Brain Advance Access originally published online on July 12, 2006
Brain 2006 129(8):2119-2131; doi:10.1093/brain/awl175
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Ross syndrome: a rare or a misknown disorder of thermoregulation? A skin innervation study on 12 subjects
1 Neurology Division ‘S. Maugeri’ Foundation I.R.C.C.S. Centre of Telese Terme (BN) Italy 2 Department of Neurological Sciences, University of Naples ‘Federico II’ Italy 3 Department of Neurological Sciences, University of Bologna Bologna, Italy
Correspondence to: Maria Nolano, MD, Neurology Department, ‘S. Maugeri’ Foundation, Via Bagni Vecchi, 1, 82037 Telese Terme (BN), Italy E-mail mnolano{at}fsm.it
Ross syndrome is described as a rare disorder of sweating associated with areflexia and tonic pupil. Since Ross's first description in 1958, 
40 cases have been described. We assessed the involvement of cutaneous innervation in 12 subjects with Ross syndrome using quantitative sensory testing, sweating assessment and immunohistochemical study of anhidrotic and hyperhidrotic skin. This evaluation was repeated over time in 4 out of 12 subjects. In addition, we enrolled four subjects with Holmes–Adie syndrome (areflexia and tonic pupil) to investigate similarities between the two conditions. We found in Ross patients a complex and progressive involvement of cutaneous sensory and autonomic innervation underlying the impairment of heat production and heat dissipation through both loss of sweating and loss of cutaneous blood flow regulation. In Holmes–Adie subjects we found a mild impairment of sweating without thermoregulatory problems. The persistence of a sudomotor vasoactive intestinal peptide-immunoreactive (VIP-ir) innervation, although deranged and poor, definitely differentiated Holmes–Adie from Ross patients. Ross syndrome is a progressive and complex disorder of thermoregulation difficult to differentiate from the probably pathogenetically related Holmes–Adie syndrome. Sweating assessment and skin biopsy are suitable tools to define a boundary between them. Owing to the large number of Ross patients observed in only 5 years, and to the long and complex medical history of most of them, doubts arise on the effective rarity of this condition, and we warn family doctors and other specialists, besides neurologists, to become aware of this complex disorder.
Key Words: skin biopsy; Ross syndrome; autonomic nervous system; Holmes–Adie syndrome; thermoregulation
Abbreviations: AVAs, arteriovenous anastomoses; DßH, dopamine-ß-hydroxylase; ENF, epidermal nerve fibres; IME, intrapapillary myelinated endings; ir, immunoreactive; MC, Meissner corpuscles; PGP, protein gene product; QST, quantitative sensory testing; SIT, Silastic imprint test; SSR, sympathetic skin response; TST, thermoregulatory sweat test; VIP, vasoactive intestinal peptide
Received March 13, 2006. Revised May 18, 2006. Accepted June 8, 2006.
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