A persisting disability was found at long-term follow-up in 88% of 54 patients with dancing eye syndrome (DES) reported from the Hospital for Sick Children, Great Ormond Street, London. The disability was severe in 30 (62%), 34 (69%) had a motor disability, 29 (59%) had learning disabilities, and 23 (47%) had a combined motor and learning disability. Neurologic sequelae were independent of the severity of symptoms of the illness and age at onset. A malignancy was diagnosed in only 4: neuroblastoma in 3 and acute lymphoblastic leukemia in 1. An intercurrent illness, usually respiratory, preceded onset of DES in one half the cases. Presenting symptoms included ataxia, abnormal head and limb movements, and opsoclonus. Emotional outbursts of temper and affection were later features. A favorable initial response to corticotrophin or predisolone, observed in all patients, was not predictive of a good neurological prognosis. [1]
COMMENT. Dancing eye syndrome (opsoclonus-myoclonus, infantile myoclonic encephalopathy) presents in infancy or early childhood (93% under 3 years) and neurologic sequelae may persist into adult life. Speech deficits, described as occasional in the above series, were more prominent in patient series reported from the Children’s Memorial Hospital, Chicago, and the Children’s National Medical Center, Washington, DC. (see Ped Neur Briefs Jan 1996;10:2).