Internuclear Ophthalmoplegia in Childhood (Guest Lecture)

This case was reported by Cogan DG and Wray SH. Internuclear ophthalmoplegia as an early sign of brain tumor. Neurology 1970; 20:629-633. The patient is Case 1. He is a 4 ½ year old boy whose parents noted that the right eye had been "drifting" for four months. On examination the only significant finding was complete and symmetrical paralysis of adduction on gaze to either side with coarse nystagmus of the abducting eye, Convergence was well performed. (Figures 1A, B and C (Pictures taken from moving film) (3)). Neurological examination was entirely normal. Clinical Diagnosis: Bilateral internuclear ophthalmoplegia (INO) The preliminary etiological diagnosis was multiple sclerosis, but the patient's very young age cast doubt on the diagnosis and he was admitted for full investigation. Skull x-rays: Revealed widening of the suture lines suggestive of chronically increased intracranial pressure (despite the absence of papilledema).. Pneumoencephalography failed to fill the ventricular system. Subsequent gas and pantopaque ventriculography disclosed symmetrical enlargement of the lateral ventricles, an expanded third ventricle, a dilatation of the anterior end of the fourth ventricle, and a block at the posterior end of the aqueduct - i.e. obstructive hydrocephalus. (Figures 2-4) Posterior fossa craniotomy revealed an inoperable tumor arising from the anterior floor of the fourth ventricle. A biopsy of the tumor was taken and a shunt inserted to bypass the aqueduct. Pathology: Histological sections were variously interpreted as consistent with a medulloblastoma or glioma. (Figure 5) The post-operative course was uneventful. Therapy: A ventriculo-peritoneal shunt was placed. The boy received radiation therapy with a total dose of 5,000 rads to the area of the mass. The spine was irradiated with 2,500 rads as a precautionary measure because of the statistical frequency of spinal seeding. Follow-up examination eleven months after completion of radiation therapy showed no change in the eye movements and only mild ataxia. Bilateral INO in childhood is rare. Cogan and I reported five other cases of internuclear ophthalmoplegia in childhood. (3) Age range 4 ½ - 17 years of age, 3 boys and 3 girls. The duration of symptoms was 9 months to 6 years. Headache, often provoked by exertion, was the presenting symptom in 4. Only one child had normal eye movements pre-operatively (Case 4). Three (Case 2, 3 and 5) had a unilateral INO and unilateral horizontal gaze palsy - a Fisher's one-and-a-half syndrome. One 17 year old boy (Case 6) with headache, increasing clumsiness and diplopia had a unilateral INO and skew deviation. The tumor was inoperable in all the children and four came to autopsy. A medulloblastoma was found in three and an infiltrating glioma in one.