摘要
目的探讨遗传性感觉神经根神经病(hereditary radicular sensory neuropathy,HSN)的诊断和治疗。方法对同患HSN的姐弟2人的临床资料进行分析。结果患者父母为近亲结婚,青春期后隐匿起病,以足部无痛性毁损性溃疡为主,病程长,进展缓慢,虽不会影响寿命,但会遗留残疾,肢端浅感觉减退呈"手套"和"袜套"型,后期渐累及手部。治疗主要是避免感染及损伤,加强护理,对症处理,严重者可截肢。结论 HSN是一种极为罕见的遗传性周围神经系统疾病,目前尚无有效治疗方法,婚前的遗传咨询可预防此病的发生。
Objective Objective To investigate the diagnosis and treatment of hereditary sensory radicular neuropathy（HSN）.Methods Clinical data of two siblings with HSN were analyzed.Results Patients had an insidious onset after puberty and the marriage of their parents was consanguineous.The main clinical feature of HSN was painless damaged foot ulcers with long disease duration and slow progress.The disorder did not influence life expectancy but was often severely disabling.Acral hypoesthesia showed ＂glove＂ and ＂sock＂ types and was gradually involved in the hand in the later period.The main treatment regimens included avoiding infection and injury,enhancing care,and symptomatic therapy.The amputation was a last resort for severe patients.Conclusion HSN is an extremely rare hereditary peripheral nervous system disease and there is no effective treatment method for the disease.Premarital genetic counseling can prevent the occurrence of HSN.
出处
《南昌大学学报（医学版）》
CAS
2012年第10期104-106,共3页
Journal of Nanchang University：Medical Sciences
关键词
遗传性感觉神经根神经病
足溃疡
无痛性
感觉障碍
近亲结婚
hereditary sensory radiculan neuropathy
foot ulcer
painless
sensory disturbance
consanguineous marriage
