[Recent data on Lafora disease. Apropos of 17 cases].

Abstract

This study reviews 99 anatomically verified case of Lafora body disease (82 from the literature and 17 personal cases). The clinical symptoms of the disease are characterised by the triad; epilepsy, myoclonus and dementia. An anatomical and histochemical study has been undertaken and as a result emphasis is given to recent hypotheses that suggest there are similarities with Type IV glycogen storage disease (Andersen's disease) which, although clinically distinct, has the same enzyme defect.

• Authors: M C Mouren, J Roger
• Journal: Archives francaises de pediatrie (Arch Fr Pediatr) Vol. 36 Issue 3 Pg. 268-77 (Mar 1979) ISSN: 0003-9764 [Print] France
• Vernacular Title: Données récentes sur la maladie de Lafora. A propos de 17 observations.
• PMID: 114144 (Publication Type: English Abstract, Journal Article)

Topics

• Adolescent
• Brain (metabolism, pathology)
• Child
• Epilepsies, Myoclonic (metabolism, pathology)
• Female
• Histocytochemistry
• Humans
• Male