Somatic and autonomic function in progressive autonomic failure and multiple system atrophy

J. Cohen, Phillip Anson Low, R. Fealey, S. Sheps, N. S. Jiang

Research output: Contribution to journalArticle

148 Citations (Scopus)

Abstract

We studied 62 consecutive patients with progressive autonomic failure (PAF) or multiple system atrophy (MSA) (26 PAF; 36 MSA). Patients were well matched in age (67 vs 66 years), duration (39 vs 36 months), and severity of autonomic failure (median values for PAF and MSA). Peripheral somatic neuropathy occurred in 2 patients with PAF and 7 patients with MSA. Postganglionic sudomotor and vasomotor functions were studied using the quantitative sudomotor axon reflex test and supine plasma norepinephrine. The extent and severity of autonomic failure were assessed by thermoregulatory sweat test, by heart rate responses to deep breathing and the Valsalva maneuver, and by blood presuure recordings. Severe and widespread anhidrosis was found in both PAF and MSA patients. Postganglionic sudomotor failure occurred in the forearm in 50% each of PAF and MSA patients and at the foot in 69% and 66% of PAF and MSA patients, respectively. However, postganglionic sudomotor funtion was preserved in some patients with anhidrosis on thermoregulatory sweat test, indicating a preganglionic lesion. Vagal abnormalities were found in 77% and 81% of PAF and MSA patients. Supine plsma free norepinephrine values were significantly reduced in PAF (p < 0.001), but not in MSA, patients. Standing plasma norepinephrine values were reduced in both PAF (p < 0.001) and MSA (p < 0.001) patients. We conclude the following: (1) PAF is characterized by combined postganglionic sudomotor and adrenergic failure. (2) MSA is associated with a similar frequency of postganglionic sudomotor failure, but postganglionic adrenergic denervation was uncommon. (3) Preganglionic neuron is also involved in both disorders, but more severely in MSA. (4) Somatic neuropathy may occur.

Original languageEnglish (US)
Pages (from-to)692-699
Number of pages8
JournalAnnals of Neurology
Volume22
Issue number6
StatePublished - 1987

Fingerprint

Shy-Drager Syndrome
Multiple System Atrophy
Hypohidrosis
Norepinephrine
Sweat
Adrenergic Agents
Valsalva Maneuver
Peripheral Nervous System Diseases
Denervation
Forearm

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Somatic and autonomic function in progressive autonomic failure and multiple system atrophy. / Cohen, J.; Low, Phillip Anson; Fealey, R.; Sheps, S.; Jiang, N. S.

In: Annals of Neurology, Vol. 22, No. 6, 1987, p. 692-699.

Research output: Contribution to journalArticle

Cohen, J, Low, PA, Fealey, R, Sheps, S & Jiang, NS 1987, 'Somatic and autonomic function in progressive autonomic failure and multiple system atrophy', Annals of Neurology, vol. 22, no. 6, pp. 692-699.
Cohen, J. ; Low, Phillip Anson ; Fealey, R. ; Sheps, S. ; Jiang, N. S. / Somatic and autonomic function in progressive autonomic failure and multiple system atrophy. In: Annals of Neurology. 1987 ; Vol. 22, No. 6. pp. 692-699.
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