Progressive myoclonic epilepsy (Unverricht type) with atypical Lafora bodies - Case report

F. C. Grahmann, R. C. Janzer, A. Hecker, M. Egli, P. C. Burger

Research output: Contribution to journalArticlepeer-review

Abstract

A patient with advanced progressive myoclonic epilepsy (Unverricht type) with Lafora bodies is presented. Although the clinical history and symptoms were classical, the regional distribution of the cerebral involvement differed from the classical picture: the corpora mamillaria, the nucleus subthalamicus, and the nucleus ruber, which are normally reported to be spared, contained multiple Lafora bodies, whereas the lateral geniculate body, which is usually involved, was intact. The number of inclusions per cell, up to 25, was extremely high and correlated with the marked cortical atrophy and the prolonged clinical course. Using electron microscopy, type I and type II Lafora bodies were found, but the latter lacked the typical filamentous ultrastructure in the peripheral zone. The lack of visceral Lafora bodies in this case suggests that liver, muscle, and skin biopsies, which are widely used for the diagnosis, may lead to false negative results and cannot always replace a stereotactic brain biopsy. The differential diagnosis of polyglucosan bodies is emphasized.

Original languageEnglish (US)
Pages (from-to)259-262
Number of pages4
JournalEuropean Archives of Psychiatry and Neurological Sciences
Volume235
Issue number4
DOIs
StatePublished - Mar 1 1986

Keywords

  • Lafora bodies
  • Polyglucosan bodies
  • Progressive myoclonic epilepsy

ASJC Scopus subject areas

  • Neuroscience(all)
  • Neuropsychology and Physiological Psychology
  • Psychiatry and Mental health

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