[Opsoclonus and myoclonus syndrome. Clinical and electronystagmography findings with course studies]

This review is based on the clinical experience with 7 patients and a total of 171 additional cases described in literature. Clinical features are presented of this "benign" cerebellar encephalopathy, which occurs preferably in association with viral infections, neuroblastomas or rarely carcinomas. Numerous descriptions of single cases stress a heterogeneous variety of other diseases which possibly may cause this syndrome. Special emphasis is put on the electronystagmographic documentation of eye movements with follow up of the spontaneous recovery over several years. The eye movement characteristics of opsoclonus allow for a clear differentiation from other forms of acquired ocular oscillations, such as ocular flutter, ocular dysmetria, square wave jerks, macro-saccadicoscillations, lightning eye movements, pendular nystagmus, obliquus superior myokymia and voluntary nystagmus. Differential diagnosis of the underlying diseases is particularly dependent on the age of onset. Histopathological findings as well as the hitherto suggested immunologic pathogenesis, therapeutic regimen and the prognosis are discussed critically on the basis of the literature.