Opsoclonus-myoclonus syndrome: a clinicopathological confrontation.

Opsoclonus-myoclonus syndrome (OMS), a movement disorder characterised by chaotic eye movements and myoclonus, is a rare clinical entity. We present two cases of opsoclonus-myoclonus syndrome of paraneoplastic origin. In the first patient the syndrome was associated with a breast carcinoma and in the second patient with a non small cell lung carcinoma. However none of the commonly associated antibodies were found in these cases. From the neuropathological findings from the first patient we find arguments that support the current hypothesis on the pathophysiology of OMS namely a dysfunction in brainstem and cerebellum. We conclude that in adults with OMS one has to be very suspicious of a possible neoplastic origin of the syndrome. The antibodies associated with some cases of OMS are thought to play a role in the pathophysiology of the syndrome although the exact immunologic mechanism remains unknown. Research into the neuropathological substrate of OMS yields a broad range of abnormalities in brain stem and cerebellum. However none of these findings seem to be pathognomonic. As for the possible therapy of OMS, several immunomodulating strategies can be used with varying success. At present there is no established standard therapy.

Cytometrical and immunocytochemical investigation of brain nuclei in dementia.

Morphometrical investigation of brain nuclei in dementia of the Alzheimertyme and in other types of dementia has been done for the last 10 years. The N. Basalis of Meynert (Acetylcholine pathway) has been screened rather extensively by several groups. The L. Coeruleus (Nor-adrenergic pathway) also has been fairly well documented. The N. Raphe Dorsalis however-an important relay nucleus in the serotonergic pathway-was only studied cytometrically by a few groups. Other nuclei were not investigated systematically f.e. N. Accumbens; this nucleus might play an important role however in memory disturbances via Gabaergic influence on excitatory amino acids. This quantitative study focuses on those less well studied brain areas. A cytometrical analysis (cell counts-cell sizing) was performed on serial sections in clinically well-documented cases of different types of dementia: Alzheimer, Pick, Huntington, Jakob-Creutzfeldt. Results were compared with controls and for the N. Accumbens also with the values obtained in the brain of catatonic schizophrenics. The cell sizing and counting was done on cresylviolet sections, on modified Bielschowsky-stained sections and on Peroxidase-Anti-Peroxidase (P.A.P.) treated sections with a polycloncal antibody against Alzheimer Paired Helical Filaments (PHF). Our results show that the nerve cell degeneration in those nuclei is quite different in the diverse types of dementia, pointing to a disease specific physiopathology.

Congenital elevation of the scapula and Brown-Sequard syndrome.

We describe the case of a 14-year-old boy with a Sprengel deformity and a Brown-Séquard syndrome on the basis of a compression of the spinal cord at level C4. Although there are several publications on anomalies associated with Sprengel's deformity, this is, to our knowledge, the first description of a case with spinal cord compression.