A. Guillain-Barré syndrome (GBS) encompasses a group of acute immunemediated polyneuropathies that present with rapidly progressive weakness, areflexia, and elevated cerebrospinal fluid (CSF) protein without pleocytosis.

B. Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is the most common subtype in North America and Europe, occurring at all ages and producing multifocal demyelination of nerve roots and cranial and peripheral nerves.

C. Axonal forms of GBS include acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN). AMAN is seen in children and young adults in northern China, Mexico, and Japan, is commonly preceded by Campylobacter jejuni infection, and generally has a good prognosis.

D. GBS is the most common cause of rapidly progressive sporadic weakness; 60% of patients recover fully, but 15% have major residual deficits and up to 5% succumb to respiratory, autonomic, and other complications of the illness.

1. The major clinical features of AIDP are rapidly evolving weakness (usually over days) and areflexia, heralded by dysesthesias of the feet or hands, or both.

a. Weakness classically ascends from legs to arms but may begin with weakness of cranial nerve-supplied muscles or of upper limb muscles and descend to the legs.

b. Proximal muscle involvement is often seen early in the course of the disease.

c. In severe cases, respiratory and bulbar muscles are affected.

2. Patients may become quadriparetic and respirator dependent within a few days, or they might have only mild nonprogressive weakness of the face and limbs throughout the course of the illness.

3. Weakness typically does not progress beyond 4 weeks.

a. Progression beyond 2 months is designated “chronic inflammatory demyelinating polyradiculoneuropathy” (CIDP), a disorder with a natural history different from GBS.

b. A small percentage (2% to 5%) of patients will have recurrent—two or more episodes—of GBS.

4. Approximately two-thirds of patients have an antecedent infectious event 1 to 3 weeks before the onset of GBS.

a. Often there is a prodromal flu-like or diarrheal illness caused by a variety of infectious agents, including cytomegalovirus, Epstein-Barr and herpes simplex viruses, Mycoplasma, Chlamydia, and C. jejuni.

b. GBS has also been associated with human immunodeficiency virus (HIV) infection, Hodgkin disease, systemic lupus erythematosus, immunization, general surgery, and renal transplantation. Lyme disease can mimic GBS.

1. There is symmetric weakness in both proximal and distal muscle groups associated with attenuation or loss of deep tendon reflexes.

2. Objective sensory loss is usually mild.

3. Between 10% and 25% of patients require ventilator assistance within 18 days after onset. Patients must be followed carefully with serial vital capacity (VC) measurements until weakness has stopped progressing.

4. Mild-to-moderate bilateral facial weakness often occurs in addition to bulbar difficulties.

5. Ophthalmoparesis is unusual unless seen in the Miller Fisher variant (characterized by ophthalmoplegia, ataxia, and areflexia, with little limb weakness).

6. Autonomic nervous system disturbances are seen in more than 50% of patients and include cardiac arrhythmias, orthostatic hypotension, hypertension, transient bladder paralysis, increased or decreased sweating, and paralytic ileus.

1. Patients with axonal forms (AMAN and AMSAN) present similarly to AIDP with rapidly progressive weakness, areflexia, and albuminocytologic dissociation, but they differ in the following ways:

a. AMAN patients lack sensory abnormalities, and this form is more commonly found in northern China, Japan, and Mexico during summer months among children and young adults, is very commonly associated with prior C. jejuni infection, and has a good prognosis for recovery.

b. AMSAN is generally associated with more pronounced deficits and longer time to recovery.

1. Cerebrospinal fluid.

a. An elevated CSF protein without an elevation in cells (albuminocytologic dissociation) is characteristic of GBS.

b. CSF protein may be normal within the first 48 hours, but often is elevated within 1 week of onset; rarely, it remains normal several weeks after the onset of GBS.

c. The cell count rarely exceeds 10 cells/cm3 and is mononuclear in nature.

d. When GBS occurs as a manifestation of HIV infection or Lyme disease, the CSF white cell count is generally increased (25 to 50 cells).

e. The CSF glucose is always normal.

2. Electrodiagnostic studies in AIDP.

a. Typically disclose slowing (<80% of normal) of nerve conduction velocity, with prolonged distal motor and sensory latencies.

b. The amplitude of the evoked motor responses is typically reduced, and the form of the responses is often dispersed.

c.

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