Neuropathology of Ataxias
The neuropathology of major types of cerebellar system degeneration is described in this chapter. Multiple system atrophy (MSA) is a major, non-hereditary spinocerebellar degeneration, characterized pathologically by the olivopontocerebellar atrophy, stri
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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2 Spinocerebellar Degeneration . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2 Sporadic Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2 Hereditary Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6 Conclusions and Future Directions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20
Abstract
The neuropathology of major types of cerebellar system degeneration is described in this chapter. Multiple system atrophy (MSA) is a major, non-hereditary spinocerebellar degeneration, characterized pathologically by the olivopontocerebellar atrophy, striatonigral degeneration, and autonomic nervous system degeneration in any combination. The occurrence of glial cytoplasmic inclusions in the oligodendrocytes is a pathologic hallmark of MSA, and involves multiple brain regions including the pontocerebellar tracts and internal capsules, as well as the other affected systems. Autosomal dominant spinocerebellar ataxias (SCAs) include several types of neurodegenerative diseases and are characterized pathologically by the combined degeneration of the cerebellum (cerebellar cortex, cerebellar nuclei) and other brain regions. Polyglutamine diseases are a major group of SCAs which is characterized by a significant correlation between CAG repeat lengths in the causative genes and disease severities. The formation of neuronal intranuclear inclusions is a characteristic feature of polyglutamine diseases. Inclusions are found in brain regions with a distribution specific to each disease. Spinocerebellar ataxia type 31, which is a common SCA in Japan, is M. Yamada (*) Division of Neuropathology, Department of Brain Disease Research, Shinshu University School of Medicine, Matsumoto-city, Nagano, Japan e-mail: [email protected] © Springer Nature Switzerland AG 2020 M. Manto et al. (eds.), Handbook of the Cerebellum and Cerebellar Disorders, https://doi.org/10.1007/978-3-319-97911-3_104-2
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M. Yamada
a recently established cerebellar degeneration which is characterized pathologically by a peculiar eosinophilic structure surrounding the remaining Purkinje cell bodies. Keywords
Spinocerebellar
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