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