Neurology India Medknow Publications on behalf of the Neurological Society of India ISSN: 0028-3886 EISSN: 1998-4022 Vol. 50, Num. 3, 2002, pp. 365-366

Neurology India, Vol. 50, No. 3, Sept, 2002, pp. 365-366

Short Report

Recurrent Miller Fisher Syndrome : A Case Report

S. Sitajayalakshmi, R. Borgohain, J. Mani, S. Mohandas

Correspondence to : Dr. S.Sitajayalakshmi, Department of Neurology, Nizam's Institute of Medical Sciences, Andhra, Pradesh - 500 082, India.

Code Number: ni02102

Summary

Miller fisher syndrome (MFS) is a variant of Guillain-Barre syndrome characterized by the triad of ophthalmoplegia, ataxia and areflexia. Recurrences are exceptional with MFS. A case with two episodes of MFS within four years is reported. He presented with findings of ophthalmoplegia, ataxia, areflexia, and oropharyngeal weakness and mild distal sensory impairment during both episodes. Electrophysiological findings showed reduced compound muscle action potentials and sensory nerve action potentials with no evidence of conduction blocks. Nerve biopsy showed segmental demyelination. MRI of brain was normal. He responded well to immunoglobulins during both episodes suggesting that immunomodulating drugs have a role in the treatment of MFS.

Key words: Miller fisher syndrome, Recurrence.

Introduction

The Miller fisher syndrome (MFS) is usually a monophasic illness of subacute onset, characterized by the triad of ophthalmoplegia, ataxia and reduced or absent tendon reflexes, with minimal, if any limb weakness,¹ often preceded by a viral or other illness. Though the presentation is alarming, it has a benign outcome with complete recovery within weeks. Though there are conflicting opinions regarding peripheral versus central nature of the site of major neuronal injury, the prevailing view is that it is peripheral and a variant of acute inflammatory polyneuropathy, Guillain-Barre syndrome (GBS), as supported by pathologic studies.^2,3 Anti GQ1b lgG antibodies have been found to be strongly associated with MFS.⁴ Though recurrences occur with GBS, recurrences of MFS are exceptional. We report an unusual case of recurrent MFS.

Case Report

A 39 year old principal of a college, presented to the neurology department in October 1995 with complaints of distal paresthesias of all four limbs, gait ataxia, nasal speech and diplopia on lateral gaze to either side of two weeks duration. He gave a preceding history of diarrhea for one day. There was no history of motor weakness or sphincter dysfunction. His family and social history were unremarkable. On examination he was alert, fully oriented and had a nasal twang. He had bilateral ptosis with frozen eye balls, absent doll's eye movements, dilated sluggishly reacting pupils, bilateral lower motor neuron type of facial palsy and reduced gag a reflexes. He had normal limb power with complete areflexia and flexor plantar responses. The most striking feature was marked limb and gait ataxia. He had distal symmetrical impairment of vibration. Meningeal signs were absent. Dysautonomia was noted with persistent tachycardia and hypertension. Routine blood tests were normal. Hepatitis B surface antigen and HIV (Elisa) were negative. Nerve conduction studies showed absent sensory nerve action potentials (SNAPs), delayed F waves and reduced motor conduction velocities in upper and lower limbs. Distal latencies and compound muscle action potentials (CMAPs) were within normal limits with no evidence of conduction block. He received a course of intravenous immunoglobulins. At the end of two weeks, autonomic dysfunction improved, external ocular movements returned, dysphagia improved and ataxia decreased. Follow up at two months showed normal ocular movements and no ataxia.

He was readmitted in February 1999 with similar complaints of distal paresthesias, diplopia, bulbar weakness, and limb and gait ataxia. There was no antecedent illness preceding this episode. Examination showed similar neurologic findings with bilateral total ophthalmoplegia, left lower motor neuron type of facial palsy and bilateral absent gag reflexes. He had normal motor power, generalized areflexia, limb and gait ataxia and minimal distal impairment of vibration with marked dysautonomia. Nerve conduction studies showed prolonged F wave latencies, reduced CMAPs and normal conduction velocities . SNAPs were not elicitable. There was no evidence of denervation. MRI brain was normal Sural nerve biopsy was suggestive of demyelinating type of neuropathy. He received intravenous immunoglobulins . He made good recovery in two weeks and at the end of two months he had no neurological deficit. Repeat conduction studies showed normal motor conductions with persistently absent sensory nerve action potentials.

Discussion

MFS by itself is a rare entity and is generally regarded as a variant of the GBS. Recurrence rate in GBS is about 1 to 6%.⁵ Recurrences of MFS are exceptional. There are few case reports of recurrent MFS.^6,7 Riche et al reported a case of a man with three recurrences of MFS within sixteen years.⁶ Dewarrat et al reported a patient who presented within ten years with three similar episodes of ophthalmoplegia, ataxia and areflexia associated with oropharyngeal weakness and signs of mild distal sensory neuropathy.⁷ Vinals et al described another case of recurrent MFS.⁸ It is widely accepted that serum anti-GQIb lgG is associated with ophthalmoplegia in patients with MFS. The anti GQIb antibody titers were found elevated during an episode, decreasing but not completely and vanishing two years later.^6,7 However we could not estimate the antibody titers in our patient. The wide spectrum of clinical features in the MFS has been emphasized in a review recently,⁹ which stated that bulbar presentation is uncommon. But our patient had bulbar weakness initially in both the episodes. The pattern of abnormalities in MFS on electrophysiologic studies was distinct from the usual features seen in the major form of acute inflammatory polyneuropathy, GBS¹⁰ The patterns seen were of an axonal neuropathy or a neuronopathy with predominant sensory nerve changes in the limbs with little electrophysiologic evidence of demyelination. Even our patient had absent SNAP'S and reduced CMAP's but had no evidence of conduction blocks. Thus, unlike GBS, the sensory nerves are more severely affected than motor nerves in the limbs and motor involvement is more prominent in the cranial nerves. But the electrophysiologic findings do not exclude demyelination as the primary process, as loss of myelin was noted in pathologic studies of MFS.^2,3 Our patient's sural nerve biopsy showed segmental demyelination. The mean recovery time in MFS is over ten weeks.⁹ The present case responded to immunoglobulins with total recovery by the end of eight weeks during both the episodes.

References

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2. Richter RB : The ataxic form of polyradiculoneuritis (Landry Guillain-Barre syndrome): clinical andpathologic observations. Neuropathol Exp Neurol 1962; 21 : 171-184.
3. Phillips MS, Stewart S, Anderson JR : Neuropathologrcal findings in Miller Fisher syndrome. J Neurol Neurosurg Psychiatry 1984; 47 : 492-495.
4. Chiba A, Kusonoki S, Shimizu T et al : Serum lgG antibody to ganglioside GQIb is a possible marker of Miller Fisher syndrome. Ann Neurol 1992; 31 : 677-679.
5. Eelco FM Wijdicks, Allan H.Ropper : Acute relapsing Guillain-Barre syndrome after long asymptomatic intervals. Arch Neurol 1990; 47 : 82-84.
6. Riche G, Caudie C, Vial C et al : Recurrent Miller Fisher syndrome and anti-GQIb antibodies. Rev Med Inteme 1998; 19 : 192-195.
7. Dewarrat A, Regli F, Steck A et al : Recurrent Miller Fisher syndrome. Significance of anti- GQIB antibodies. Schweiz Arch Neurol Psychiatr 1995; 146 : 180-184.
8. Vinals M, Anciones B, Cruz Martinez A et al : Recurrent Miller-Fisher syndrome associated with brachial neuritis. Neurologia 1989; 4 : 61-65.
9. Berlit P, Rakicky J : The Miller fisher syndrome: review of the literature. J Clin Neuro Ophthalmol 1992; 12 : 57-63.
10. Robin D. Fross, Jasper R Daube : Neuropathy in the Miller Fisher syndrome: Clinical and electrophysiologic findings. Neurology 1987; 37 : 1493-1498.

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