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QUANTITATIVE SENSORY TESTING AND SWEAT FUNCTION IN FRIEDREICH'S ATAXIA. CORRELATION WITH CUTANEOUS INNERVATION
Author(s) -
Nolano M.,
Provitera V.,
Crisci C.,
Saltalamacchia A.,
Filla A.,
WendelshaferCrabb G.,
Kennedy W.R.,
Santoro L.,
Caruso G.
Publication year - 2000
Publication title -
journal of the peripheral nervous system
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1
H-Index - 67
eISSN - 1529-8027
pISSN - 1085-9489
DOI - 10.1046/j.1529-8027.2000.00513-44.x
Subject(s) - anatomy , sweat gland , nerve fiber , pilocarpine , medicine , sweat , thigh , ataxia , plexus , pathology , psychiatry , epilepsy
To evaluate small fiber function in Friedreich's Ataxia (FA), we performed in 7 patients pin‐prick, thermal thresholds, and sweat test. All tests were performed in four different sites: hand dorsum, anterior thigh, lateral distal leg, and foot dorsum. The same subjects underwent 3 mm punch skin biopsy from fingertip, anterior thigh, and lateral distal leg. We used a thin needle mounted on a calibrated nylon wire for the pin‐prick test, and a Medoc 2001 TSA system for thermal threshold assessment. Sweat test was performed using a silicon mold after stimulation with pilocarpine by iontophoresis. Skin specimens, cut into 100‐μm‐thick sections, were double‐stained using primary antibodies specific for collagen and nervous fibers and secondary antibodies labeled with Cy3 and Cy5 fluorophores. Tridimensional digitized images were obtained from z‐series of 2‐μm‐thick optical sections acquired with a confocal microscope. We found in all patients in the more distal sites definite signs of functional impairment of the small fibers. These data correlated with the skin innervation morphological findings that showed, in the same sites, a sensible loss of small fibers regarding both the epidermal free endings and the subepidermal neural plexus. Less severe morphological abnormalities were found in the proximal sites. The large fiber neuropathy in FA is well documented. Our data show a length‐dependent involvement of small fibers in the pathological process.