Status marmoratus and Bielschowsky bodies. A report of two cases and review of the literature.
Intraneuronal inclusions, consisting of polyglucosan and having histochemical and ultrastructural features identical to Lafora body of familial myoclonic epilepsy (Unverricht-Lafora disease), have been found restricted to the lateral pallidum in five patients. Two of these patients were also found to have status marmoratus of the basal ganglia. These lateral pallidal inclusions have been named after Bielschowsky, their original discoverer. We report two additional patients with status marmoratus and Bielschowsky bodies and suggest that these two conditions are frequent concomitant phenomena arising independently from a common cause.
D000293:Adolescent; D001344:Autopsy; D001479:Basal Ganglia / Q000648:ultrastructure; D001921:Brain / Q000473:pathology*; D001925:Brain Damage, Chronic / Q000473:pathology*; D002547:Cerebral Palsy / Q000473:pathology*; D005260:Female; D005917:Globus Pallidus / Q000648:ultrastructure; D006801:Humans; D002479:Inclusion Bodies / Q000648:ultrastructure*; D008854:Microscopy, Electron; D008875:Middle Aged