Abstract
PurposeThe initial symptoms of multiple system atrophy (MSA) and, in particular, early autonomic symptoms, have received less attention than motor symptoms. Whereas pathognomonic motor signs are essential to diagnostic specificity, early symptoms important to recognition of a neurodegenerative disorder may be less apparent or diagnostically ambiguous. This observational study sought to identify the very earliest symptoms in the natural history of MSA.MethodsDetailed clinical histories focusing on early symptoms were obtained from 30 subjects recently diagnosed with MSA. Historical data were correlated with neurological examinations and laboratory autonomic testing.ResultsSubjects’ mean age was 63.9 years. Ten were classified as having MSA-P and 20 MSA-C. The evaluations occurred 2.9 ± 0.4 months after diagnosis. The first symptom of MSA was autonomic in 22 (73%) and motor in 3 (10%) subjects (p < 0.0001). The most frequent first symptom was erectile failure, which occurred in all men beginning 4.2 ± 2.6 years prior to diagnosis. After erectile failure, postural lightheadness or fatigue following exercise, urinary urgency or hesitancy, and violent dream enactment behavior consistent with REM behavioral sleep disorder were the most frequent initial symptoms. Neither the order of symptom progression, which was highly variable, nor autonomic severity scores differentiated between MSA-P and MSA-C.ConclusionsThe first symptoms of MSA are frequently autonomic and may predate recognition of motor manifestations. Orthostatic hypotension and, in men, erectile failure are among the first symptoms that, when evaluated in the context of associated clinical findings, may facilitate accurate and earlier diagnosis.
Highlights
Multiple system atrophy (MSA) is a rare, sporadic, and fatal α-synucleinopathy with features of parkinsonism or cerebellar dysfunction, autonomic failure, and pyramidal dysfunction occurring in various combinations [1, 2]
The results of this study indicate that early symptoms in MSA are frequently autonomic and predate recognition of motor manifestations
At the time of initial diagnosis, careful attention to historical details found that a majority (77%) of MSA patients recalled early autonomic symptoms that initially were not recognized as being a manifestation of a neurodegenerative disease
Summary
Multiple system atrophy (MSA) is a rare, sporadic, and fatal α-synucleinopathy with features of parkinsonism or cerebellar dysfunction, autonomic failure, and pyramidal dysfunction occurring in various combinations [1, 2]. The diagnosis of MSA is often delayed [7] because its presenting signs and symptoms emerge gradually and may closely resemble those of more common disorders such as Parkinson’s disease (PD). It is not unusual for key autonomic symptoms to be overlooked or not recognized as being potentially serious. MSA is diagnosed by clinical criteria that rely on neurological signs and symptoms. As MSA is categorized according to its extrapyramidal motor deficits into MSA-cerebellar (MSA-C) and
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