CEREBRAL PALSY

By: DR HARDEV SINGH MODERATOR: DR SAMIR GROVER

Snr CONSULTANT ORTHOPAEDIC

CEREBRAL PALSY

• A motor function disorder – caused by permanent, non-progressive brain lesion – present at birth or shortly thereafter.

• Non-curable, life-long condition• Damage doesn’t worsen• May be congenital or acquired

A Heterogenous Group of Movement Disorders

CEREBRAL PALSY

Three distinctive features common to all patients with cerebral palsy: (1) Some degree of motor impairment is present,

(2) An insult to the developing brain has occurred,. (3) A neurological deficit is present that is nonprogressive,

In CP

• Muscles are unaffected

• Brain is unable to send the appropriate signals necessary to instruct muscles when to contract and relax

CAUSESOF CEREBRAL PALSY

An insult or injury to the brain

–Fixed, static lesion(s)

– In single or multiple areas of the motor centers of the brain

• Development Malformations– The brain fails to develop correctly.

• Neurological damage – Can occur before, during or after delivery

* Unknown in many instances

CAUSES

Severe deprivation of oxygen or

blood flow to the brain

–Hypoxic-ischemic encephalopathy

or intrapartal asphyxia

CHIEF CAUSE

RISK FACTORS

• Prenatal factors – Before birth– Maternal characteristics

• Perinatal factors – at the time of birth to 1mo

• Postnatal factors– In the first 5 mos of life

Prenatal factors

1)Risk factors inherent to the fetus (most commonly genetic disorders),

2)Factors inherent to the mother (seizure disorders, mental retardation, and previous pregnancy loss),

3)Factors inherent to the pregnancy itself (Rh incompatibility, polyhydramnios, placental rupture, and drug or alcohol exposure).

4)External factors, such as TORCH (toxoplasmosis, other agents, rubella, cytomegalovirus, herpes simplex),

Perinatal Factors

1)Typically is associated with asphyxia or trauma that occurs during labor.

2)Oxytocin augmentation,

3)Umbilical cord prolapse,

4)Breech presentation all have been associated with an increased occurrence of cerebral palsy.

5)Cerebral palsy often is associated with low Apgar scores during this period

Low-birth-weight infants (<1500 g) are at dramatically increased risk of cerebral palsy.

This increased incidence is believed to be due to the fragility of the periventricular blood vessels, which are highly susceptible to physiological fluctuations during pregnancy.

These fluctuations, which include hypoxic episodes, placental pathology, maternal diabetes, and infection, can injure these vessels and lead to subsequent intraventricular hemorrhages.

Grading of Periventricular Lesions

I Bleeding confined to germinal matrix

II Bleeding extends into ventricles

III Bleeding into ventricles with dilation

IV Bleeding into brain substance

Postnatal Causes

Trauma, head injury

Infections

Lack of oxygen

Stroke in the young

Tumor, cyst

TYPESOF CEREBRAL PALSY

Classification of CP

1.Geographical Classification 1.Geographical Classification

2. Physiological Classification2. Physiological Classification

One extremity involved, usually lower

Monoplegia

Geographical Classification

Hemiplegia

In hemiplegia, one side of the body is involved, with the upper extremity usually more affected than the lower extremity.

Patients with hemiplegia, approximately 30% of patients with cerebral palsy, typically have sensory changes in the affected extremities as well.

Hemiplegialimbs on only one side

• Hemiplegia on right side

–Hip and knee contractures

–Talipes equinus (“tip-toeing”

- sole permanently flexed)

–Asteriognosis may be present.

(inability to identify objects by

touch)

Diplegia Diplegia is the most common anatomical type of cerebral palsy, constituting approximately 50% of all cases.

Patients with diplegia have motor abnormalities in all four extremities, with the lower extremities more affected than the upper.

The close proximity of the lower extremity tracts to the ventricles most likely explains the more frequent involvement of the lower extremities with periventricular lesions .

Diplegia/ Paraplegia•both legs•both legs w/ slight

involvementelsewhere

Diplegia

May also have

Contractures of

hips and knees

and

talipes equinovarus (clubfoot).

This type of cerebral palsy is most common in premature infants

Intelligence usually is normal.

Most children with diplegia walk eventually, although walking is delayed usually until around age 4 years.

Quadriplegia In quadriplegia, all four extremities are equally involved, and many patients have significant cognitive deficiencies that make care more difficult

Head and neck control usually are present, which helps with communication, education, and seating.

Quadriplegia

Physiological Classification

3 MAIN TYPES

1. PYRAMIDAL - originates from the motor areas of the cerebral cortex

2. EXTAPYRAMIDAL

- basal ganglia and cerebellum

3. MIXED

4 MAIN TYPES

PYRAMIDAL 1. Spastic CP

EXTAPYRAMIDAL 2. Athethoid CP

3. Ataxic CP

MIXED 4. Spastic & Athethoid CP

Spastic CP

• Increased muscle tone,

tense and contracted muscles – Have stiff and jerky or

awkward movements.

– limbs are usually underdeveloped

– increased deep tendon reflexes

• most common form

• 70-80% of all affected

Spastic is the most common form of cerebral palsy, constituting approximately 80% of cases, and usually is associated with injury to the pyramidal tracts in the immature brain.

Booth showed histologically that this altered muscle function leads to the deposition of type I collagen in the endomysium of the affected muscle, leading to thickening and fibrosis, the degree of which correlated to the severity of the spasticity.

Joint contractures, subluxation, and degeneration are common in patients with spastic cerebral palsy.

•Spastic Quadriplegia

Characteristic “scissors” positions of lower limbs due to adductor spasms.

Athetoid/ Dyskinetic CP

• Fluctuating tone

– involves abnormal involuntary

movements

– that disappear during sleep and

increase with stress.

– Interferes with speaking, feeding,

reaching, grabbing, and any other

skills

– 20% of the CP cases,

Athetoid cerebral palsy is caused by an injury to the extrapyramidal tracts and is characterized by dyskinetic, purposeless movements that may be exacerbated by environmental stimulation.

Choreiform

Choreiform cerebral palsy is characterized by continual purposeless movements of the patient's wrists, fingers, toes, and ankles. This continuous movement can make bracing and sitting difficult.

Rigid Patients with rigid cerebral palsy are the most hypertonic of all cerebral palsy patients.

These patients have a “cogwheel” or “lead pipe” muscle stiffness that often requires surgical release.

Ataxic CP• Poor balance and lack of coordination

– Wide-based gait

– Depth perception usually affected.

– Tendency to fall and stumble

– Inability to walk straight line.

– Least common 5-10% of cases

Ataxic cerebral palsy is very rare

As a result of an injury to the developing cerebellum.

It is important to distinguish true ataxia from

spasticity because with treatment many children

with ataxia are able to improve their gait function

without surgery.

MIXED CP

• A common combination is

spastic and athetoid

• Spastic muscle tone and involuntary movements.

• 25% of CP cases, fairly common

Signs and Symptoms

OF CEREBRAL PALSY

a.

b.

c.

d.e.

f.

g.

h.

Early Signs

• Stiff or floppy posture

• Weak suck/ tongue thrust/ tonic bite/ feeding difficulties

• Poor head control

• Excessive lethargy or irritability/ High pitched cry

Infancy (0-3 Months)

• Abnormal or prolonged primitive reflexes

Moro’s reflexAsymmetric tonic neck reflex

Placing reflexLandau reflex

Early Signs

Late infancy• Inability to perform motor skills as indicated:

– Control hand grasp by 3 months– Rolling over by 5 months– Independent sitting by 7 months

• Abnormal Developmental Patterns: – Hand preference by 12 months– Excessive arching of back– Log rolling– Abnormal or prolonged parachute response

Abnormal Developmental Patterns after 1 year of age:

• “W sitting” – knees flexed,

legs extremely rotated

• “Bottom shuffling” Scoots along the floor

• Walking on tip toe or hopping

Behavioral Symptoms

• Poor ability to concentrate,

• unusual tenseness,

• Irritability

ASSOCIATED PROBLEMSOF CEREBRAL PALSY

• Hearing and visual problems

• Sensory integration problems

• Failure-to-thrive, Feeding problems

• Behavioral/emotional difficulties,

• Communication disorders

• Bladder and bowel control problems, digestive problems

(gastroesophageal reflux)

• Skeletal deformities, dental problems

• Mental retardation and learning disabilities in some

• Seizures/ epilepsy

DiagnosisOF CEREBRAL PALSY

History and physical examination are the primary tools in making the diagnosis of cerebral palsy.

The history should include a thorough investigation of the pregnancy and delivery.

Ancillary studies, such as radiographs, hematological studies, chromosomal analysis, CT, MRI, and positron emission tomography, rarely are needed to make the diagnosis, but may be helpful in determining the type and extent of cerebral palsy present.

Diagnosis of cerebral palsy before age 2 years can be very difficult.

Nelson and Ellenberg found that 55% of children diagnosed with cerebral palsy by 1 year of age did not meet the criteria by age 7 years.

Transient dystonia of prematurity is a condition characterized by increased tone in the lower extremities between 4 and 14 months old and often is confused with cerebral palsy.

ASSESSMENT

CRITERIAP osturing / Poor muscle control and strength

O ropharyngeal problems

S trabismus/ Squint

T one (hyper-, hypotonia)

E volutional maldevelopment

R eflexes (e.g. increaseddeep tendon)

*Abnormalities 4/6 strongly point to CP

TreatmentOF CEREBRAL PALSY

- No treatment to cure cerebral palsy.

- Brain damage cannot be corrected.

• Crucial for children with CP:

–Early Identification;

–Multidisciplinary Care; and

–Support

NON OPERATIVE TREATMENT

• Commonly used as primary treatment or in conjuction with surgery

Medication

Splinting

bracing

Physical therapy

MEDICAL MANAGEMENT

• Common agents - diazepam

- baclofen

- dantrolene

- botulinum toxin

Oral agents:

Dantrolene-acts at the level of skeletal muscle and decreases muscle calcium ion release. It has an affinity for fast twitch muscle fibers and selectively decreases abnormal muscle stretch reflexes and tone.

Diazepam- increases inhibitory neurotransmitter activity (GABA)

Baclofen -inhibit abnormal monosynaptic extensor activity and poly synaptic flexor activity and decrease substance P levels

Intrathecal injection of baclofen requires 1/30 the dose of oral baclofen to achieve a similar or better response.

An implantable programmable pump dramatically decreases

the dose required to affect spasticity and decreases some of

the side effects such as sedation. This pump typically is

implanted subcutaneously in the abdominal wall and requires

refilling approximately every 2 to 3 months.

• -intrathecal baclofen injection

Botulinum toxin type A (BTX-A) (Botox, Dysport) has been used to weaken muscles selectively in patients with cerebral palsy.

BTX-A injected directly into the muscle acts at the level of the motor end plate, blocking the release of the neurotransmitter acetylcholine and inhibiting muscle contraction

This effect begins approximately 24 hours after injection and lasts 2 to 6 monthsCare

– Intra muscular botulinum toxin for 2-6 months

– Safe maximal dose 36-50 U/kg

Physical therapy

Physical therapy typically is used as a primary treatment modality and in conjunction with other modalities, such as casting, bracing, BTX-A, and surgery.

Bracing in patients with cerebral palsy most commonly is used to prevent or slow progression of deformity.

The most commonly used braces for the treatment of cerebral palsy include ankle-foot orthoses, hip abduction braces, hand and wrist splints, and spinal braces or jackets.

Ankle foot orthosis

Floor reaction AFO

• Uses floor reaction force through toe aspect of foot plate to prevent forward tibial progression & subsequent knee collapse

• May be articulated

Knee brace

Ankle-foot-knee orthrosis

Abduction spint

Assisted Gait Trainer

Walking aids

• Axillary crutches

• Elbow crutches

• Walking sticks

SURGICAL MANAGEMENT

Operative Treatment

Operative treatment typically is indicated when contractures or deformities decrease function, cause pain, or interfere with activities of daily living. It is the only effective treatment when significant fixed contractures exist.

Selective posterior rhizotomy In some cases nerves need to be severed to decrease

muscle tension of inappropriate contractions.

How it Works • The sensory nerve fibers in the spinal cord, usually

between the bottom of the rib cage and the top of the hips are divided

• The nerve fibers are then stimulated and the responses of the leg muscles are observed.

• Those that have an abnormal or excessive response are severed.

• Those with a normal response are left intact.• Intensive rehabilitation is required after the surgery,

usually up to six weeks, followed by physical therapy on an ongoing basis

OPERATIVE MENAGEMENT HIP

• Indications

• contracture or deformities causing

pain

Decrease function

Interfere with ADL

PREREQUISITES FOR EFFECTIVE SURGERY

• hemiplegics / diplegics : good results

quadriplegics : minimal improvement

• Age : 3- 12 years

• IQ : good

• Good upper limb function : for walking

• Underlying muscle power should be good

• surgery hardly changes status in walkers/nonwalker, but improves gait

PROCEDURE

• Neurosurgical procedures

• Tendon release

• Muscle tendon lengthening

• Capsulotomies

• Osteotomies

• Resection and replacement procedures

DEFORMITIES• DEFORMITIES OF HIP:

• Flexion deformities

• adduction

• Subluxation and dislocation

SECODARY DEFORMITIES:

• Knee Flexion

• Equinus foot

• Pelvic tilt

• Scoliosis and lordosis

• Pseudoadduction deformity

Flexion internal rotation of hip

Incresed femoral anteversion

External tibial torsion

Planovalgus feet

Pt sit in the ‘W’ position

Migration index (MI) is calculated by dividing width of uncovered femoral head A by total width of femoral head B

Hip flexion deformity

Indication for surgery

• Hip flexion deformity never decrease by physiotherapy.

• Hip flexion deformity > 20 needs surgery

Hip flexion contracture hip , knee & ankle contracture

single stage

15-30 deg flexion >30 deg flexion multi level correction

ilioPsoas lengthening more release of

-rectus femoris

-sartorius

-TFL

-anterior fibers of Gl.minimus

Gl.medius

• Iliopsoas recession is most commonly used than complete tenotomy to avoid excessive

flexion weakness

ADDUCTION DEFORMITY

• Mild contracture severe contractures

Early hip subluxation

Adductor tenotomy more release of

-gracilis

-anterior half of

adductor bevis

• Leave adductor brevis ( the major hip stabilizer )

HIP SUBLUXATION• MI > 30 % Soft tissue release for very young

Flexion adduction deformities

• MI > 50% open reduction + femoral osteotomy

Correction of femoral valgus and anteversion

• AI > 25 deg pelvic osteotomy

(Correction of acetabular deformities)

• Femoral varus and derotational(external rotation) osteotomy

• Acetabular osteotomies:

• Salter osteotomyredirection of the acetabulum anterioly and laterally

• Postero-superior deficiencyshelfs osteotomy

HIP DISLOCATION• MI=100%

Types:

• Posterior dislocation (m.c)

• Anterior dislocation

Seen in

-spastic diplegics

-spastic quadriplegics

TRETMENT OPTIONS IN HIP DISLOCATION

• Observation

• Relocation procedure on femur and acetabulum

• Proximal femoral resection

• Hip arthrodesis

• Total hip arthroplasty

Spastic Diplegia Spastic quadriplegia

hip dislocation :

if detected early: surgery

if detected late : no pain – leave

pain – proximal resection

Combined one stage correction of spastic dislocated hip

1. Soft tissue release

2. Open reduction

3. femoral osteotomy

4. Pericapsular pelvic osteotomy

Varus derotational shortening femoral osteotomy

Pericapsular acetabuloplasty

PROXIMAL FEMORAL RESECTION

FOOTThe most common deformity is ankle equinus, with equinovarus and equinovalgus deformities being equally common.

Equinus Deformity

Equinus deformity is the most common foot deformity in patients with cerebral palsy, affecting 70% of children.

Surgical Correction of Equinus Deformity

Open Lengthening of the Achilles Tendon

Z-Plasty Lengthening of the Achilles Tendon

Percutaneous Lengthening of the Achilles Tendon

Lengthening of the Gastrocnemius-Soleus Muscle

Equinovarus Deformity

Varus deformity, which usually is accompanied by equinus, is caused most commonly by an abnormal posterior tibial muscle that is overactive or firing out of phase.

Lengthening of the Posterior Tibial Tendon

Z-Plasty Lengthening of the Posterior Tibial Tendon

Step-Cut Lengthening of the Posterior Tibial Tendon

Musculotendinous Recession of the Posterior Tibial Tendon

Osteotomy of the Calcaneus

Equinovalgus Deformity

Medial Displacement Calcaneal Osteotomy

Subtalar Arthrodesis

KNEEFlexion Deformity

Flexion is the most common knee deformity in patients with cerebral palsy and frequently occurs in ambulatory children

Spastic hamstrings, weak quadriceps, or a combination of both can cause isolated knee flexion.

Fractional Lengthening of Hamstring Tendons

Combined Hamstring Lengthening, Posterior Capsular Release, and Quadriceps Shortening

Distal Transfer of Rectus Femoris

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