BASICS

DESCRIPTION

• A syndrome of acute inflammatory demyelination of peripheral (including cranial) nerves.

• Spectrum includes typical Guillain–Barré syndrome (GBS, also known as acute inflammatory demyelinating polyneuropathy; AIDP), Fisher syndrome (FS), and Bickerstaff Brainstem Encephalitis (BBE) and some other variants

• Often postinfectious

EPIDEMIOLOGY

Incidence

• GBS: 0.6–4/100,000

• FS accounts for 1–7% in western countries but up to 25% in Japan.

• Men > women

• Caucasian > African

• Most often 3rd to 7th decade with bimodal peak for FS in 4th and 6th decade.

RISK FACTORS

• Infection:

– Campylobacter jejuni

– Hemophilus influenza

– Viral—CMV, EBV

• Surgery

• Immunization

PATHOPHYSIOLOGY

• Immune inflammatory demyelination of peripheral nerve.

– Perivascular inflammatory infiltrates

– Axonal involvement may occur

• Wallerian degeneration

ETIOLOGY

Exposure to antigen with generation of autoimmune response.

COMMONLY ASSOCIATED CONDITIONS

Antecedent infection in 2/3 of patients.

DIAGNOSIS

HISTORY

• Acute weakness

• Most often beginning in distal lower extremities and ascending over hours to days

• Variable numbness

• Often ascends to involve cranial nerves with facial droop, ptosis, diplopia

• FS presentation—diplopia and gait disturbance

PHYSICAL EXAM

• Weakness, usually ascending

• Weakness in bulbar distribution may lead to apnea.

• Loss of deep tendon reflexes

• Ptosis and ophthalmoparesis may occur with all GBS subtypes but are the hallmark of FS.

• Classic triad of FS variant

– Ptosis and ophthalmoparesis

– Ataxia

– Areflexia

• Ophthalmoparesis may have both peripheral and central nervous system features including: combinations of 3rd, 4th, 6th nerve paresis, symmetric patterns that do not fit one nerve or muscle, internuclear ophthalmoplegia, vertical gaze palsy, nystagmus, and other supranuclear patterns. GBS (especially FS) is the most common cause of complete bilateral ophthalmoparesis.

• BBE includes ophthalmoparesis, ataxia, impaired consciousness and hyper reflexia

• Many patients have mydriasis with poor pupillary light reaction. Light near dissociation may occur.

DIAGNOSTIC TESTS & INTERPRETATION

Lab

• Lumbar Puncture—«“albumino-cytologic dissociation” »—elevated protein with few WBCs. May take a few days until protein elevates.

• Electromyography—peripheral neuropathy with demyelinating and sometimes axonal features.

• AntiGQ1b antibody—especially elevated in FS (>85%). Clinically correlates with ophthalmoplegia and ataxia.

Imaging

• Consider MRI for differential diagnosis of brainstem infarction, Wernicke encephalopathy, brainstem encephalitis.

• Rarely see brainstem signal changes in GBS.

• May see enhancement of cranial nerves.

Pathological Findings

Perivenular inflammatory infiltrate and demyelination of peripheral nerves.

DIFFERENTIAL DIAGNOSIS

• Brainstem encephalitis, infarction, mass, or hemorrhage

• Myasthenia gravis

• Demyelinating disease

• Wernicke’s encephalopathy

• Botulism

• Cavernous sinus lesion

TREATMENT

MEDICATION

• For GBS

• Plasmapheresis (1)[A]

• Intravenous immunoglobulin (2)[A]

• No controlled trial of treatment in FS

ADDITIONAL TREATMENT

General Measures

• ICU monitoring

• Respiratory support

• Monitoring for autonomic dysfunction

Additional Therapies

• Physical therapy for weakness

• Monocular occlusion or prism for acute or nonresolving diplopia

• Care for corneal exposure with facial paresis

SURGERY/OTHER PROCEDURES

With suboptimal recovery of ptosis or ophthalmoparesis-strabismus, or lid surgery.

IN-PATIENT CONSIDERATIONS

Initial Stabilization

With typical GBS and ascending paralysis frequent monitoring of respiratory status and cardiac rhythm.

Admission Criteria

All GBS patients except relatively stable FS patients.

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