NEUROLOGIC EMERGENCIES

Nick Busch

Henry Ford EM Grand Rounds

August 14th, 2014

GOALS

Basic pathophysiology of each disease

Clinical Features (for ER diagnosis)

Board Exam Points

Practical ER Management

OUTLINE

Review of nerve and neuromuscular junction

Transverse Myelitis

Guillain-Barré Syndrome

Botulism

Myasthenia Gravis

Conclusion

TM

GBS

MG

Botulism

MYELIN SHEATH

CASE: PROGRESSIVE WEAKNESS

CASE CONTINUED

MYELITIS

“Acute transverse myelitis”

Pathogenesis: infectious, autoimmune, idiopathic

30% post viral infection

Clinical features

Paraplegia, sensory impairment, sphincter

disturbance

Fever, back pain, paresis, hypertonia, hyperreflexia,

clonus, +Babinski

MYELITIS

Diagnosis: CSF not useful, r/o other disease

Differential: MS, spinal epidural abscess, spinal

neoplasm, or hematoma

Management

Steroids (?)

MRI to exclude compressive lesions

Neuro consult and hospitalization

Common Theme

MYELITIS

Clinical Course:

24 hours: two thirds reach maximal deficit

3-6 months: maximal improvement

5 years:

55% good or fair recovery

30 % poor outcome

15 % dead

GUILLAIN-BARRÉ SYNDROME

„ghee-yan bah-ray‟

GUILLAIN-BARRÉ SYNDROME

Autoimmune, demyelinating

Clinical features

Progressive, symmetrical distal weakness

“ASCENDING WEEKNESS”

Usually lower extremities, 32% all, 10% upper

Decrease in deep tendon reflexes

Sphincter and ocular muscles spared (usually)

Half have autonomic dysfunction

GUILLAIN-BARRÉ SYNDROME

Diagnosis:

CSF: high protein, slight pleocytosis

MRI

Differential: MS, spinal epidural abscess, spinal

neoplasm, or hematoma

GUILLAIN-BARRÉ SYNDROME

Subtypes

Acute inflammatory demyelinating polyneuropathy

(90%) - AIDP

Acute motor axonal neuropathy - AMAN

Acute motor and sensory neuropathy - AMSAN

GUILLAIN-BARRÉ SYNDROME

Causes

AIDP – myelin invasion of the myelin sheath

Common organisms

Campylobacter jejuni ***

CMV, EBV and mycoplasma

GUILLAIN-BARRÉ SYNDROME

Management

Respiratory Function

IVIG

Plasma exchange

Neuro consult ICU

Do not treat high blood pressure

Usually transient, followed by unpredictable hypotension

No Difference in outcomes

GUILLAIN-BARRÉ SYNDROME

Respiratory Function

GUILLAIN-BARRÉ SYNDROME

Clinical Course:

Highly variable

Predominately sensory = better outcome

½ peak in 1 week, ½ long term sequelae

1/3 need vent support

3% mortality

3% recurrence

CASE CONCLUSION

• Follow up– Rehab

– 5 months walker, foley out

– 1 year: at home, no walker, continued paresthesias

BOTULISM

“Toxin mediated illness causing acute weakness”

Toxin targets peripheral NMJ and autonomic

synapses

Clostridium Botulinum

Strictly anaerobic, gram positive, rod-shaped

BOTULISM

Justinus Kerner (1786-1862)

Latin word for sausage = "botulus"

PHYSIOLOGY REVIEW

BOTULISM

7 toxin types (A,B,E and F cause disease in

humans)

110 cases confirmed per year by CDC

BOTULISM

Food-Borne

Infant

Wound

Unclassified (adult infectious)

Inadvertent (Botox)

Bioterrorism

BOTULISM (FOOD-BORNE)

Clinical Features (food-borne)

18-36 hrs typically (6 hrs – 8 days)

Early: malaise, lightheadedness, N/V, constipation

Neuro signs: CN first affected (diplopia, blurred

vision, dysphonia, dysphagia, dysarthria, vertigo)

DECENDING SYMETRIC PARALYSIS

Autonomic dysfunction: dry mouth, illeus, retention

Dilated and fixed pupils

• 8 cases of botulism after wine

consumption

• 3/8 intubated

• All received antitoxin

– 4-5 days post consumption

– Average 12 hrs after presentation

BOTULISM MANAGEMENT

Respiratory Management

Vital capacity <12 mL/kg criteria for intubation

NG tube

Foley

Anti-toxin

BOTULISM ANTI-TOXIN

Anti-toxin

Equine trivalent: antibodies to A, B, and E

Circulating toxin only (will not unbind)

Prevents progression, decrease hospital stay,

decreased respiratory failure

CDC or state health department

One 10 mL vial (no repeated doses)

INFANT BOTULISM

Ingestion of spores

Classic: Honey <1 yr

Hypotonia, poor feeding, constipation

Longer incubation period

Large Differential: Sepsis, IEM, ect.

• BabyBIG: human immunoglobulin

Adult anti-toxin = high risk anaphylaxis

MYASTHENIA GRAVIS

Βαρύςgravis severe

Μῦς mys muscle

ἀσθένεια astheneiaweakness

MYASTHENIA GRAVIS

Auto-antibodies against nicotinic acetylcholine

receptor at NMJ

Complement mediated destruction

Compete with Ach for receptors

MYASTHENIA GRAVIS

Epidemiology

Bimodal

Clinical Features

Ocular symptoms: ptosis, diplopia, blurred vision

Respiratory failure (rarely first symptom, 17% will

have in first 2 years)

Fatigability (worse at end of the day)

MYASTHENIA GRAVIS

Diagnosis

Edrophonium Test

Ice bag Test

MYASTHENIA GRAVIS

Myasthenic Crisis

Respiratory failure leading to mechanical ventilation

Causes: infection, aspiration, med changes, surgery,

pregnancy

Monitor, NIPPV, mechanical ventilation

DRUGS EXACERBATING MG

Betablockers

Ca-Channel Blockers

Quinidine

Lidocaine

Procainamide

Aminoglycosides

Tetracyclines

Clindamycin

Polymyxin B

Phenytoin

Neuromuscular Blockers

Corticosteroids

Thyroxine

MYASTHENIA GRAVIS

Treatment

Cholinesterase Inhibitors

Immunosuppressants

Thymectomy

Immunoglobulin Therapy

Plasmapheresis

THE PLASTIC CIGAR

Vital Capacity

Negative Inspiratory Force

PULMONARY FUNCTION TESTING

CONCLUSION

Transverse Myelitis

Guillain-Barré Syndrome

Botulism

Myasthenia Gravis

REFERENCES

Available Upon Request