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Cutaneous innervation in ross syndrome: a functional and morphological study in 8 patients
Author(s) -
Nolano M,
Provitera V,
Perretti A,
Saltalamacchia AM,
Tugnoli V,
Donadio V,
Manganelli F,
Santoro L
Publication year - 2004
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 - 10.1111/j.1085-9489.2004.009209ah.x
Subject(s) - sudomotor , sensory system , hyporeflexia , medicine , subclinical infection , cholinergic , pathology , anatomy , neuroscience , biology , weakness
Ross syndrome is described as a very rare disorder characterized by segmental anhydrosis, hyporeflexia and tonic pupils. We observed 8 subjects (6 males and 2 females; age range 31–57 years) affected by Ross syndrome in the last 4 years. They had suffered heat intolerance for at least 7 years with severe impairment of thermoregulation before diagnosis of Ross syndrome was defined. All patients underwent neurological examination, electrophysiological study, quantitative sensory testing, Minor test, silastic imprint test, sympathetic skin response, cardiovascular reflexes and 3 mm punch biopsies from fingertip, thigh, leg and from residual hyperhydrotic areas. Skin samples were cut in 80‐micron sections and incubated with a panel of primary antibodies and then with secondary antibodies conjugated with fluorophores Cy2, Cy3, Cy5 to visualize neural and vascular structures. Different primary antibodies were used to distinguish myelinated and sensory and autonomic unmyelinated fibers. Digital images were obtained using a non‐laser confocal microscope (CARV, Atto Biosciences, Rockville MD, USA) and analyzed by dedicated image analysis software (Neurolucida, Microbrightfield, Willistone VT, USA). As we previously described in 3 patients (Perretti et al ., Clin Neurophysiol, 2003), besides a lack of cholinergic sudomotor fibers we found a milder involvement of unmyelinated and myelinated sensory fibers. These morphological findings were in keeping with a moderate subclinical impairment of tactile and thermal thresholds and mechanical pain detection. Sensory nerve conduction study was normal indicating a very distal involvement of sensory nerve endings. This study confirms our previous observation that Ross syndrome is a degenerative process involving progressively, besides cholinergic autonomic fibers, sensory unmyelinated and myelinated nerve fibers.