Pathologic anatomy of the neurolipidoses
Kazakova, P.B.; Khokhrina, N.T.; Kalmykova, L.G.
Zhurnal Nevropatologii i Psikhiatrii Imeni S.S. Korsakova 78(7): 1095-1100
1978
ISSN/ISBN: 0044-4588 PMID: 685619 Document Number: 126725
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.