CEREBELLAR ATROPHY ASSOCIATED WITH ÉTAT MARBRÉ OF THE BASAL GANGLIA.

THIS paper records the occurrence in a 33-year-old idiot of bilateral etat marbre of caudate nucleus, putamen, and thalamus, together with a small atrophic cerebellum, atrophied inferior olives, and sclerosis of the nuclei pontis and the transverse pontine fibres. This combination of lesions is surprising since etat marbre of the corpus striatum is in the large majority of cases a phenomenon of infancy (Scholz et alia, 1938) and olivo-ponto-cerebellar atrophy is in general a disease of the sixth or fifth decades. A search of the literature concerning the former condition has failed to reveal its association with systematic cerebellar atrophy, although small foci of degeneration in the cerebellum may be found in cases of striatal etat marbre, especially when the cerebral cortex is involved and epilepsy present. On the other hand, it is well known that symptoms referable to disease of the extrapyramidal motor system may complicate olivo-pontocerebellar atrophy. Among several cases may be mentioned the examples recorded by Guillain and co-workers (1926) in which Parkinsonian rigidity and tremor were observed, and the case of Van Bogaert and Bertrand (1930) in which there was tremor without rigidity. Pathological confirmation of extrapyramidal lesions of a more conclusive character than the above-mentioned observers were able to furnish was provided by Scherer (1933), who in four cases of olivo-pontocerebellar atrophy demonstrated well-defined lesions in the substantia nigra and putamen. Ittat marbre was not observed. It would seem that the present case, although having affinities with these adult conditions, differs from them in several respects, notably in the early onset of the pathological process. Another peculiar feature is the absence of clinical signs suggestive either of extrapyramidal or cerebellar disease. For these reasons I have thought it worthy of record.