Trigeminal root compression due to tumors

■ Dental problems

■ Multiple sclerosis

Surgery

Microvascular surgery can decompress the nerve while different rhizotomy

procedures (alcohol injections, glycerol injections, or partial sensory

rhizotomy) can destroy the offending nerve.

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86

Prognosis

While TN is not fatal, it can cause incapacitation due to severe pain.

Referral to Other Health-Care Providers

■ Neurologists

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Axonal Polyneuropathy

Alcoholic Neuropathy

Alcoholic neuropathy results from long-term alcohol abuse, which causes

a toxic effect on the nerve. The symmetric pattern of neuropathy begins

distally and progresses proximally. Dysesthesia and pain are common

complaints.

Diabetic Neuropathy (Polyneuropathy in Diabetes)

Diabetic neuropathy (DN), one of the most common complications of

diabetes mellitus, can affect all tissues of the body. It usually occurs as

an insidious onset of symmetric damage to the nerve fibers and can

result in pain, impaired sensation, and motor dysfunction. Neuropathy

begins distally and advances proximally.

Diabetes can also result in autonomic neuropathy. The symptoms can

include:

■ Orthostatic hypotension

■ Dysphagia

■ Elevated heart rate

■ Myocardial infarction

■ Arrhythmia

■ Diminished thermoregulatory functions

■ Gastroesophageal reflux disease (GERD)

■ Delayed gastric emptying

■ Constipation

■ Diarrhea

■ Sexual dysfunction

Polyneuropathy Due to Other Toxic Agents

Exposure to environmental and occupational agents such as lead,

arsenic, and mercury can result in PN. Antiviral drugs, including ddI and

ddC, chemotherapy agents such as vinca alkaloids (vincristine), cisplatin,

and paclitaxel and other medications can also cause polyneuropathies.

Once symptoms are recognized, exposure should be eliminated;

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88

chemotherapy dosage may have to be adjusted or terminated. Although

symptoms usually subside with removal of the neurotoxic agent, it may

take months for recovery. However, long-term exposure can result in

permanent neuronal damage, especially in the presence of other medical

conditions, including diabetes and alcoholism.

Polyneuropathy in Other Diseases

Chronic renal failure results in a buildup of uremic toxins. Patients

experience symmetrical sensory and motor neuropathy, which is more

common in the lower limbs than the upper limbs. PN is also a common

occurrence in HIV.

Special Tests

■ Blood glucose levels

■ Electromyography and nerve conduction studies

■ Genetic testing to rule out inherited neuropathies, such as

Charcot-Marie-Tooth disease

Tests and Measures

The values of the following tests & measures are generally within normal

limits in the absence of other complications: Arousal, attention, cognition;

ergonomics and body mechanics; joint integrity and mobility; neuromotor

development; ventilation and respiration; and work, community, and

leisure integration.

Integumentary Integrity Considerations

Assessment

■ Assess skin temperature, usually performed by palpation, but can be

objectively measured with a hand-held infrared skin thermometer,

such as Temp Touch (Xilas Medical (obtain a baseline and compare

temperatures on each side of the body)

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Muscle Performance Assessment

■ Perform manual muscle testing

■ Use dynamometer to test grip strength

Orthotic, Protective, and Supportive DevicesConsiderations

■ The need for properly fitted footwear is vital to protect the foot and

prevent damage

■ Bony prominences should be protected

Differential Diagnosis

■ Myelopathy

■ Radiculopathy

■ Muscle disease

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MedicationsIndications Generic Name Brand Name Common Side Effects

Mild pain Local anesthetic Xylocaine Nervousness, lightheaded-

including: ness, and drowsiness

lidocaine

capsaicin

Peripheral Anticonvulsants Neurontin Low white blood cell

neuropathy including: Tegretol count, nausea, vomiting,

gabapentin Lyrica and dizziness

carbamazepine

pregabalin

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Prognosis

■ Prognosis is dependent on the status of treatment of underlying

cause and/or disease process

■ Diabetic neuropathy progresses slowly and can be halted with

treatment

Referral To Other Health-Care Providers

■ Ophthalmologist for visual acuity

■ Mental health workers for depression that accompanies the pain

associated with polyneuropathies; for treatment of alcoholism

■ Podiatry for foot care

■ Diabetic specialist

Resources.

American Diabetes Association http://www.diabetes.org

Juvenile Diabetes Research Foundation International www.jdrf.org

Lower Extremity Amputation Prevention www.hrsa.gov/leap/default.htm

Guillain-Barré Syndrome

Description/Overview

It occurs following an infectious illness, such as an upper respiratory or

bacterial infection. Progressive paralysis ensues in a matter of days to

weeks. A diagnosis of GBS is made based on clinical findings and his-

tory. Severity varies from mild to complete tetraplegia accompanied by

the need for tracheotomy and mechanical ventilation.

Special Medical Tests

■ EMGs reveal the pattern and severity of nerve involvement and to

confirm the diagnosis

■ Nerve conduction studies reveal slowed conduction velocity and

severity of peripheral neuropathy damage, which may include axonal

degeneration

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■ Lumbar puncture usually contains an elevated level of CSF protein

■ Pulmonary function tests are used for measures of respiratory func-

tion to predict diaphragmatic and abdominal strength and need for

ventilatory support.

■ Videofluoroscopy is used to assess swallowing problems

Tests and Measures

The values of the following tests & measures are generally within normal

limits in the absence of other complications: Anthropometric characteris-

tics; arousal, attention, cognition; ergonomics and body mechanics; joint

integrity and mobility; neuromotor development; posture; and range of

motion including muscle length.

Integumentary Integrity Assessment

■ Assess skin using the Classification of Pressure Sores (Refer to Tab 2).

Potential findings

■ In the early course of GBS, there is a risk of breakdown due to the

significant immobility

Motor FunctionAssessment

■ Assess functional abilities, including getting up from a chair

Self-Care and Home ManagementAssessment

■ Assess bowel and bladder function

Findings

■ Patients may have micturitional disturbances secondary to bladder

areflexia or impaired bladder sensation

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Ventilation and Respiration Assessment

Potential findings

■ Patients requiring ventilatory support are at-risk for developing

pneumonia

Differential Diagnosis

■ GBS is questionable if findings reveal asymmetrical muscle weakness

or a demarcated line of sensory impairment

■ Lyme disease

■ Nutritional and toxic neuropathies

■ Acute myasthenia gravis

■ Chronic inflammatory demyelinating polyneuropathy

■ Neoplastic meningitis

Prognosis

Recovery from GBS varies; most patients have functional recovery within

6 to 9 months. The greatest residual effects are weakness in the anterior

tibialis and intrinsic muscles of the hands and feet. Residual weakness is

present after 3 years in about 30% of people with GBS.

The risk of death is low but increases with age and for those needing

ventilatory assistance, especially when complicated by pneumonia or

sepsis.

Referral to Other Health-Care Providers

■ Speech-language pathologist to address dysphagia and other

oral-motor issues

■ Social work and psychology

Resources

Guillain-Barré Syndrome International http://www.gbsfi.com/

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Nonprogressive Disorders of the Spinal Cord (Spinal Cord Injury)

Description/Overview

■ Spinal cord injury (SCI) exists in a 4:1 male versus female ratio

■ According to the National Spinal Cord Injury Database, the distribu-

tion of SCI injuries is as follows

■ Incomplete tetraplegia (34.5%)

■ Complete paraplegia (23.1%)

■ Complete tetraplegia (18.4%)

■ Incomplete paraplegia (17.5%)

■ Patients with paraplegia may demonstrate motor & sensory loss of

the trunk & lower limbs & functional loss in the pelvic organs

■ Patients with tetraplegia may demonstrate motor & sensory loss in

the trunk, upper, & lower limbs, & functional loss in the pelvic organs

■ Patients with complete SCI lack all motor & sensory function below

the injury level, including S4 to S5, whereas patients with incomplete

SCI retain some motor & sensory function below the injury level

■ The neurological level recorded for a patient with SCI is the most

caudal (distal) level of the spinal cord with normal motor & sensory

function on both sides of the body

Special Tests

■ Radiograph to determine the level & extent of injury to the spine

■ CAT scan to determine extent of the bony abnormality or fracture

■ MRI to determine extent of SCI or other soft tissue injury

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94

Disease-Specific Tests and Measures

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Differential Diagnosis

■ Brain tumor

■ Spinal cord tumor

■ Transverse myelitis

Prognosis

According to the Spinal Cord Injury Information Network, the life expectancy

for patients with SCI has increased over the last three decades, though the

higher the lesion level & involvement, the shorter the life expectancy. The

life expectancy for patients with paraplegia is on average 5 to 10 years

shorter than for those without SCI & the life expectancy is 15 to 22 years

shorter for patients with tetraplegia than for those without SCI.

Referral to Other Health-Care Providers

The following referrals should be made:

■ Driver rehabilitation specialist

■ Nurse

■ Occupational therapist

■ Orthotist

■ Neurologist

■ Psychologist

■ Assistive technology/wheelchair specialist

■ Urologist

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