Ross综合征是否为一种罕见且常被误解的体温调节障碍疾病:12例患者皮肤神经支配研究  

Ross syndrome: A rare or a misknown disorder of thermoregulation? A skin innervation study on 12 subjects

作　　者：Nolano M. Provitera V. Perretti A. 张磊 

机构地区：[1]Neurology Department, ‘S. Maugeri’Foundation, Via Bagni Vecchi, 1, 82037 Telese Terme (BN), Italy,Dr.

出　　处：《世界核心医学期刊文摘（眼科学分册）》2006年第12期1-1,共1页Digest of the World Core Medical Journals:Ophthalmology

摘　　要：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.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.

关 键 词：Ross综合征 患者皮肤 神经支配 体温控制 疾病 调节障碍 Adie综合征 免疫组织化学方法 

分 类 号：R442.8[医药卫生—诊断学]