Acute idiopathic polyneuritis with ophthalmoplegia, ataxia, and areflexia (Fisher's syndrome). A case report and a review of the literature

T. Suda, K. Nihei, S. Kamoshita

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Abstract

A 13-year-old girl with idiopathic polyneuritis with ophthalmoplegia, ataxia, and areflexia (Fisher's syndrome) is reported. Nine days before admission, the patient had a two-day history of mild fever, sore throat, and nasal discharge. Two days prior to admission, she noted double vision, dysarthria, unsteadiness on walking, and numbness of both hands and feet. On admission, she was husky and flushed, and showed bilateral ptosis. Neurological examination revealed a symmetrical external ophthalmoplegia. The eyes were immobile, and only medial movement was preserved. There was impaired vibration sense of the extremities. Ataxia seemed to be of a cerebellar type, because dysmetria and adiadochokinesis were also noted. Deep tendon reflexes were absent. The optic disc was normal, and visual activity was not impaired. Mental status was normal. Albuminocytologic dissociation in the cerebrospinal fluid was first noted on the 18th day after onset, and continued until the 74th day. EMG showed a neurogenic pattern in the muscles of lower extremities, but nerve conduction velocity was normal. Difficulties in swallowing necessitated infusion and tube feeding for 35 days. She was treated with prednisone 40 mg per day starting on the second hospital day. On the 35th day, truncal ataxia and dysarthria disappeared. On the 43rd day, there was no sensory paresis. Mild abducens nerve palsy and areflexia were persistent until the 100th day, but then, both gradually improved to complete recovery. No relapse has been noted for more than 2 years. The review of 60 cases of Fisher's syndrome in the literature revealed there are 22 cases under 15 years of age. As the differential diagnosis, Guillain-Barre syndrome, brain stem encephalitis, and acute cerebellar ataxia should be listed, all of which more or less resemble Fisher's syndrome with regard to pathogenesis and prognosis. Fisher's syndrome can be considered a clinical entity charcterized by total external ophthalmoplegia (45/57), severe ataxia (60/60), areflexia (60/60), and rare relapses (2/60). It is not a common disease, but a relatively high incidence in children should be stressed.

Original languageEnglish
Pages (from-to)321-327
Number of pages7
JournalNo To Hattatsu
Volume11
Issue number4
Publication statusPublished - 1979

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Miller Fisher Syndrome
Neuritis
Ophthalmoplegia
Ataxia
Cerebellar Ataxia
Dysarthria
Abducens Nerve Diseases
Recurrence
Abnormal Reflexes
Guillain-Barre Syndrome
Diplopia
Hypesthesia
Pharyngitis
Patient Admission
Neural Conduction
Optic Disk
Neurologic Examination
Enteral Nutrition
Paresis
Encephalitis

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health

Cite this

Acute idiopathic polyneuritis with ophthalmoplegia, ataxia, and areflexia (Fisher's syndrome). A case report and a review of the literature. / Suda, T.; Nihei, K.; Kamoshita, S.

In: No To Hattatsu, Vol. 11, No. 4, 1979, p. 321-327.

Research output: Contribution to journalArticle

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