Is Ross syndrome a dysautonomic disorder only? An electrophysiologic and histologic study

A. Perretti, M. Nolano, G. De Joanna, V. Tugnoli, G. Iannetti, V. Provitera, G. Cruccu, L. Santoro

Research output: Contribution to journalArticle

Abstract

Objective: To define the involvement of peripheral nerve fibers in Ross syndrome. Methods: Mechanical pain perception, tactile and thermal thresholds on hand, foot dorsum, thigh, median nerve orthodromic sensory conduction velocity (SCV) and motor conduction velocity (MCV), sural nerve antidromic SCV, peroneal nerve MCV, H-reflex, F-wave, median, tibial nerve somatosensory evoked potentials (SSEPs), perioral, hand CO2 laser late (LEPs) and ultralate evoked potentials, sympathetic skin response (SSRs), cardiovascular, Minor sweat, silastic imprint, histamine, photopletysmographic and pupil pilocarpine tests, cutaneous innervation immunohistochemical techniques were studied in 3 patients with Ross syndrome. Results: Quantitative sensory testing showed altered results in patients 1 and 2, and patient 3 had a slight impairment of mechanical pain perception. Nerve conduction, except for a median nerve distal reduction of sensory conduction in patient 1, F-wave and SSEP findings were normal; H-reflex was absent at rest in all patients. Hand LEPs were absent in patient 2, ultralate potentials were absent in patients 1 and 2. Skin biopsy showed a disease duration related reduction of unmyelinated and myelinated sensory fibers and a lack of unmyelinated autonomic fibers in all patients. Conclusions: Our data suggest that Ross syndrome is a degenerative disorder involving progressive sudomotor fibers, and then epidermal sensory unmyelinated and myelinated fibers.

Original languageEnglish
Pages (from-to)7-16
Number of pages10
JournalClinical Neurophysiology
Volume114
Issue number1
DOIs
Publication statusPublished - Jan 1 2003

Keywords

  • Ross syndrome
  • Sensory fibers
  • Sudomotor fibers

ASJC Scopus subject areas

  • Clinical Neurology
  • Radiology Nuclear Medicine and imaging
  • Neurology
  • Sensory Systems
  • Physiology (medical)

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