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Besides the classic presentation of ascending paralysis in demyelinating GBS, clinical variants are based on the types of nerve fibers involved (motor, sensory, sensory and motor, cranial, or autonomic); predominant mode of fiber injury (demyelinating vs axonal); and the presence of alteration in consciousness.
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All patients should be treated with either PE or IVIG, even if the disease is mild.
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Although therapy should be initiated within 2 weeks of onset, it is still appropriate to treat patients
Guillain-Barré Syndrome and Variants
Section snippets
Key points
Historical note
Jean-Baptiste Octave Landry in 18591 first described a case of distal sensory “formications” and ascending weakness after a prodromal fever, malaise, and pain who progressed to paralysis over 3 weeks and died from respiratory failure, in addition to another four cases. Sixty years later, Georges Guillain, Jean-Alexandre Barré, and Andre Strohl2 reported two cases with albuminocytologic dissociation on cerebrospinal fluid (CSF) testing and distinguished this syndrome from poliomyelitis-induced
Epidemiology
GBS is an acute monophasic immune-mediated polyradiculoneuropathy with a mean age of onset of 40 years that affects slightly more males than females of all ages, races, and nationalities. The worldwide incidence of GBS ranges from 0.6 to 4 per 100,000 people.3, 4, 5, 6, 7 A systematic literature review of the epidemiology of GBS found the overall incidence of GBS to be 1.1 to 1.8 per 100,000; however, it was lower in children at 0.34 to 1.34 per 100,000.8 Compared with younger cases, the
Clinical features
The most common initial symptom of GBS is acroparesthesia with little objective sensory loss.14 Severe radicular back pain or neuropathic pain affects most cases. Within a few days, weakness ensues commonly in a symmetric “ascending pattern.” Most patients present initially with leg weakness and arm weakness (32%) or selective proximal and distal leg weakness (56%) often spreading to the arm, whereas some have onset of weakness in the arms (12%). A descending presentation mimicking botulism,
GBS variants
Besides classic presentation of GBS, clinical variants are based on the types of nerve fibers involved (motor, sensory, sensory and motor, cranial, or autonomic); predominant mode of fiber injury (demyelinating vs axonal); and the presence of alteration in consciousness. The first GBS variant was MFS and consists of ophthalmoplegia, ataxia, and areflexia without any weakness.17 Most of the patients with MFS present with at least two features and have in support an elevated CSF protein and
Pathogenesis
Although GBS is presumed to be autoimmune, the precise molecular pathogenesis of GBS and its variants is uncertain. Data have implicated essentially every component of the cellular and humoral immune systems. GBS is a complex autoimmune disease of especially the proximal peripheral nerves and the nerve roots mediated in AIDP by lymphocytic mononuclear cell infiltration and intense macrophage-associated segmental demyelination. Much of the evidence for disease pathogenesis is derived from
Antecedent events
An antecedent infection is noted 2 to 4 weeks before the onset in most GBS cases.30 The most common are upper respiratory infections without any specific organism identified. Known viral precipitants, such as Epstein-Barr virus (mononucleosis or hepatitis) and cytomegalovirus (CMV), occur in only 6% of cases. CMV affects younger patients with cranial neuropathies, severe disease, and a higher likelihood of respiratory failure. In HIV, GBS occurs at the time of seroconversion or early in the
Electrophysiologic features
When GBS is suspected, electrophysiologic studies are essential to confirm the diagnosis and exclude its mimics. The differential of pure motor syndrome includes other diseases associated with quadriparesis and paralysis, such as myasthenic crisis, acute presentation of the idiopathic inflammatory myopathies, and the unusual motor neuron disease patient presenting with acute respiratory failure. Associated clinical features are often helpful in distinguishing these from GBS. The finding of
Laboratory features
Routine laboratory testing is unrevealing in GBS, such as a mild and nonspecific elevation of creatine kinase or transaminases. Hyponatremia should in the proper setting raise suspicion for porphyria or syndrome of inappropriate antidiuretic hormone. Marked vomiting, delayed hair loss, or Mees lines may support the need for heavy metal testing. CSF analysis is critically important in all GBS cases and reveals albuminocytologic dissociation, an elevated protein up to 1800 mg/dL45 with 10 or less
General Supportive Care
Observational studies and expert opinion consensus provide guidance to the general management of GBS.55 Given that up to 30% of GBS cases progress to respiratory failure, good supportive care is the most important element of management. Patients with GBS are mostly admitted to the neurologic intensive care unit or an intermediary care telemetry unit to allow for close and frequent monitoring of respiratory, bulbar, and autonomic function. A rapid decline of the expiratory forced vital
Prognosis
Most patients with GBS begin to recover at 28 days with mean time to complete recovery being 200 days in 80% of cases. However, many (65%) have minor residual signs or symptoms often making recovery less than complete.15, 46 Besides that, major residual neurologic deficits affect 10% to 15% of patients. In a study of 79 cases a year after the onset of GBS, 8% had died (all older than 60); 4% remained bedbound or ventilator dependent; 9% were unable to walk unaided; 17% were unable to run; and
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