Evolving Cognitive Dysfunction in Children with Neurologically Stable Opsoclonus–Myoclonus Syndrome
| Autor: | Stephanie Satariano, En Lin Goh, Ming K. Lim, Geetha Anand, Kate Scarff |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
Pediatrics
medicine.medical_specialty Context (language use) Disease Article Disease course 03 medical and health sciences fluids and secretions 0302 clinical medicine cognitive dysfunction 030225 pediatrics mental disorders Opsoclonus myoclonus syndrome Cognitive development Medicine reproductive and urinary physiology neurodevelopment business.industry lcsh:RJ1-570 lcsh:Pediatrics Cognition Mild disease course medicine.disease Pediatrics Perinatology and Child Health opsoclonus–myoclonus syndrome business Off Treatment 030217 neurology & neurosurgery |
| Zdroj: | Children Volume 7 Issue 9 Children, Vol 7, Iss 103, p 103 (2020) |
| ISSN: | 2227-9067 |
| DOI: | 10.3390/children7090103 |
| Popis: | Cognitive and acquired neurodevelopmental deficits have been reported in children with opsoclonus&ndash 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&rsquo s ages at disease onset were between 17 and 35 months and they were followed up for 4&ndash 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&ndash 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&ndash 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|