Childhood opsoclonus–myoclonus syndrome: A case series from Tunisia

Publication date: Available online 23 May 2017
Source:Brain and Development
Author(s): Nedia Ben Achour, Saloua Mrabet, Ibtihel Rebai, Ines Abid, Hanene Benrhouma, Hedia Klaa, Aida Rouissi, Ichraf Kraoua, Ilhem Ben Youssef Turki
IntroductionOpsoclonus myoclonus syndrome (OMS) is a rare immune-mediated disorder characterized by opsoclonus, myoclonus, ataxia and behavioral changes. The aim of our study was to investigate the epidemiology, clinical features, etiological aspects and outcome of OMS in Tunisian children.MethodsWe conducted a retrospective study over 11years (2005–2016) including all patients aged under 18years who were managed for newly diagnosed OMS in a tertiary care research centre for children with neurological symptoms. Epidemiological and clinical data were analyzed.ResultsFifteen patients were included. The male–female ratio was 7:8. Median age of onset was 4.32years (range: 14months–16years). Time to diagnosis ranged between 2days and 10months. Median follow-up period was 3.8years (range: 2–6years). Acute ataxia was the preponderant inaugural feature. Mean severity score was 9 (range: 3–14). In "Tumor group" (n=7), the main underlying malignancy was neuroblastoma identified in 5 patient. In "No tumor group" (n=8), parainfectious and idiopathic OMS were identified in 5 and 3 patients, respectively. All patients received immunomodulatory treatment. Complete recovery of OMS symptoms was obtained in 12 children. Comparing the "Tumor group" and the "No tumor group", there were no differences in age of onset, sex ratio, main presenting symptom, median OMS severity score or responsiveness to treatment. However, sleep and behavioral disturbances were more frequent in the "No tumor group" (p=0.04). Neurological sequelae were equally found in both groups.ConclusionAnnual incidence of OMS in Tunisia could be estimated as 0.6 patients in children per million per year. Diagnosis may be challenging especially when the triad is incomplete. Although behavioral disturbances seem to be more frequent in the "No tumor group", our study suggests that there is no specific features differentiating paraneoplastic OMS from non paraneoplastic OMS. Acute symptoms are responsive to immunomodulatory treatment but long term follow up can reveal neurological (mainly cognitive) sequelae.



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