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
|