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  Česky / Czech version Čes. a slov. Neurol. Neurochir., 67/100, 2004, No. 2, p. 137–140.
 
Opsoclonus-myoclonus syndrome: review and case report 
Klempíř J., Vymětalová P., Ulmanová L., Roth J. 

Neurologická klinika, 1. lékařská fakulta, Univerzita Karlova, Praha
 


Summary:

       The opsoclonus-myoclonus syndrome is a rare, acutely or subacutely developing neurological affection. It manifests in childhood as well as adulthood through fast, involuntary, unconjugated eye movements (opsoclonus), myoclonic twitches of the limbs and trunk in combination with other symptoms (especially ataxia, tremor, dysarthria and psychic changes). It can represent a manifestation of a neoplastic disease, arise on an infectious basis or, in a minority of cases, be idiopathic. The pathogenesis is not satisfactorily explained but the participation of immune response is undeniable.As an illustrative case, we present a 31-year old woman with insignificant family and medical history. The first symptoms of the disease manifested themselves when cleaning up a flooded house, through ten-day general weakness, nausea and vomiting without temperature. Additionally, head bobbing and gait instability appeared. The neurologic examination revealed opsoclonus, dysarthria, myoclonic twitches of upper and lower extremities of the resting-static type, more pronounced on the right. When sitting, twitches of the whole body could be seen, with maximum in the neck. Cerebrospinal fluid showed mild cell-protein association. Only in the serum, coxsackie virus type B antibodies were found. At the time of the first examination, IgM anti-borrelia antibodies were positive, but were not detected at the subsequent examinations. The patient was treated with a combination of antibiotics and corticoids. Clonazepam brought a marked symptomatic relief of the myoclonus. During three months, symptoms significantly subsided and treatment could be gradually stopped.

        Key words: opsoclonus-myoclonus syndrome, neuronal auto-antibodies
       

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