Mishra Ajay Kumar, Kharkongor Meban, Kuriakose Cijoy K, George Anu Anna, Peter Dincy, Carey Ronal Albert Benton, Mathew Vivek, Hansdak Samuel George
1Department of General Medicine,Christian Medical College,Vellore,Tamil Nadu,India.
2Department of Dermatology, Venereology and Leprosy,Christian Medical College,Vellore,Tamil Nadu,India.
Can J Neurol Sci. 2017 May;44(3):318-321. doi: 10.1017/cjn.2016.417.
Ross syndrome is diagnosed by the presence of segmental anhidrosis, areflexia, and tonic pupils. Fewer than 60 cases have been described in literature so far. There have been reports of presence of antibodies in such patients, suggesting an autoimmune pathogenesis.
We describe the clinical profile in this case series of 11 patients with Ross syndrome and discuss the current status of autoimmunity in its pathogenesis and the management.
Of the 11 patients with Ross syndrome there was an almost equal sex distribution (male:female ratio was 1.17:1) and the mean age of onset of symptoms was 26 years. Patients took an average of 6 years to present to a tertiary center. Sixty-three percent of the patients presented with complaints of excessive sweating, whereas only 27% had complaints of decreased sweating over a particular area of the body. Only 45% of the patients had the complete triad of Ross syndrome, which included segmental anhidrosis, tonic pupil, and absent reflexes. Eighty-nine percent of the patients had documented absent sympathetic skin response on electromyography. The various markers of autoimmunity were negative in all patients who were investigated for the same in this series. Ninety percent of the patients were managed conservatively.
These findings suggest that, in Ross syndrome, generalized injury to ganglion cells or their projections are not purely autoimmune-mediated.
罗斯综合征通过节段性无汗、反射消失和强直性瞳孔来诊断。迄今为止，文献中描述的病例少于60例。已有报道称此类患者体内存在抗体，提示自身免疫性发病机制。
我们描述了这组11例罗斯综合征患者的临床特征，并讨论了自身免疫在其发病机制和治疗中的现状。
11例罗斯综合征患者中，性别分布几乎相等（男：女比例为1.17：1），症状出现的平均年龄为26岁。患者平均花费6年时间才到三级中心就诊。63%的患者主诉多汗，而只有27%的患者主诉身体特定部位出汗减少。只有45%的患者具备罗斯综合征的完整三联征，包括节段性无汗、强直性瞳孔和反射消失。89%的患者在肌电图检查中记录到交感神经皮肤反应消失。在本系列中接受自身免疫相关检查的所有患者中，各种自身免疫标志物均为阴性。90%的患者接受了保守治疗。
这些发现表明，在罗斯综合征中，神经节细胞或其投射的广泛性损伤并非单纯由自身免疫介导。
