Paraneoplastic Opsoclonus Myoclonus

The brain in patients with paraneoplastic opsoclonus-myoclonus rarely reveals Purkinje cell loss, changes in the dentate nucleus, or demyelination of cerebellar white matter. Less certain are examples without neuronal loss and scattered perivascular lymphocytes. y '591 '501

A subset of those with paraneoplastic opsoclonus-myoclonus produces high titers of an antibody, anti-Ri, that cross-reacts with an antigen in the nuclei of all central nervous system neurons and in tumor cells of affected patients. Anti-Ri antibody recognizes a protein of 55 kd as well as another of 80 kd. The distribution of these proteins is widespread. Although the exact pathogenetic anatomical location is unknown, anti-Ri-associated immunological responses appear to target the basis pontis and dorsal mesencephalon.'^

This syndrome occurs in association with childhood neuroblastoma and in adults in association with malignancy. y Commonly, these cancers are of breast, gynecologic, or small cell lung origin. The disorder produces saccadic eye movements in combination with myoclonus involving facial muscles, limbs, or trunk and truncal ataxia. Sporadically, opsoclonus-myoclonus occurs after viral infections or medication use.

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