Abstract
A 13-year-old girl of idiopathic polyneuritis with ophthalmoplegia, ataxia, and areflexia (Fisher's syndrome) was reported. Nine days be-fore 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 extremities, but little motor wakness. Ataxia seemes to be of a cerebellar type, because dysmetry and adiadochokinesis were also noted. Deep tendon reflexes were absent. The optic disc was normal, and visual activity was not impai-red. Mental status was normal.
The albuminocytologic dissociation in the cer-ebrospinal 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 40mg per day starting on the second hospital day. On the 35 th day, truncal ataxia and dysarthria disappeared. On the 43 rd day, there was no sensory paresis. Mild abducens nerve palsy and areflexia were persistent until the 100 th day, but after then, both were 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, Guil-lain-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 clinical entity characterized 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.
