The nosological position of Fisher's syndrome (Ophthalmoplegia, Ataxia and Areflexia)

Abstract

Fisher's syndrome was studied in six patients, one of whom was an 11-year-old girl, and the nosological position of this neuro-ophthalmological disorder was reviewed. The clinical features of the ophthalmoplegia in these cases were reported as secondary to a transient inflammatory lesion in the mid-brain; this was demonstrated in one of the cases by an enhancing area on CT scan. This mid-brain localization could explain not only the classical Fisher's triad but also many variations of the syndrome. The overlap of our cases of Fisher's syndrome with so-called brain-stem encephalitis of Bickerstaff has been emphasized. Many cases of Fisher's syndrome could therefore correspond to an inflammatory process at brain-stem level with or without nerve-root involvement. Clinical and neurophysiological findings show, however, that there is peripheral damage in some cases, so that a neutral attitude needs to be maintained on the nosological position of Fisher's syndrome.

Miller Fisher Syndrome: A Hospital-Based Retrospective Study

“…Some published case reports describe MFS cases associated with evidence of brainstem or cerebellar lesions [9, 13, 34, 35, 36, 37, 38, 39], while others revealed normal findings [40, 41, 42]. In our study, the results of those patients who had brain CT or MRI examinations with or without contrast enhancement were all within normal limits.…”

“…The results have been controversial. Although some studies revealed no evidence of affection of the peripheral nerves [9, 11, 12, 13], most studies revealed peripheral nerve involvement such as abnormalities in distal motor latencies, motor conduction velocities, sensory conduction velocities, F wave latencies and H reflexes [19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34]. Three out of our 10 patients had abnormal electrophysiological findings.…”

“…However, there have been controversies since the introduction of 8 cases with a similar syndrome by Bickerstaff [8]in 1957, which was entitled ‘brainstem encephalitis’. Some authors believed that the syndrome of AAO could be explained by a central origin [9, 10, 11, 12, 13, 14]. Whether MFS is a peripheral or central nervous system disorder cannot be determined because it is uniformly benign with consequently few autopsy data.…”

Detection of a Central Nervous System Lesion in a Patient with Fisher's Syndrome.

“…Although CNSlesions have been suggested in addition to peripheral lesions based on the clinical symptoms of Fisher's syndrome (12,13), only two cases (14,15) showed a CNS lesion on CT, and one on MRI (16). Electrophysiologically, only a few cases (17)(18)(19) showed prolonged III-V inter-peak latency by BAEP and N13-N19 inter-peak latency by SEP. Prolongation of the inter-peak latencies, waves III-V of BAEPs and N13-N20 of SEPs indicated a CNSlesion in the present patient (20).…”

Fisher’s Syndrome Associated with <i>Campylobacter jejuni </i>Infection

“…Fisher's syndrome -ophthalmoplegia, ataxia and areflexia -is generally considered a variant of GuillainBarre's syndrome [ 1 ]. We describe a case of Fisher's syn drome with a positive blood and spinal fluid serology for Campylobacter jejuni.…”