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The sympathetic skin response in peripheral autonomic failure — evaluation in pure autonomic failure, pure cholinergic dysautonomia and dopamine-beta-hydroxylase deficiency

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Abstract

The sympathetic skin response (SSR) detects changes in the electrical potential in the skin in response to physiological and electrical stimuli and, therefore, may indicate the integrity of sympathetic cholinergic neural pathways to sweat glands. This has been evaluated in 21 patients with three different forms of peripheral autonomic failure. Of these, 15 had pure autonomic failure (PAF) without additional neurological features; investigations indicated both sympathetic and parasympathetic failure. Four patients had pure cholinergic dysautonomia (PCD), with clinical and laboratory features indicating only cholinergic failure. Two siblings had dopamine-betahydroxylase (DBH) deficiency with only sympathetic adrenergic failure. None was on drugs affecting cholinergic function. Ten normal individuals were aged-matched with PAF patients and studied as controls. The SSR was recorded from the palmar hand and plantar foot surfaces, using previously described techniques, in response to physiological (auditory, cough and inspiratory gasp) and electrical stimuli. Nerve conduction studies excluded an associated motor or sensory neuropathy.

The SSR was present in all normal individuals, and in both patients with DBH deficiency who had preserved cholinergic and subdomotor function. It was absent in all 15 PAF and all four PCD patients with impaired cholimergic function. Therefore, we conclude that the SSR reflected sympathetic cholinergic function in these three different groups with peripheral autonomic failure. Clin Auton Res 8:133–138

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Authors and Affiliations

  1. Autonomic Unit, University Department of Clinical Neurology, The National Hospital and Institute of Neurology, University College London, Queen Square, London, UK

    F. Magnifico &  C. J. Mathias

  2. Neurovascular Medicine Unit, St Mary's Hospital/Imperial College School of Medicine, University of London, Praed Street, W2 1NY, London, UK

    F. Magnifico & V. P. Misra

  3. Neurophysiology Department, the National Hospital and Institute of Neurology, University College London, Queen Square, London, UK

    N. M. F. Murray &  C. J. Mathias

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  1. F. Magnifico
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  2. V. P. Misra
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  3. N. M. F. Murray
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  4. C. J. Mathias
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Magnifico, F., Misra, V.P., Murray, N.M.F. et al. The sympathetic skin response in peripheral autonomic failure — evaluation in pure autonomic failure, pure cholinergic dysautonomia and dopamine-beta-hydroxylase deficiency. Clinical Autonomic Research 8, 133–138 (1998). https://doi.org/10.1007/BF02281117

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