[Skip to Content]
Access to paid content on this site is currently suspended due to excessive activity being detected from your IP address 50.16.107.222. Please contact the publisher to request reinstatement.
[Skip to Content Landing]
Article
February 1, 1965

LAFORA'S DISEASE

JAMA. 1965;191(5):408. doi:10.1001/jama.1965.03080050054018
Abstract

Pursuit of the etiology of some obscure disorders of the nervous system has led to the discovery of previously unknown systemic diseases, in which the neural disturbance has been the primary or only manifestation of the general illness. Progresssive familial myoclonic epilepsy, described in 1881 by Unverricht, now seems to be one of these conditions.

In 1911, Lafora described amyloid intraneuronal inclusion bodies as the unique pathologic feature of Unverricht's syndrome. Subsequently, other neural alterations were reported in patients with Unverricht's syndrome. Abiotrophy, lipid disturbances, and degenerative processes were evoked as etiologic factors. Then, in 1955, Harriman and Millar1 found material similar to the Lafora bodies in cardiac muscle and hepatic cells and identified the intracellular substance as an acid mucopolysaccharide. They emphasized the unity of this form of Unverricht's syndrome, both clinically and pathologically.

In the February issue of the Archives of Neurology, Schwarz and Yanoff2 describe inclusion bodies

First Page Preview View Large
First page PDF preview
First page PDF preview
×