NEURONAL LIPIDOSIS WITH VISCERAL INVOLVEMENT IN AN ADULT

A 35-year-old woman died after a neurological illness of 6 weeks duration characterized by cerebellar ataxia and involuntary movements. Histological examination proved this to be a case of neuronal lipidosis. Severe nerve cell loss and gliosis were found in the thalamus and cerebellar cortex. Although the spleen was not enlarged, groups of large granular macrophages were observed in the red pulp. Similar cells, in fewer numbers, were also present in the hepatic sinusoids. Histochemical reactions of the swollen neurons differed from those of the splenic macrophages. The material contained in neurons was histochemically similar to lipofuscin. In keeping with these data chemical analysis did not show an increase of ganglioside, and examination with the electron microscope disclosed dense lipofuscin bodies and no membranous cytoplasmic bodies. The material stored in the spleen was a glycolipid, whose staining reactions suggested to be a ganglioside. © 1969 Springer-Verlag.