A prospective study of the presentation and management of dancing eye syndrome/opsoclonus-myoclonus syndrome in the United Kingdom.
The incidence, mode of presentation and management of Dancing Eye Syndrome/Opsoclonus-Myoclonus Syndrome (DES/OMS) was prospectively evaluated in 20 United Kingdom (UK) paediatric neurology centres by questionnaire over a 24-month period between 2003 and 2005. Nineteen children were notified, giving an incidence of 0.18 cases per million total population per year. Mean age at presentation was 18 months (range 3-42 months). Fifteen families consented to participate in the study. Atypical features were present in 6/15 cases including very delayed presentation of opsoclonus, dysphagia, and rapid spontaneous improvement without treatment. Only 4/15 cases were associated with neuroblastoma (NB) but current practice in excluding this is diverse and a standardised approach is suggested.
Pang
K Ki
KK
Department of Paediatrics, Newcastle General Hospital, Newcastle upon Tyne, UK.
de Sousa
Carlos
C
Lang
Bethan
B
Pike
Michael G
MG
D000008:Abdominal Neoplasms / Q000453:epidemiology / Q000473:pathology; D001932:Brain Neoplasms / Q000453:epidemiology / Q000473:pathology; D002395:Catecholamines / Q000652:urine; D002675:Child, Preschool; D003680:Deglutition Disorders / Q000453:epidemiology; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009447:Neuroblastoma / Q000453:epidemiology / Q000473:pathology; D053578:Opsoclonus-Myoclonus Syndrome / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy*; D015995:Prevalence; D011446:Prospective Studies; D012075:Remission, Spontaneous
D002395:Catecholamines