Acute Inflammatory Demyelinating Polyneuropathy
Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP, Guillain-Barré syndrome) – an acquired disease of autoimmune etiology characterized by ascending paresthesias and weakness that can progress to total body paralysis, autonomic disturbances, and respiratory failure. Campylobacter jejuni, mycoplasm, cytomegalovirus, Epstein-Barr virus, and Haemophilus influenza are pathogens commonly associated with AIDP. Global incidence is about 0.4 to 1.7/100,000; a mild flulike illness precedes ≈60% of cases by 1 to 4 weeks. More than 50% of patients will complain of pain that is initially described as muscular aching and may transition to neuropathic pain as disease progresses. Extraocular muscles and sphincter function are typically spared. Diagnosis is supported by areflexia, progressive weakness in all limbs, relative symmetry of involvement, CSF cytoalbuminologic dissociation (elevated protein, <10 mononuclear cells/mm3), and electrodiagnostic findings.
Plasmapheresis or IV Ig (400 mg/kg/day × 5 days) given during the evolution of symptoms (within 2 weeks of onset) is effective and has proven to decrease the overall recovery time. Glucocorticoids are not effective. Early rehabilitation should emphasize stretching and gradual strengthening; aggressive therapies may cause overwork weakness. A tilt table may be useful in patients with autonomic instability. Prescription of appropriate assistive mobility devices and lower limb orthotics is often indicated.