J Korean Neurol Assoc.  2020 Aug;38(3):194-203. 10.17340/jkna.2020.3.5.

Comparison of Neuropathological Characteristics between Multiple System Atrophy Cerebellar Type and Parkinsonian Type

Affiliations
  • 1Department of Neurology and Medical Research Institute, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea
  • 2Department of Neurology, Pusan National University Yangsan Hospital, Pusan National University School of Medicine, Yangsan, Korea
  • 3Department of Psychiatry Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea
  • 4Department of Nuclear Medicine, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea
  • 5Department of Rehabilitation Medicine, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea
  • 6Department of Neurosurgerye, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea
  • 7Department of Pathology Pusan National University School of Medicine, Yangsan, Korea
  • 8Department of Anatomy, Pusan National University School of Medicine, Yangsan, Korea
  • 9Department of Forensic Medicine, Pusan National University School of Medicine, Yangsan, Korea

Abstract

Background
Multiple system atrophy (MSA) is a sporadic neurodegenerative disease characterized by various combinations of parkinsonism, cerebellar ataxia, autonomic dysfunction and pyramidal signs. Two clinical subtypes are recognized: MSA with predominant cerebellar ataxia (MSA-C) and MSA with predominant parkinsonism (MSA-P). The aim of this study was to compare pathological features between MSA-C and MSA-P.
Methods
Two autopsy confirmed cases with MSA were included from the Pusan National University Hospital Brain Bank. Case 1 had been clinically diagnosed as MSA-C and case 2 as MSA-P. The severity of neuronal loss and gliosis as well as the glial and neuronal cytoplasmic inclusions were semiquantitatively assessed in both striatonigral and olivopontocerebellar regions. Based on the grading system, pathological phenotypes of MSA were classified as striatonigral degeneration (SND) predominant (SND type), olivopontocerebellar degeneration (OPC) predominant (OPC type), or equivalent SND and OPC pathology (SND=OPC type).
Results
Both cases showed widespread and abundant α-synuclein positive glial cytoplasmic inclusions in association with neurodegenerative changes in striatonigral or olivopontocerebellar structures, leading to the primary pathological diagnosis of MSA. Primary age-related tauopathy was incidentally found but Lewy bodies were not in both cases. The pathological phenotypes of MSA were MSA-OPC type in case 1 and MSA-SND=OPC type in case 2.
Conclusions
Our data suggest that clinical phenotypes of MSA reflect the pathological characteristics.

Keyword

Multiple system atrophy-cerebellar type; Multiple system atrophy-parkinsonian type; α-synuclein; Glial cytoplasmic inclusion
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