Cognitive, adaptive behavior, academic, speech and language, and motor abilities were assessed in 19 children with opsoclonus-ataxia and neuroblastoma, followed at University of Southern California, Los Angeles, CA. Patients were examined twice with a minimum interval of 1 year. Age at onset of symptoms ranged from 11 to 31 months (median: 18 months). Age at the time of 1st and 2nd evaluations was a median of 3.1 and 6.4 years, respectively. Cognitive scores improved between testing sessions for the group as a whole, and a larger average gain in IQ/DQ was seen with younger children. Motor abilities improved in gross and fine motor function, but not at an age-appropriate rate. Behavioral and emotional functioning was overall stable and did not worsen significantly with age. Speech and language testing showed persistent deficits in the majority of patients, and all were receiving therapy. The necessity for treatment (steroids or immune-modulating medication) during the interval between evaluations did not influence change in test scores, but a history of relapse was strongly associated with deficits in cognition, academic achievement, and visual motor integration. Of 5 children without relapse, 4 had test scores in the normal range. Despite initial severity of symptoms, a minority of patients had an excellent outcome, with cognitive, academic, behavioral, and motor function within the normal range, and all immunotherapy had been discontinued. Fourteen with multiple relapses, and a chronic course associated with developmental sequelae, required prolonged therapy. [1]

COMMENT. Early and periodic neuropsychological testing is recommended in children diagnosed with opsoclonus-ataxia. Normal cognitive and behavioral development can be expected in the minority of patients without relapse, but a chronic and relapsing course is associated with developmental deficits. This study confirms earlier reports of a high incidence of delayed development, motor incoordination, behavioral, and speech deficits in children with opsoclonus-myoclonus syndrome (Ped Neur Briefs March & Aug 1995, Jan 1996). [2, 3]