The Miller Fisher syndrome. Review of the literature.

The triad of ataxia, areflexia and ophthalmoplegia was first described as a variant of the Guillain-Barre syndrome in 1932 by Collier. In 1956, Miller Fisher reported three patients with ataxia, areflexia, and ophthalmoplegia as a separate entity. Since then, 223 cases of Miller Fisher syndrome have been published. The male/female ratio is 2:1 with a mean age of 43.6 years at the onset of the disease. A viral infection preceded the neurological symptoms in 71.8% of cases with an average symptom-free interval of 10 days. First symptoms were diplopia (38.6%) or ataxia (20.6%). An areflexia was present in 81.6% of cases. Cranial nerves other than the oculomotor nerves were involved in 127 cases (56.9%): cranial nerves 7 (45.7%), 9 and 10 (39.9%), and 12 (13%) were involved. In 53 cases a tetraparesis occurred. An elevated protein value was present in 134 patients (64.4%); cerebrospinal fluid findings were normal in 56 patients. Eighteen patients showed a mild pleocytosis. Besides the cerebellar type of ataxia, initial disturbances of consciousness (n = 8), supranuclear oculomotor signs (n = 22), and pathology findings in electroencephalography (n = 38), computed tomography (n = 8), and magnetic resonance imaging (n = 2) were reported as evidence for a central nervous system involvement in the Miller Fisher syndrome. The prognosis of Miller Fisher syndrome was good--recovery occurred after a mean time period of 10.1 weeks. Residual symptoms were present in 74 cases (33.2%), and a recidivism of the Miller Fisher syndrome was reported in seven patients. Eight patients died. Of six patients with autopsy findings, four exhibited central nervous system lesions.(ABSTRACT TRUNCATED AT 250 WORDS)