Madras型运动神经元病(Madras type motor neuron disease,MMND)是一种少见的以青少年起病、且具有独特临床表现的运动神经元病。最早于1970年由印度学者Meenakshisundaram等人首次报道并描述,主要好发于印度南部的Madras地区,因此而...Madras型运动神经元病(Madras type motor neuron disease,MMND)是一种少见的以青少年起病、且具有独特临床表现的运动神经元病。最早于1970年由印度学者Meenakshisundaram等人首次报道并描述,主要好发于印度南部的Madras地区,因此而得名。世界其它地区如泰国、意大利亦有报道,但多为散发型。展开更多
腓骨肌萎缩症(Charcot-Marie-Tooth disease,cMT)是一组具有高度临床和遗传异质性的单基因遗传性运动感觉神经病(hereditary motor and sensory neuropathy,HMSN)。此组疾病多于儿童和青少年时期起病,临床上以缓慢进展的对称性肢...腓骨肌萎缩症(Charcot-Marie-Tooth disease,cMT)是一组具有高度临床和遗传异质性的单基因遗传性运动感觉神经病(hereditary motor and sensory neuropathy,HMSN)。此组疾病多于儿童和青少年时期起病,临床上以缓慢进展的对称性肢体远端肌肉无力与萎缩、轻至中度末梢型感觉障碍、腱反射减弱或消失和高弓足为典型表现。展开更多
A patient of ALS like disorder in an HIV 1 clade C infected heterosexual m ale is being reported. A 37 year old gentleman presented with subacute, progre ssive asymmetrical onset of weakness and wasting of upper limbs...A patient of ALS like disorder in an HIV 1 clade C infected heterosexual m ale is being reported. A 37 year old gentleman presented with subacute, progre ssive asymmetrical onset of weakness and wasting of upper limbs associated with brisk muscle stretch reflexes and without any sensory or sphincter involvement. While nerve conduction tests were normal, the EMG of proximal and distal limb mu scles on both sides revealed evidence of denervation and reinnervation. Routine blood and urine tests and investigations for underlying causes of motor neuron d isease were noncontributory. He was HIV 1, subtype clade C seropositive. A diag nosis of HIV related anterior horn cell disease was considered and zidovudine, lamivudine and nevirapine were started. After 1 month, there was a subjective im provement of 10%and objective improvement in strength of muscles of proximal up per limb on both sides by one grade power on MRC scale. Reports of amyotrophic l ateral sclerosis (ALS) like illness in HIV are sparse. The reversibility of “A LS” like features in this subgroup of patients might offer an insight into the pathogenesis of amyotrophic lateral sclerosis. This is a first report of ALS l ike illness caused by subtype C of HIV 1 strain.展开更多
文摘Madras型运动神经元病(Madras type motor neuron disease,MMND)是一种少见的以青少年起病、且具有独特临床表现的运动神经元病。最早于1970年由印度学者Meenakshisundaram等人首次报道并描述,主要好发于印度南部的Madras地区,因此而得名。世界其它地区如泰国、意大利亦有报道,但多为散发型。
文摘腓骨肌萎缩症(Charcot-Marie-Tooth disease,cMT)是一组具有高度临床和遗传异质性的单基因遗传性运动感觉神经病(hereditary motor and sensory neuropathy,HMSN)。此组疾病多于儿童和青少年时期起病,临床上以缓慢进展的对称性肢体远端肌肉无力与萎缩、轻至中度末梢型感觉障碍、腱反射减弱或消失和高弓足为典型表现。
文摘A patient of ALS like disorder in an HIV 1 clade C infected heterosexual m ale is being reported. A 37 year old gentleman presented with subacute, progre ssive asymmetrical onset of weakness and wasting of upper limbs associated with brisk muscle stretch reflexes and without any sensory or sphincter involvement. While nerve conduction tests were normal, the EMG of proximal and distal limb mu scles on both sides revealed evidence of denervation and reinnervation. Routine blood and urine tests and investigations for underlying causes of motor neuron d isease were noncontributory. He was HIV 1, subtype clade C seropositive. A diag nosis of HIV related anterior horn cell disease was considered and zidovudine, lamivudine and nevirapine were started. After 1 month, there was a subjective im provement of 10%and objective improvement in strength of muscles of proximal up per limb on both sides by one grade power on MRC scale. Reports of amyotrophic l ateral sclerosis (ALS) like illness in HIV are sparse. The reversibility of “A LS” like features in this subgroup of patients might offer an insight into the pathogenesis of amyotrophic lateral sclerosis. This is a first report of ALS l ike illness caused by subtype C of HIV 1 strain.