CPC CASE NUMBER 1

Symptoms

Chief Complaint = “I am getting weak” Painful sensations with increasing

muscle weakness in both LE (started in ankles)

Prickly numbness and tightness in lower abdomen

H & P Findings Coronary Artery Disease Campylobacter jejuni diarrhea a few weeks

ago, successfully treated with antibiotics Smokes 1 ppd Vitals Normal except BP Δ from 130/80 to

90/60 when going from supine to standing. Absent patella and achilles reflex Decreased pain and touch in feet Decreased proprioception in LE

CLASS DIFFERENTIAL ?

Our Differentials

Diabetes Herniated Disc Neoplasia Lead Poisoning Demyelinating Disease

DIABETES

Diabetic Peripheral Neuropathy

Diabetic nerve pain result of damage to nerves because of high blood sugar levels over time.

Can affect any nerves and thus a list of possible symptoms, but usually develop over a number of years.

Causes;Not totally clearBelieved to be a number of contributors

○ Metabolic, neurovascular, autoimmune factors○ Mechanical injury to nerves, inherited traits, or

lifestyle factors

Diabetic Peripheral Neuropathy Symptoms that Correspond

painful sensations and increasing muscle weakness of both lower extremities.

has difficulty rising from a chair, climbing stairs, and complains of an unsteady gait.

a prickly numbness in both legs and a band-like tightness across his lower abdomen

indigestion, nausea, or vomitingdiarrhea or constipationloss of reflexes

Diabetes

Hemoglobin A1C - $37On high side of normal range 6.1 (3.8-6.4)

HERNIATED DISC

Herniated Disc

Bulging of nucleus pulposus, with or without nerve root compression

Most common in lumbar region Symptoms:

Back painLeg pain

Occurs mostly in 30’s and 40’s

Herniated Disc

Patient’s symptoms consistent with disc herniation:Painful sensations in LETingling sensations in LEMuscular weakness in LE

Herniated Disc

Patient’s symptoms inconsistent with disc herniation:Bilateral LE pain uncommon25 y/oAtaxia

Herniated Disc

X-ray of LS spine - $190Normal

MRI spine - $1400normal

NEOPLASM

Cauda Equina Syndrome

Compression of large nerve trunksTumor, infection, narrowing of spinal canal

Cauda Equina Syndrome

His symptoms that correspondProgressive loss of sensationMuscle weakness

Cauda Equina Syndrome

His symptoms that don’t correspondBowl or bladder dysfunctionNo muscle atrophy

Neoplasia – Cauda Equina Syndrome

MRI spine - $1400normal

LEAD POISONING

Lead Poisoning

Occupational Hazard: potential lead exposure

His symptoms that correspondPain, numbness, and tingling in the

extremitiesMuscle weaknessAbdominal Tightness

Lead Poisoning

Headache Memory Loss Mood Disorder

Lead Poisoning

Blood lead testNormal range for unexposed individual

DEMYELINATING DISEASE

Demyelinating Disease

Blood Glucose87 (70-110)

LPNormal CSF glucose 100 ( > 2/3 BG) and

high CSF protein 85 (25-45) EMG – nerve conduction

40% slowed nerve conduction in legs. Indicates proximal demyelination

GUILLAIN BARRE = DX

Guillain-Barre Syndrome

General information:Immune system attacks peripheral nerves

○ Antibodies generated for C. jejuni attack gangliosides in PNS

Ascending starting w/ weakness and tingling in legs

Potential to ascend to C3-5 and affect diaphragm/respiratory innervations

Rare 1:100,000 affectedRecovery ranges from weeks to a few years

Guillain-Barre Syndrome National Institute of Neurological Disorders and Stroke

(NINDS) Diagnostic Criteria1:Required Features:

○ Progressive weakness of more than one limb, ranging from minimal weakness of the legs to total paralysis of all four limbs, the trunk, bulbar and facial muscles, and external opthalmoplegia

○ Areflexia. While universal areflexia is typical, distal areflexia with hyporeflexia at the knees and biceps will suffice if other features are consistent

Supportive Features:○ Progression of symptoms over days to 4 weeks○ Relative symmetry○ Mild sensory symptoms or signs○ Elevated CSF protein with a cell count <10mm3○ Electrodiagnostic abnormalities consistent with GBS

Guillain-Barre Syndrome Patient: Dx

High CSF protein w/o increased cell countEMG showing conduction slowing and loss of F

wavesCommonly follows infection

○ camplyobacter jejuni, CMV, Epstein-Barr, herpes, and viral hepatitisU.K. study showed that 26% of GBS affected Pts had

evidence of a recent C. jejuni infection2

Swedish study estimated that the risk for developing GBS within two months of C. jejuni infection was 100x higher than risk in general population3

Orthostatic hypotension (130/80 to 90/60 )

Guillain-Barre Syndrome

Acute inflammatory demyelinating polyneuropathyMost common form of GBS in the United States

and Europe, representing 85-90% of cases.Earliest abnormalities see on clinical

neurophysiology studies are prolonged or absent F waves, reflecting demyelination at the level of the nerve roots.4

Treatment No known cure High dose of intravenous immunoglobin

(IVIG) therapy or plasma exchange therapyEqually beneficial with no apparent benefit of

combining the two therapies Long-term management of neuropathic pain

tricyclic antidepressants, gabapentin, carbamazepine

Monitor for progression of disease

What bacterial infection commonly precedes Guillain Barre?

A. Strep agalactiae

B. C. jejuni

C. Epstein Barr

D. Cryptococcus

E. Serratia Marcescens

Which of these can cause peripheral neuropathy?

A. Diabetes

B. Lead Poisoning

C. Cauda Equina

D. Guillain Barre

E. All of the above

What is the treatment method for Guillain Barre?

A. Surgery

B. Blood Transfusion

C. Plasma Exchange

D. Amputation

E. All the above

Sources 1. Criteria for diagnosis of Guillain-Barre Syndrome. Ann Neurol

1978; 3:565. 2. Rees, JH, Soudain, SE, Gregson, NA, Hughes, RAC.

Campylobacter jejuni infection and Guillain-Barre Syndrome. NEngl J Med1995; 333:1374.

3. McCarthy, N, Giesecke, J. Incidence of Guillain-Barre syndrome following infection with Campylobacter jejuni. Am J Epidemiol 2001; 153:610.

4. Gordon, PH, Wilbourn, AJ. Early electrodiagnostic findings in Guillain-Barre Syndrome. Arch Neurol 2001; 58:913.