Статья

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

Eunjoo KimDepartment of Neurology and Medical Research Institute, Pusan National University Hospital, Pusan National University School of Medicine, Busan, KoreaSuk Min LeeDepartment of Neurology and Medical Research Institute , Pusan National University Hospital , Pusan National University School of Medicine , Busan , KoreaSung-Hwan JangDepartment of Neurology and Medical Research Institute , Pusan National University Hospital , Pusan National University School of Medicine , Busan , KoreaMyung Jun LeeDepartment of Neurology and Medical Research Institute, Pusan National University Hospital, Pusan National University School of Medicine, Busan, KoreaJae‐Hyeok LeeDepartments of Psychiatry b ,Jin‐Hong ShinDepartments of Psychiatry b , Nuclear Medicine c , Rehabilitation Medicine d , and Neurosurgery e , Pusan National University Hospital, Pusan National University School of Medicine, Busan, KoreaYoung Min LeeDepartment of Forensic Medicine, Pusan National University School of Medicine and Medical Research Institute, 49 Busandaehak-ro, Mulgeum-eup, Yangsan 50612, KoreaKyoungjune PakDepartment of Neurology and Medical Research Institute , Pusan National University Hospital , Pusan National University School of Medicine , Busan , KoreaNa‐Yeon JungDepartments of Pathology f , Anatomy g , and Forensic Medicine h , Pusan National University School of Medicine, Yangsan, KoreaJin A YoonDepartment of Forensic Medicine, Pusan National University School of Medicine and Medical Research Institute, 49 Busandaehak-ro, Mulgeum-eup, Yangsan 50612, KoreaJun Kyeung KoDepartment of Forensic Medicine, Pusan National University School of Medicine and Medical Research Institute, 49 Busandaehak-ro, Mulgeum-eup, Yangsan 50612, KoreaJae Meen LeeDepartment of Neurology, Pusan National University Yangsan Hospital, Pusan National University School of Medicine, Yangsan, KoreaKang‐Yoon LeeDepartment of Forensic Medicine, Pusan National University School of Medicine and Medical Research Institute, 49 Busandaehak-ro, Mulgeum-eup, Yangsan 50612, KoreaChungsu HwangDepartment of Forensic Medicine, Pusan National University School of Medicine and Medical Research Institute, 49 Busandaehak-ro, Mulgeum-eup, Yangsan 50612, KoreaJae Woo AhnDepartment of Neurology and Medical Research Institute, Pusan National University Hospital, Pusan National University School of Medicine, Busan, KoreaSuk SungDepartment of Neurology and Medical Research Institute, Pusan National University Hospital, Pusan National University School of Medicine, Busan, KoreaKyung-Un ChoiDepartment of Neurology and Medical Research Institute, Pusan National University Hospital, Pusan National University School of Medicine, Busan, KoreaGi Yeong HuhDepartment of Forensic Medicine, Pusan National University School of Medicine and Medical Research Institute, 49 Busandaehak-ro, Mulgeum-eup, Yangsan 50612, Korea

2020en

ABI

Аннотация

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.

Перевод пока недоступен

Идентификаторы

DOI: 10.17340/jkna.2020.3.5

Цитирования и источники

Цитирований: 0Использованных источников: 19