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罗丝综合征:单侧多汗、阿狄瞳孔和广泛反射消失。

Ross syndrome: Unilateral hyperhidrosis, Adie's tonic pupils and diffuse areflexia.

机构信息

Haydarpaşa Numune Training and Research Hospital, Dermatology Department, Istanbul/Turkey.

出版信息

J Dtsch Dermatol Ges. 2010 Dec;8(12):1004-6. doi: 10.1111/j.1610-0387.2010.07400.x.

Abstract

Ross syndrome is a rare disorder first described in 1958 with partial autonomic dysfunction. It has three basic components including unilateral or bilateral segmental anhidrosis, Adie's tonic pupils and areflexia or hyporeflexia of deep tendon reflexes. The most disturbing symptom in the patients is segmental compensatory hyperhidrosis and often the hypohidrosis or anhidrosis is not even noticed. While the pathogenesis of Ross syndrome is unclear, degenerative changes or damage to the peripheral autonomic nerve system or dorsal root ganglia have been suggested as possible causes. About 50 cases have been reported, usually by neurologists and ophthalmologists, and less often by dermatologists. We present a 26-year-old patient who displayed the classic triad of this syndrome, emphasizing that the presenting complaint may be hyperhidrosis and that multidisciplinary evaluation in neurology and ophthalmology is essential.

摘要

罗斯综合征是一种罕见的疾病,于 1958 年首次描述,伴有部分自主神经功能障碍。它有三个基本组成部分,包括单侧或双侧节段性无汗症、阿狄森瞳孔和深腱反射的反射消失或减弱。患者最困扰的症状是节段性代偿性多汗症,而无汗症或少汗症甚至可能没有被注意到。虽然罗斯综合征的发病机制尚不清楚,但已经提出了外周自主神经系统或背根神经节的退行性变化或损伤可能是其原因。大约有 50 例病例被报道,通常由神经病学家和眼科医生报告,皮肤科医生报告的则较少。我们报告了一位 26 岁的患者,其表现出该综合征的经典三联征,强调首发症状可能是多汗症,神经科和眼科的多学科评估是必要的。

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