Guillain Barre Syndrome

Guillain-Barre syndrome, also known as Landry's ascending paralysis or acute inflammatory demyelinating polyneuropathy, is characterized by rapidly progressive ascending paralysis following a viral or bacterial infection. The incidence is approximately one per 50,000 individuals in the United States (84). Initially, patients exhibit weakness or abnormal sensations in the extremities. Paralysis often ensues, with progression of the extremity weakness involving the muscles of respiration and often facial paralysis or other cranial neuropathies.

Current theories of the pathogenesis of Guillain-Barre syndrome postulate a postviral autoimmune demyelination of the nervous system. Guillain-Barre disorder should always be considered in any patient presenting with bilateral facial nerve involvement. In a study of 43 patients with bilateral facial involvement, six patients were diagnosed with Guillain-Barre syndrome or a variant thereof (85). Many variations of Guillain-Barre syndrome have been described, some with particular predilection for cranial nerve involvement. In fact, a syndrome of multiple cranial nerve palsies as a variant of Guillain-Barre syndrome was first described by Guillain himself in 1937 and included bilateral facial paralysis (86). Another variant known as Miller-Fisher syndrome consists of ataxia, areflexia and ophthalmoplegia and has been strongly associated with facial diplegia (87).

Diagnosis of Guillain-Barre syndrome and its variants is largely based on clinical symptoms, but lumbar puncture and nerve conduction testing can be confirmatory. Treatment with intravenous immunoglobulins or plasmapheresis can hasten the course and dampen the severity of the paralysis. Corticosteroid therapy, sometimes prolonged, has also been found beneficial in randomized trials. During the acute stages of the disease, patients often require extensive support, including artificial respiration. Despite the dramatic progression, the prognosis is quite good, with over 80% of patients achieving a full recovery.

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