Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 24-29: Polyneuropathies & Mononeuritis Multiplex + Key Features Download Section PDF Listen +++ +++ Essentials of Diagnosis ++ Acute or subacute progressive polyradiculoneuropathy Weakness is more severe than sensory disturbances Acute dysautonomia may be life-threatening +++ General Considerations ++ A symmetric sensory, motor, or mixed deficit, often most marked distally Probably has an immunologic basis, but the mechanism is unclear Sometimes follows infections, innoculations, or surgical procedures There is an association with preceding Campylobacter jejuni enteritis The axonal subtypes of the syndrome (acute motor axonal neuropathy [AMAN] and acute motor and sensory axonal neuropathy [AMSAN]) are caused by antibodies to gangliosides on the axon membrane, including anti-GM1, anti-GM1b, anti-GD1a, anti-GD1b, and (in AMAN) anti-GalNAC-GD1a antibodies The Miller Fisher syndrome is another subtype Characterized by the clinical triad of ophthalmoplegia, ataxia, and areflexia Associated with anti-GQ1b antibodies + Clinical Findings Download Section PDF Listen +++ +++ Symptoms and Signs +++ Motor symptoms ++ The main complaint is of weakness Varies widely in severity in different patients Often has a proximal emphasis and symmetric distribution Usually begins in the legs, spreading to a variable extent but frequently involving the arms and often one or both sides of the face The muscles of respiration or deglutition may also be affected +++ Sensory symptoms ++ Sensory symptoms are usually less conspicuous than motor ones, but distal paresthesias and dysesthesias are common, and neuropathic or radicular pain is present in many patients +++ Autonomic symptoms ++ Autonomic disturbances are common, may be severe, and are sometimes life-threatening; they include the following Tachycardia Cardiac rhythm irregularities Hypotension or hypertension Facial flushing Abnormalities of sweating Pulmonary dysfunction Impaired sphincter control +++ Differential Diagnosis ++ Chronic inflammatory demyelinating polyneuropathy (CIDP) Porphyria Diphtheritic neuropathy Toxic neuropathy, eg, lead, mercury, organophosphates, hexacarbon solvents HIV infection Poliomyelitis Botulism Tick paralysis Spinal cord lesion Transverse myelitis West Nile virus infection Periodic paralysis syndrome + Diagnosis Download Section PDF Listen +++ +++ Laboratory Tests ++ The cerebrospinal fluid characteristically contains a high protein concentration with a normal cell count, but this change may take up to 2 weeks to develop White blood cell counts > 50 cells/mcL should prompt consideration of alternative diagnoses +++ Diagnostic Procedures ++ Electrophysiologic (nerve conduction) studies may reveal marked abnormalities, which do not necessarily parallel the clinical disorder in their temporal course + Treatment Download Section PDF Listen +++ +++ Medications ++ Marked hypotension may respond to volume replacement or pressor agents Intravenous immunoglobulin (400 mg/kg/day for 5 days) is helpful Low-dose heparin to prevent pulmonary embolism should be considered Prednisone is ineffective and may prolong recovery time +++ Therapeutic Procedures ++ Respiratory toilet and chest physical therapy help prevent atelectasis Plasmapheresis is of value Best performed within the first few days of illness Best reserved for clinically severe or rapidly progressive cases or those with ventilatory impairment + Outcome Download Section PDF Listen +++ +++ Follow-Up ++ Monitor spirometry Patients should be admitted to intensive care units if their forced vital capacity is declining Intubation is considered if the forced vital capacity reaches 15 mL/kg, the mean inspiratory force reaches –30 mm Hg dyspnea becomes evident, or oxygen saturation declines +++ Prognosis ++ Most patients eventually make a good recovery However, recovery may take many months About 20% of patients have residual disability Approximately 3% of patients have one or more clinically similar relapses, sometimes several years after the initial illness +++ When to Refer ++ All patients should be referred +++ When to Admit ++ All patients should be hospitalized until their condition is stable and there is no respiratory compromise + Reference Download Section PDF Listen +++ + +Verboon C et al. Treatment dilemmas in Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry. 2017 Apr;88(4):346–52. [PubMed: 27837102]