Evolving Cognitive Dysfunction in Children with Neurologically Stable Opsoclonus–Myoclonus Syndrome

Abstract

Cognitive and acquired neurodevelopmental deficits have been reported in children with opsoclonus–myoclonus syndrome (OMS) and are known to be associated with more severe and relapsing disease course. However, there is a paucity of data regarding cognitive dysfunction in children with stable neurological disease. We report three children with OMS and evolving cognitive dysfunction in the context of a mild disease course. The children’s ages at disease onset were between 17 and 35 months and they were followed up for 4–10 years. Neuroblastoma was identified in one child. OMS severity scores ranged between 8 and 12/15 at presentation. They underwent immunotherapy and all were in remission by 7 months (range 4–13 months), with treatment maintained for 1 year. One child remained relapse-free, while two others had one clinical relapse each and were immunotherapy-responsive again. In all cases, evolving cognitive dysfunction was reported despite being in remission and stable off treatment for a median of 20 months (range of 12–31 months; two OMS scores of 0/15 and one of 2/15). In children with OMS who have completed treatment and have made full or near full neurological recovery, concerns remain regarding long-term outcome in terms of future learning and cognitive development.

Clinical Analysis of Pediatric Opsoclonus-Myoclonus Syndrome in One of the National Children's Medical Center in China

“…We found that the DQ of patients at the last follow-up was 77.6 ± 9.8. And three OMS patients in En Lin et al's study had evolving cognitive dysfunction despite being in remission and been followed up for a long time (ranged from 5 to 10 years) (38). In our study, we found the DQ of patients at the last follow-up was not correlated with the age at onset, the OMS severity score onset or last follow-up, the number of relapses, the duration of follow-up.…”

Diagnosis and Management of Opsoclonus-Myoclonus-Ataxia Syndrome in Children

“…One series of 3 patients described progressive cognitive dysfunction despite a stable clinical course following treatment. 59 …”

“…One series of 3 patients described progressive cognitive dysfunction despite a stable clinical course following treatment. 59 Given the significant symptoms and impact on development, treatment is targeted at inducing full remission, tolerating no symptoms, and acting promptly to avoid or mitigate relapses. Ongoing physical, occupational, and speech therapy should be recommended for all patients with OMAS, although many children will not tolerate therapies early in the course due to irritability.…”

Review of Opsoclonus-Myoclonus Ataxia Syndrome in Pediatric Patients

“…They also reported that the risk factors for neurological sequelae were the female sex, a residual tumor, and a non-adrenal-gland-located tumor [ 90 ]. Goh et al followed three children with OMAS from 4 to 10 years of age and reported that significant deficits in attention, processing speed, visuospatial skills, and language were observed in the long term [ 91 ]. In 2011, Brunklaus et al concluded, from a retrospective review of 101 patients with OMAS over a 53-year period, that 60% of the patients had residual motor problems, while 66% had speech abnormalities, 51% had learning disabilities, and 46% had behavioral problems.…”