Multiple system atrophy-parkinsonism with slow progression and prolonged survival: a diagnostic catch.
BACKGROUND
Multiple system atrophy (MSA) is a neurodegenerative disease leading to severe physical impairment, with a disease duration from onset to death of 6-9 years.


METHODS
The clinical and neuropathological features of 4 MSA cases with disease duration of 15 years or more were analyzed.


RESULTS
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.


CONCLUSIONS
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.
National Hospital for Neurology and Neurosurgery, Queen Square, and the Reta Lila Weston Institute of Neurological Studies, UCL Institute of Neurology, London, United Kingdom.
Igor N
IN
Petrovic
Helen
H
Ling
Yasmine
Y
Asi
Zeshan
Z
Ahmed
Prashanth L
PL
Kukkle
Lili-Naz
LN
Hazrati
Anthony E
AE
Lang
Tamas
T
Revesz
Janice L
JL
Holton
Andrew J
AJ
Lees
D000203:Activities of Daily Living; D000328:Adult; D017668:Age of Onset; D000368:Aged; D001344:Autopsy; D001921:Brain / Q000473:pathology; D003072:Cognition Disorders / Q000150:complications / Q000523:psychology; D003951:Diagnostic Errors; D018450:Disease Progression; D005260:Female; D006212:Hallucinations / Q000209:etiology / Q000523:psychology; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000175:diagnosis / Q000473:pathology*; D009483:Neuropsychological Tests