다계통위축증 소뇌형과 파킨슨형의 부검 소견 비교
Comparison of Neuropathological Characteristics between Multiple System Atrophy Cerebellar Type and Parkinsonian Type
김은주, 이숙민, 장성환, 이명준, 이재혁, 신진홍, 이영민, 박경준, 정나연, 윤진아, 고준경, 이재민, 이강윤, 황청수, 안재우, 성석, 최경운, 허기영,
소속 상세정보
김은주 ( Kim Eun-Joo )
Pusan National University School of Medicine Pusan National University Hospital Department of Neurology
이숙민 ( Lee Suk-Min )
Pusan National University School of Medicine Pusan National University Hospital Department of Neurology
장성환 ( Jang Sung-Hwan )
Pusan National University School of Medicine Pusan National University Hospital Department of Neurology
이명준 ( Lee Myung-Jun )
Pusan National University School of Medicine Pusan National University Hospital Department of Neurology
이재혁 ( Lee Jae-Hyeok )
Pusan National University School of Medicine Pusan National University Yangsan Hospital Department of Neurology
신진홍 ( Shin Jin-Hong )
Pusan National University School of Medicine Pusan National University Yangsan Hospital Department of Neurology
이영민 ( Lee Young-Min )
Pusan National University School of Medicine Pusan National University Hospital Department of Psychiatry
박경준 ( Pak Kyoung-June )
Pusan National University School of Medicine Pusan National University Hospital Department of Nuclear Medicine
정나연 ( Jung Na-Yeon )
Pusan National University School of Medicine Pusan National University Yangsan Hospital Department of Neurology
윤진아 ( Yoon Jin-A )
Pusan National University School of Medicine Pusan National University Hospital Department of Rehabilitation Medicine
고준경 ( Ko Jun-Kyeung )
Pusan National University School of Medicine Pusan National University Hospital Department of Neurosurgery
이재민 ( Lee Jae-Meen )
Pusan National University School of Medicine Pusan National University Hospital Department of Neurosurgery
이강윤 ( Lee Kang-Yoon )
Pusan National University School of Medicine Pusan National University Hospital Department of Psychiatry
황청수 ( Hwang Chung-Su )
Pusan National University School of Medicine Pusan National University Hospital Department of Pathology
안재우 ( Ahn Jae-Woo )
Pusan National University School of Medicine Department of Forensic Medicine
성석 ( Sung Suk )
Pusan National University School of Dentistry Department of Oral Anatomy
최경운 ( Choi Kyung-Un )
Pusan National University School of Medicine Department of Pathology
허기영 ( Huh Gi-Yeong )
Pusan National University School of Medicine Department of Forensic Medicine
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.
키워드
Multiple system atrophy-cerebellar type; Multiple system atrophy-parkinsonian type; α-synuclein; Glial cytoplasmic inclusion
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