Status Marmoratus of the Thalamus and Striatum

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Abstract
Oppen-heim, Freund and Vogt distinguished etat marbre as an entity and considered it as a "pure striatal syndrome" characterized by athetoid activity, spasmodic laughing and crying, variable degrees of paresis and intellectual deterioration. Subsequent studies have not substantiated this contention. A case of athetosis and dystonia associated with status marmoratus of the thalamus and striatum was reported in detail. Birth was traumatic, instrumental, premature and complicated by difficulty in resuscitation. Death followed an attempt to ameloriate the condition surgically. Postmortem examination of the neuraxis revealed classic status marmoratus of the thalamus and striatum, being most marked in the thalamus. No conspicuous cellular loss or gliosis was apparent in these structures. This case was considered unusual because the thalamus was involved by the mar-moratic process to a greater extent than the striatum and athetosis and dystonia were present. Theories concerning the pathogenesis of this interesting condition were discussed.

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