Guillain-Barré Syndrome

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Chapter 608 Guillain-Barré Syndrome

Guillain-Barré syndrome is a postinfectious polyneuropathy involving mainly motor but sometimes also sensory and autonomic nerves. This syndrome affects people of all ages and is not hereditary. Most patients have a demyelinating neuropathy, but primarily axonal degeneration is documented in some cases mainly in China and Japan.

Clinical Manifestations

The paralysis usually follows a nonspecific viral infection by about 10 days. The original infection might have caused only gastrointestinal (especially Campylobacter jejuni, but also Helicobacter pylori) or respiratory tract (especially Mycoplasma pneumoniae) symptoms. West Nile virus also can cause Guillain-Barré-like syndrome, but more often it causes motor neuron disease similar to poliomyelitis. Guillain-Barré syndrome is reported following administration of vaccines against rabies, influenza, and poliomyelitis (oral) and following administration of conjugated meningococcal vaccine, particularly serogroup C.

Weakness usually begins in the lower extremities and progressively involves the trunk, the upper limbs, and finally the bulbar muscles, a pattern known as Landry ascending paralysis. Proximal and distal muscles are involved relatively symmetrically, but asymmetry is found in 9% of patients. The onset is gradual and progresses over days or weeks. Particularly in cases with an abrupt onset, tenderness on palpation and pain in muscles is common in the initial stages. Affected children are irritable. Weakness can progress to inability or refusal to walk and later to flaccid tetraplegia. Paresthesias occur in some cases. The differential diagnosis of acute weakness is noted in Table 599-3 and of Guillain Barré syndrome in Table 608-1.

Bulbar involvement occurs in about half of cases. Respiratory insufficiency can result. Dysphagia and facial weakness are often impending signs of respiratory failure. They interfere with eating and increase the risk of aspiration. The facial nerves may be involved. Some young patients exhibit symptoms of viral meningitis or meningoencephalitis. Extraocular muscle involvement is rare, but in an uncommon variant, oculomotor and other cranial neuropathies are severe early in the course. Miller-Fisher syndrome consists of acute external ophthalmoplegia, ataxia, and areflexia. Papilledema is found in some cases, although visual impairment is not clinically evident. Urinary incontinence or retention of urine is a complication in about 20% of cases but is usually transient. Miller-Fisher syndrome overlaps with Bickerstaff brainstem encephalitis, which also shares many features with Guillain-Barré syndrome with lower motor neuron involvement and might indeed be the same basic disease.

Tendon reflexes are lost, usually early in the course, but are sometimes preserved until later. This variability can cause confusion when attempting early diagnosis. The autonomic nervous system is also involved in some cases. Lability of blood pressure and cardiac rate, postural hypotension, episodes of profound bradycardia, and occasional asystole occur. Cardiovascular monitoring is important. A few patients require insertion of a temporary venous cardiac pacemaker.

Chronic inflammatory demyelinating polyradiculoneuropathies (CIDP, sometimes called chronic inflammatory relapsing polyneuritis or chronic unremitting polyradiculoneuropathy) are chronic varieties of Guillain-Barré syndrome that recur intermittently or do not improve or progress slowly and relentlessly for periods of months to years. About 7% of children with Guillain-Barré syndrome suffer an acute relapse. Patients are usually severely weak and can have a flaccid tetraplegia with or without bulbar and respiratory muscle involvement. Hyporeflexia or areflexia are almost universal. Motor deficits occur in 94% of cases, sensory paresthesias in 64%, and cranial nerve involvement in less than a third of patients. Autonomic and micturitional involvement is variable. CSF shows no pleocytosis and protein is variably normal or mildly elevated. Nerve conduction velocity (NCV) studies and sural nerve biopsy are abnormal. Polymorphic nucleotide repeats in the SH2D2A gene has been associated with a predisposition to CIDP.

Congenital Guillain-Barré syndrome