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Axel Lipp, MD; Paola Sandroni, MD, PhD; J. Eric Ahlskog, MD, PhD; Robert D. Fealey, MD; Kurt Kimpinski, MD, PhD; Valeria Iodice, MD; Tonette L. Gehrking; Stephen D. Weigand, MS; David M. Sletten, BA; Jade A. Gehrking; Kim K. Nickander, BA; Wolfgang Singer, MD; Demetrius M. Maraganore, MD; Sid Gilman, MD; Gregor K. Wenning, MD, PhD; Clifford W. Shults, MD; Phillip A. Low, MD

Arch Neurol. 2009;66(6):742-750. doi:10.1001/archneurol.2009.71

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.

Author Affiliations: Departments of Neurology (Drs Lipp, Sandroni, Ahlskog, Fealey, Kimpinski, Iodice, Singer, Maraganore, and Low; Mss T. L. Gehrking, J. A. Gehrking, and Nickander; and Mr Sletten) and Biostatistics (Mr Weigand), Mayo Clinic, Rochester, Minnesota; Department of Neurology, University of Michigan, Ann Arbor (Dr Gilman); Universitätsklinik für Neurologie, Innsbruck, Austria (Dr Wenning); and VA Medical Center, San Diego, California (Dr Shults).

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