Multiple system atrophy: Clinical, evolutive and histopathological characteristics of a series of cases.
Afectación cerebelosa
Autonomic function
Cerebellar impairment
Función autonómica
Glial cytoplasmic inclusions
Inclusiones citoplasmáticas gliales
Oligodendrocitos
Oligodendrocytes
Parkinsonism
Parkinsonismo
Sinucleína
Synuclein
Journal
Neurologia
ISSN: 2173-5808
Titre abrégé: Neurologia (Engl Ed)
Pays: Spain
ID NLM: 101778590
Informations de publication
Date de publication:
26 May 2021
26 May 2021
Historique:
received:
02
12
2020
revised:
12
03
2021
accepted:
06
04
2021
entrez:
30
5
2021
pubmed:
31
5
2021
medline:
31
5
2021
Statut:
aheadofprint
Résumé
Multiple system atrophy is a rare and fatal neurodegenerative disorder, characterized by autonomic dysfunction in association with either parkinsonism or cerebellar signs. The pathologic hallmark is the presence of alpha-synuclein aggregates in oligodendrocytes, forming glial cytoplasmic inclusions. Clinically, it may be difficult to distinguish form other parkinsonisms or ataxias, particularly in the early stages of the disease. In this case series we aim to describe in detail the features of MSA patients. Unified MSA Rating Scale (UMSARS) score, structural and functional imaging and cardiovascular autonomic testing, are summarized since early stages of the disease. UMSARS proved to be useful to perform a follow-up being longitudinal examination essential to stratify risk of poor outcome. Neuropathological diagnosis showed an overlap between parkinsonian and cerebellar subtypes, with some peculiarities that could help to distinguish from other subtypes. A better description of MSA features with standardized test confirmed by means of neuropathological studies could help to increase sensitivity.
Sections du résumé
BACKGROUND AND OBJECTIVE
OBJECTIVE
Multiple system atrophy is a rare and fatal neurodegenerative disorder, characterized by autonomic dysfunction in association with either parkinsonism or cerebellar signs. The pathologic hallmark is the presence of alpha-synuclein aggregates in oligodendrocytes, forming glial cytoplasmic inclusions. Clinically, it may be difficult to distinguish form other parkinsonisms or ataxias, particularly in the early stages of the disease. In this case series we aim to describe in detail the features of MSA patients.
MATERIAL AND METHODS
METHODS
Unified MSA Rating Scale (UMSARS) score, structural and functional imaging and cardiovascular autonomic testing, are summarized since early stages of the disease.
RESULTS
RESULTS
UMSARS proved to be useful to perform a follow-up being longitudinal examination essential to stratify risk of poor outcome. Neuropathological diagnosis showed an overlap between parkinsonian and cerebellar subtypes, with some peculiarities that could help to distinguish from other subtypes.
CONCLUSION
CONCLUSIONS
A better description of MSA features with standardized test confirmed by means of neuropathological studies could help to increase sensitivity.
Identifiants
pubmed: 34052041
pii: S0213-4853(21)00073-6
doi: 10.1016/j.nrl.2021.04.007
pii:
doi:
Types de publication
Journal Article
Langues
eng
spa
Informations de copyright
Copyright © 2021 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.