Neuropathology
MSA is pathologically characterised by selective vulnerability of the striatonigral and olivopontocerebellar systems which may show varying degrees of neurodegeneration and underlie the stratification of this heterogeneous disorder into MSA-P and MSA-C cl
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Neuropathology Kurt Jellinger
Contents 3.1 Macroscopy ....................................................................................................................... 3.2 Histopathology .................................................................................................................. 3.2.1 Distribution of Pathology....................................................................................... 3.2.2 Involvement of the Autonomic Nervous System ................................................... 3.2.3 Involvement of the Peripheral Nervous System .................................................... 3.2.4 White Matter Lesions ............................................................................................ 3.3 Inclusion Pathology ........................................................................................................... 3.3.1 Distribution of Inclusions ...................................................................................... 3.3.2 Composition of GCIs ............................................................................................. 3.3.3 Ultrastructure of Cellular Inclusions ..................................................................... 3.3.4 Glial Reactions....................................................................................................... 3.4 Staging Schemes for MSA ................................................................................................ 3.5 Concomitant Pathologies ................................................................................................... References ..................................................................................................................................
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Abstract
MSA is pathologically characterised by selective vulnerability of the striatonigral and olivopontocerebellar systems which may show varying degrees of neurodegeneration and underlie the stratification of this heterogeneous disorder into MSA-P and MSA-C clinical variants, which correlate to the morphological phenotypes of striatonigral degeneration (SND) and olivopontocerebellar atrophy (OPCA). The lesions are not limited to these most consistently and severely affected systems, but may involve many other parts of the central, peripheral and autonomic nervous systems (Wakabayashi et al. 2010; Jellinger 2011b; Ubhi et al. 2011), underpinning the multisystem character of MSA.
K. Jellinger, MD Institute of Clinical Neurobiology, Medical University Vienna, Kenyongasse 18, Vienna 1070, Austria e-mail: [email protected] G.K. Wenning, A. Fanciulli (eds.), Multiple System Atrophy, DOI 10.1007/978-3-7091-0687-7_3, © Springer-Verlag Wien 2014
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K. Jellinger
Fig. 3.1 Coronal section of MSA brain showing atrophy and greyish-blue discoloration of both putamina (MSA multiple system atrophy) (Reproduced from Wenning and Jellinger (2005a), with the permission from Springer-Verlag GmbH)
MSA is pathologically characterised by selectiv
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