ETIOLOGICAL FACTORS OF OPSOCLONUS MYOCLONUS ATAXIA SYNDROME: A SINGLE CENTER EXPERIENCE WITH EIGHT CHILDREN

VEYSEL GÖK, GÜLSÜM GÜMÜŞ, HABİBE SELVER DURMUŞ, EKREM ÜNAL, HAKAN GÜMÜŞ, MUSA KARAKÜKCÜ, AYŞE KAÇAR BAYRAM, HÜSEYİN PER

Trends in Pediatrics - 2022;3(4):120-125

Erciyes University Faculty of Medicine, Department of Pediatrics, Division of Pediatric Hematology and Oncology, Kayseri, Türkiye

 

Objective: Opsoclonus myoclonus ataxia syndrome (OMAS) is a rare neurological disorder characterized by acute/subacute onset multi-directional chaotic eye movements, accompanied by myoclonus and cerebellar ataxia; as well as sleep disturbance, cognitive dysfunction, and behavioral disturbance can be observed. METHODS: We examined the information of eight patients (four females, four males) who applied to the hospital with OMAS between 2013 and 2020 from the medical records of the patients. Results: The median age of onset of the initial symptoms was 17.5 months (8-30 months). The most common initial complaints were abnormal eye movement and gait unsteadiness, respectively. Paraneoplastic OMAS was observed in three patients (37.5%), whereas idiopathic and infection-related OMAS was detected in three, and two patients, respectively. Conclusion: We emphasize that all symptoms of OMAS may not occur simultaneously, therefore comprehensive systemic investigations, and close observation should be made in patients with suspected OMAS.