An example of a fully developed clinical syndrome due to generalized diffuse Lewy deposits is diffuse Lewy body disease/dementia complex.

MULTIPLE SYSTEM ATROPHY

In multiple system atrophy (MSA), the unifying pathologic feature is the oligodendroglial cytoplasmic inclusion bodies (GCIs), which are present in striatonigral degeneration (SND), sporadic olivopontocerebellar atrophy (OPCA), and Shy-Drager syndrome, nosologic entities once considered unrelated disorders but now grouped under the rubric of MSA. The autonomic abnormalities that characterize Shy-Drager syndrome are found eventually in both disorders. Thus MSA is divided in two major groups, MSA-C (cerebellar) and MSA-P (parkinsonism). Other pathologic features include variable neuronal cell loss with gliosis in the putamen and, to a lesser degree, the pallidum, brainstem (particularly the basis pontis and inferior olive), cerebellum, intermediolateral columns of the spinal cord, and peripheral nerves. When OPCA/MSA-C is present, atrophy is predominant in the pons, cerebellum, and medullary olives. Tau and alpha-synuclein are the predominant components of GCIs. The MRI appearance is sometimes characteristic (see Plate 7-8).

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