Multiple system atrophy: Clinical, evolutive and histopathological characteristics of a series of cases

IF 2.9 4区医学 Q2 CLINICAL NEUROLOGY Neurologia Pub Date : 2023-11-01 DOI:10.1016/j.nrl.2021.04.007

M. Carmona-Abellan , R. Del Pino , A. Murueta-Goyena , M. Acera , B. Tijero , K. Berganzo , I. Gabilondo , J.C. Gómez-Esteban

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引用次数: 5

Abstract

Background and 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

Unified MSA Rating Scale (UMSARS) score, structural and functional imaging and cardiovascular autonomic testing, are summarized since early stages of the disease.

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

A better description of MSA features with standardized test confirmed by means of neuropathological studies could help to increase sensitivity.

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多系统萎缩:一系列病例的临床、进化和组织病理学特征

背景与目的多系统萎缩是一种罕见且致命的神经退行性疾病，以自主神经功能障碍为特征，与帕金森病或小脑体征相关。病理特征是α -突触核蛋白聚集在少突胶质细胞中，形成胶质细胞质包涵体。临床上，它可能很难与其他帕金森病或共济失调症区分开来，特别是在疾病的早期阶段。在本病例系列中，我们旨在详细描述MSA患者的特征。材料与方法从疾病早期开始，对MSA统一评定量表(UMSARS)评分、结构和功能成像以及心血管自主功能测试进行总结。结果sumsars被证明是进行随访的有用的，纵向检查是分层不良结局风险的必要条件。神经病理学诊断显示帕金森和小脑亚型之间有重叠，有一些特点可以帮助与其他亚型区分开来。结论通过神经病理学研究，通过标准化的测试来更好地描述MSA的特征，有助于提高敏感性。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Neurologia 医学-临床神经学

CiteScore

5.90

自引率

2.60%

发文量

135

审稿时长

48 days

期刊介绍： Neurología es la revista oficial de la Sociedad Española de Neurología y publica, desde 1986 contribuciones científicas en el campo de la neurología clínica y experimental. Los contenidos de Neurología abarcan desde la neuroepidemiología, la clínica neurológica, la gestión y asistencia neurológica y la terapéutica, a la investigación básica en neurociencias aplicada a la neurología. Las áreas temáticas de la revistas incluyen la neurologia infantil, la neuropsicología, la neurorehabilitación y la neurogeriatría. Los artículos publicados en Neurología siguen un proceso de revisión por doble ciego a fin de que los trabajos sean seleccionados atendiendo a su calidad, originalidad e interés y así estén sometidos a un proceso de mejora. El formato de artículos incluye Editoriales, Originales, Revisiones y Cartas al Editor, Neurología es el vehículo de información científica de reconocida calidad en profesionales interesados en la neurología que utilizan el español, como demuestra su inclusión en los más prestigiosos y selectivos índices bibliográficos del mundo.