title: [Pathologic anatomy of the neurolipidoses].
abstract: 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.
mesh_terms: D000293:Adolescent; D000328:Adult; D001921:Brain / Q000378:metabolism / Q000473:pathology*; D001933:Brain Stem / Q000473:pathology; D002531:Cerebellum / Q000473:pathology; D002540:Cerebral Cortex / Q000473:pathology; D002648:Child; D006651:Histocytochemistry; D006801:Humans; D050356:Lipid Metabolism; D008064:Lipidoses / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology*; D008062:Lipofuscin / Q000378:metabolism; D008297:Male
chemical_list: D008062:Lipofuscin
vernacular_title: K patologicheskoĭ anatomii neĭrolipidozov.