Multiple system atrophy–parkinsonism with slow progression and prolonged survival: A diagnostic catch
2012; Wiley; Volume: 27; Issue: 9 Linguagem: Inglês
10.1002/mds.25115
ISSN1531-8257
AutoresIgor Petrović, Helen Ling, Yasmine T. Asi, Zeshan Ahmed, Prashanth Lingappa Kukkle, Lili‐Naz Hazrati, Anthony E. Lang, Tamás Révész, Janice L. Holton, Andrew J. Lees,
Tópico(s)Genetic Neurodegenerative Diseases
ResumoMultiple system atrophy (MSA) is a neurodegenerative disease leading to severe physical impairment, with a disease duration from onset to death of 6-9 years.The clinical and neuropathological features of 4 MSA cases with disease duration of 15 years or more were analyzed.All patients presented with parkinsonism and had a mean latency of 11 years before the development of dysautonomia. Mean duration from onset of first symptoms to anterocollis, inspiratory stridor, and dysphagia was 9 years. Despite the limited levodopa response, all patients developed levodopa-induced dyskinesia.Late appearance of dysautonomia is a favorable prognostic factor in MSA-P. Greater awareness of this uncommon "benign" subgroup of MSA will improve diagnostic accuracy and help to more accurately inform treatment options.
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