Prospective differentiation of multiple system atrophy from Parkinson disease, with and without autonomic failure.
OBJECTIVE
To report preliminary results of a prospective ongoing study of multiple system atrophy (MSA) and Parkinson disease (PD), with a large subset of patients with PD with autonomic failure (25%), to evaluate autonomic indices that distinguish MSA from PD.


METHODS
We used consensus criteria, detailed autonomic studies (Composite Autonomic Symptom Scale, Composite Autonomic Scoring Scale, thermoregulatory sweat test, and plasma catecholamines), and functional scales (Unified MSA Rating Scale [UMSARS] I-IV and Hoehn-Yahr grading) on a prospective, repeated, and ongoing basis.


RESULTS
We report the results of a study on 52 patients with MSA (mean [SD], age, 61.1 [7.8] years; body mass index (calculated as weight in kilograms divided by height in meters squared), 27.2 [4.6]; Hoehn-Yahr grade, 3.2 [0.9]; UMSARS I score, 21.5 [7.4]; and UMSARS II score, 22.7 [9.0]) and 29 patients with PD, including PD with autonomic failure (mean [SD], age, 66.0 [8.1] years; body mass index, 26.6 [5.5]; Hoehn-Yahr grade, 2.2 [0.8]; UMSARS I score, 10.4 [6.1]; and UMSARS II score, 13.0 [5.9]). Autonomic indices were highly significantly more abnormal in MSA than PD (P < .001) for the Composite Autonomic Scoring Scale (5.9 [1.9] vs 3.3 [2.3], respectively), Composite Autonomic Symptom Scale (54.4 [21.8] vs 24.7 [20.5], respectively), and thermoregulatory sweat test (percentage anhidrosis, 57.4% [35.2%] vs 9.9% [17.7%], respectively). These differences were sustained and greater at 1-year follow-up, indicating a greater rate of progression of dysautonomia in MSA than PD.


CONCLUSIONS
The severity, distribution, and pattern of autonomic deficits at study entry will distinguish MSA from PD, and MSA from PD with autonomic failure. These differences continue and are increased at follow-up. Our ongoing conclusion is that autonomic function tests can separate MSA from PD. Autonomic indices support the notion that the primary lesion in PD is ganglionic and postganglionic, while MSA is preganglionic.
Lipp
Axel
A
Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA.
Sandroni
Paola
P
Ahlskog
J Eric
JE
Fealey
Robert D
RD
Kimpinski
Kurt
K
Iodice
Valeria
V
Gehrking
Tonette L
TL
Weigand
Stephen D
SD
Sletten
David M
DM
Gehrking
Jade A
JA
Nickander
Kim K
KK
Singer
Wolfgang
W
Maraganore
Demetrius M
DM
Gilman
Sid
S
Wenning
Gregor K
GK
Shults
Clifford W
CW
Low
Phillip A
PA
D000368:Aged; D001833:Body Temperature Regulation / Q000502:physiology; D002395:Catecholamines / Q000032:analysis / Q000097:blood; D003937:Diagnosis, Differential; D003951:Diagnostic Errors / Q000517:prevention & control; D004185:Disability Evaluation; D005260:Female; D005725:Ganglia, Autonomic / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology*; D009460:Neurologic Examination / Q000379:methods; D010300:Parkinson Disease / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology*; D011237:Predictive Value of Tests; D011446:Prospective Studies; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D012791:Shy-Drager Syndrome / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology*; D013543:Sweat Gland Diseases / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology
D002395:Catecholamines