Author/Editor		Špegel, Milan; Kopač, Štefan
Title		Sindrom opsoklonus-mioklonus-ataksija
Translated title		Opsoclonus-myoclonus-ataxia syndrome
Type		članek
Source		Zdrav Vestn
Vol. and No.		Letnik 71, št. 1
Publication year		2002
Volume		str. 31-3
Language		slo
Abstract		Background. Opsoclonus-myoclonus-ataxia syndrome (OMAS) is observed mostly in children under 3 years of age. Incidence is not known. The leading clinical sings are: opsoclonus, myoclonus and ataxia; only two signs may be present. The syndrome may follow an infectious disease usually of viral origin, but may be paraneoplastic, associated with a neuroblastoma. The course is often prolonged with recurrences but gradual and stepwise improvement. The majority of affected children are left with mental retardation, motor and/ orspeech disturbances. There are no known criteria to predict prognosis. Corticosteroid or corticotropin are recommended treatment regimes. Conclusions. The boy with OMAS is described. The diagnosis was made in retrospect. He was not treated, however inspite of this fac the has only mild mental retardation and tremorof the hands. He also developed epileptic fits which responded to antiepileptic treatment.
Summary		Izhodišča. Sindrom opsoklonus-mioklonus-ataksija (SOMA) se pojavlja predvsem pri otrocih pred tretjim letom starosti. Pogostost ni znana. Od glavnih znakov - opsoklonus, mioklonus, ataksija - sta lahko prisotna le dva. Pojavi se lahko po okužbah, zlasti virusnih, lahko pa je paraneoplastični pojar, povezan z nevroblastomom. Potek bolezni je dolgotrajen, z izboljšanji in poslabšanji, s postopnim izzvenevanjem znakov, toda pri večini otrok ostanejo umska manjvrednost, motorične in/ali govorne motnje. Nimamo kriterijev, po katerih bi bilo mogoče napovedati izid bolezni. Za zdravljenje se uporabljajo kortikosteroidi ali kortikotropin (ACTH). Zaključki. Opisan je deček s SOMA. Diagnoza je bila postavljena retrogradno. Ni bil zdravljen, kljub temu ima le blago umsko manjvrednost in tremor rok. Imel je tudi epileptične napade, ki so izzveneli po uvedbi protiepileptičnih zdravil.
Descriptors		OCULAR MOTILITY DISORDERS MYOCLONUS ATAXIA INFANT TREATMENT OUTCOME