DEPOSITS IN CNS OCCURING WITH PROTRACTED COURSE (TYPE LUNDBORG) OF MYOCONUS BODIES DISEASE

Neuropathological findings of a case of a protracted course of myoclonus epilepsy, first described by Lundborg, are discussed. The findings of myoclonus bodies in a typical pattern, most of them lying in the subst. nigra, allows the diagnosis of myoclonus body disease. Most of myoclonus bodies were free in the neuropil, outside of the pericaryon of the nerve cells. The essential findings of this case are the homogenous deposits being present mainly in the neuropil of the gray matter. Cytoarchitectonic circumstances may be determinant factors of their distribution, for instance, layer IV c of area striata is favored. The multiform homogenous deposits give, different to myoclonic bodies, a reaction only with PAS and probably contain no acid groups. In infared spectrogramm only the hydroxyl groups are largely increased, whereas the rate of sulfate and phosphate groups are within normal. Finally the etiology of the homogenous polysaccharid-like deposits is discussed. The type Lundborg of myoclonic epilepsy is characterised by masses of homogenous deposits and proportional rare findings of myoclonic bodies. © 1969 Springer-Verlag.