Corneal confocal microscopy detects small-fiber neuropathy in Charcot-Marie-Tooth disease type 1A patients

Mitra Tavakoli, Andy Marshall, Siddharth Banka, Ioannis N. Petropoulos, Hassan Fadavi, Helen Kingston, Rayaz Malik

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49 Citations (Scopus)

Abstract

Introduction: Although unmyelinated nerve fibers are affected in Charcot-Marie-Tooth type 1A (CMT1A) disease, they have not been studied in detail due to the invasive nature of the techniques needed to study them. We established alterations in C-fiber bundles of the cornea in patients with CMT1A using non-invasive corneal confocal microscopy (CCM). Methods: Twelve patients with CMT1A and 12 healthy control subjects underwent assessment of neuropathic symptoms and deficits, electrophysiology, quantitative sensory testing, corneal sensitivity, and corneal confocal microscopy. Results: Corneal sensitivity, corneal nerve fiber density, corneal nerve branch density, corneal nerve fiber length, and corneal nerve fiber tortuosity were significantly reduced in CMT1A patients compared with controls. There was a significant correlation between corneal sensation and CCM parameters with the severity of painful neuropathic symptoms, cold and warm thresholds, and median nerve CMAP amplitude. Conclusions: CCM demonstrates significant damage to C-fiber bundles, which relates to some measures of neuropathy in CMT1A patients.

Original languageEnglish
Pages (from-to)698-704
Number of pages7
JournalMuscle and Nerve
Volume46
Issue number5
DOIs
Publication statusPublished - Nov 2012
Externally publishedYes

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Keywords

  • C-fiber
  • CMT1A
  • Corneal confocal microscopy
  • Corneal nerves
  • Electrophysiology
  • Neuropathy

ASJC Scopus subject areas

  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)
  • Physiology

Cite this

Tavakoli, M., Marshall, A., Banka, S., Petropoulos, I. N., Fadavi, H., Kingston, H., & Malik, R. (2012). Corneal confocal microscopy detects small-fiber neuropathy in Charcot-Marie-Tooth disease type 1A patients. Muscle and Nerve, 46(5), 698-704. https://doi.org/10.1002/mus.23377