Nalini A, Desai Anita, Mahato Simendra Kumar
Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India.
Ann Indian Acad Neurol. 2009 Apr;12(2):127-30. doi: 10.4103/0972-2327.53084.
During the last 20 years at least 23 cases of motor neuron disease have been reported in HIV-1 seropositive patients. In this report we describe the clinical picture of a young man with HIV-1 clade C infection and flail arm-like syndrome, who we were able to follow-up for a long period. We investigated and prospectively monitored a 34-year-old man with features of flail arm syndrome, who developed the weakness and wasting 1 year after being diagnosed with HIV-1 infection after a routine blood test. He presented in 2003 with progressive, symmetrical wasting and weakness of the proximal muscles of the upper limb of 2 years' duration. He had severe wasting and weakness of the shoulder and arm muscles. There were no pyramidal signs. He has been on HAART for the last 4 years and the weakness or wasting has not worsened. At the last follow-up in July 2007, the patient had the same neurological deficit and no other symptoms or signs of HIV-1 infection. MRI of the spinal cord in 2007 showed characteristic T2 hyperintense signals in the central part of the spinal cord, corresponding to the central gray matter. Thus, our patient had HIV-1 clade C infection associated with a 'flail arm-like syndrome.' The causal relationship between HIV-1 infection and amyotrophic lateral sclerosis (ALS)-like syndrome is still uncertain. The syndrome usually manifests as a lower motor neuron syndrome, as was seen in our young patient. It is known that treatment with antiretroviral therapy (ART) stabilizes/improves the condition. In our patient the weakness and atrophy remained stable over a period of 3.5 years after commencing HAART regimen.
在过去20年里,至少有23例运动神经元病报告见于HIV-1血清反应阳性患者。在本报告中,我们描述了一名感染HIV-1 C亚型且患有连枷臂样综合征的年轻男子的临床情况,我们对其进行了长期随访。我们对一名具有连枷臂综合征特征的34岁男子进行了调查并进行前瞻性监测,该男子在常规血液检测诊断出HIV-1感染1年后出现肌无力和肌肉萎缩。他于2003年就诊,上肢近端肌肉进行性、对称性萎缩和无力已持续2年。他肩部和手臂肌肉严重萎缩且无力。无锥体束征。在过去4年里他一直在接受高效抗逆转录病毒治疗(HAART),肌无力或肌肉萎缩未加重。在2007年7月的最后一次随访中,患者仍有相同的神经功能缺损,且无其他HIV-1感染的症状或体征。2007年脊髓MRI显示脊髓中央部分有特征性T2高信号,对应于中央灰质。因此,我们的患者患有与“连枷臂样综合征”相关的HIV-1 C亚型感染。HIV-1感染与肌萎缩侧索硬化(ALS)样综合征之间的因果关系仍不确定。该综合征通常表现为下运动神经元综合征,正如我们这位年轻患者所见。已知抗逆转录病毒疗法(ART)治疗可使病情稳定/改善。在我们的患者中,开始HAART治疗方案后3.5年,肌无力和萎缩保持稳定。