Multiple system atrophy: Clinical, evolutive and histopathological characteristics of a series of cases.
Neurologia (Engl Ed)
; 38(9): 609-616, 2023.
Article
en En
| MEDLINE
| ID: mdl-37996211
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 ANDMETHODS:
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.Palabras clave
Texto completo:
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Base de datos:
MEDLINE
Asunto principal:
Enfermedades del Sistema Nervioso Autónomo
/
Atrofia de Múltiples Sistemas
/
Trastornos Parkinsonianos
Límite:
Humans
Idioma:
En
Revista:
Neurologia (Engl Ed)
Año:
2023
Tipo del documento:
Article