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We previously reported (Europ, Neurol., 3, 347-364, 1970) on two clinical observations of progressive myoclonus epilepsy in the same family. Now, we complete the clinical study showing the neuropathological findings concerning one of the two patients who died at the age of twenty. Degenerative neuronal alterations were found involving the olivo-cerebello-rubral system and, in a minor degree, the brain stem and thalamic structures. No Lafora bodies were found. These pathological features make it possible to suggest a differentiation from a first group of progressive myoclonus epilepsy (Unverricht-Lundborg) and another group (with epileptic manifestations) of dyssynergia cerebellaris myoclonica (Ramsay Hunt). We agree with the suggestion of other authors that it is difficult to establish precise differentiations between these groups at neuropathological level. We think however that the distinction is still possible on the basis of the clinical picture. Our observations cupport the hypothesis that our patient is an "abiotrophic" case of Unverricth-Lundborg syndrome, as we previously had suggested on clinical and especially neurophysiological findings.

A case of progressive myoclonic epilepsy (P.M.E.) is described. The clinical picture consisted of epileptic seizures, myoclonus and slight mental deterioration associated with a severe progressive cerebellar syndrome. The disease had a course of almost 20 years. Histological studies of the C.N.S. showed severe loss of Purkinje cells, sligth regressive changes in both dentate and olivary nuclei, nerve cells atrophy of anterior horn motoneurons, degeneration of Goll's and Burdach's spino-olivary and anterior spino-cerebellar tracts. Features of cellular lipidosis and/or neuronal amiloid inclusions were not seen. The case was therefore classified in the group of degenerative P.M.E. Its peculiar pathologic aspects consisted of slight but diffuse brain stem regressive changes associated with systemic degeneration involving the spino-cerebellar pathways. The clinical features of our patient emphasize the problem of differential diagnosis between. P.M.E. and D.C.M. thought of by French authors to be an autonomous entity both clinically )severe cerebellar syndrome, intentional myoclonus, absence or late appearance of epilepsy, slight or absent mental deterioration) and anatomically (primary atrophy of the dentate system). However there are many cases, similar to the one reported, which have a clinical course suggesting a diagnosis of D.C.M., but which differ neuropathologically from Hunt's syndrome because of the absence of primary atrophy of the dentate system. These borderline cases give supporting evidence to the concept of continuity between classical P.M.E. and D.C.M., as well as to the authors' opinion that 1unt's syndrome must be classified as a varient of degenerative P.M.E.