Summary
Intraneuronal inclusions, consisting of polyglucosan and having histochemical and ultrastructural features identical to Lafora body of familial myoclonic epilepsy (Unverrict-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.
Article PDF
Avoid common mistakes on your manuscript.
References
Bielschowsky M (1912) Beiträge zur Histopathologie der Ganglienzelle. J Psychol Neurol (Lpz) 18:513–521
Lafora GR (1911) Über das Vorkommen amyloider Körperchen im Innern der Ganglienzellen; zugleich ein Beitrag zum Studium der amyloiden Substanz im Nervensystem. Virchows Arch A [Pathol Anat] 205:295–303
Sakai M, Austin J, Witmer F, Trueb L (1970) Studies in myoclonus epilepsy (Lafora body form): II. Polyglucosans in the systemic deposits of myoclonus epilepsy and in corpora amylacea. Neurology 20:160–176
Vanderhaeghen JJ, Manil J, Franken L, Cappel R (1967) Deux observations de spasme de torsion accompagné de choréoathétose avec nombreux corps de Lafora dans la partie externe du globus pallidus. Acta Neuropathol (Berl) 9:45–52
de León G (1974) Bielschowsky bodies: Lafora-like inclusions associated with atrophy of the lateral pallidum. Acta Neuropathol (Berl) 30:183–188
Ule G, Volk B (1975) Torpide verlaufende Degeneration des äußeren Pallidungliedes mit Bielschowsky-Körperchen. Lichtund elektronenmikroskopische Befunde. J Neurol 210:191–198
Author information
Authors and Affiliations
Additional information
Supported in part by USPHS grants (nos. 07098 and NS 03356)
Rights and permissions
About this article
Cite this article
Adler, D., Horoupian, D.S., Towfighi, J. et al. Status marmoratus and Bielschowsky bodies. Acta Neuropathol 56, 75–77 (1982). https://doi.org/10.1007/BF00691185
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00691185