[Pathologic anatomy of the neurolipidoses]

The authors give a description of 2 cases (sibs) with neurolipidosis of the group of amaurotic idiocy. There was an atypicity of the clinical and pathomophological picture: prolonged development of the disease (24 and 29 years, with an onset at 3-4 years), the absence of ocular changes, a massive lesion of the cortex in relative preservation of the subcortical areas, brain stem and cerebellum. The perspectiveness of a comprehensive clinico-morphological study is discussed in the interpretation of the genesis of different forms of neurolipidosis.