Neuronal ceroid lipofuscinosis: clinical and histochemical observations in 2 cases

Two cases of neuronal ceroid lipofuscinosis are presented with detailed accounts of the clinical, histopathological and histochemical changes in the brain. In one of them, neurochemical analysis of the brain was found to be essentially normal. Despite the pathological and histochemical similarity in these two cases, the clinical forms were quite distinct. The clinical patterns are attributed to differences in the topography of neuronal involvement. The pathogenesis of this extremely rare form of neurolipidosis is discussed with reference to recent literature.