[Clinical diagnosis and therapy of opsoclonus-myoclonus syndrome].
OBJECTIVE
Clinical manifestations of opsoclonus-myoclonus syndrome (OMS) in children were summarized and analyzed and the clinical features and therapeutic approaches to OMS were investigated in order to improve its diagnosis and management.


METHODS
Clinical information on features and management of 6 cases with OMS inpatients being followed up from 2006 to 2007 were collected and analyzed.


RESULTS
Among the 6 cases, one was male and the other five were female. The age at the onset ranged from 12 to 26 months (average 21.0 months). Four of them had history of prior infection. The symptoms were opsoclonus, myoclonus, ataxia, sleep disturbances and behavioural problems in the 6 cases. Urinary DL-3-methoxy-4-hydroxy-acid amygdalin (VMA) was positive in 1 case. Abdominal B-mode ultrasound showed a mild hepatomegaly in 4 cases. The EEG showed abnormal findings such as slow background activity in 3 cases. Epileptiform discharges were found in none of the patients. MRI showed a high signal in medial longitudinal fasciculus and tectospinal tract on T2-weighted image in 1 case. Computerized tomography found L3-4 arachnoid cysts in 1 case and was normal in the others. Adrenocorticotropic hormone (ACTH) was given to all these patients and was effective in all during acute stage. In 2 cases the disease relapsed during follow-up stage.


CONCLUSIONS
OMS is a rare neurological condition with opsoclonus, myoclonus, ataxia, sleep disturbances and behavioral problems, which might relapse easily and is associated with adverse neurological outcome. ACTH therapy is effective in management of OMS.
Xiong
Hui
H
Department of Pediatrics, First Hospital, Peking University, Beijing, China.
Peng
Jing
J
Zhang
Yue-hua
YH
Bao
Xin-hua
XH
Jiang
Yu-wu
YW
Qin
Jiong
J
D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D053578:Opsoclonus-Myoclonus Syndrome / Q000175:diagnosis* / Q000628:therapy*; D011379:Prognosis; D012008:Recurrence; D016896:Treatment Outcome
D000324:Adrenocorticotropic Hormone