Verma Ashok, Berger Joseph R
Department of Neurology, Kessenich Family MDA-ALS Center, University of Miami Miller School of Medicine, 1150 NW 14 Street, Suite 701, Miami, FL 33136, USA.
J Neurol Sci. 2006 Jan 15;240(1-2):59-64. doi: 10.1016/j.jns.2005.09.005. Epub 2005 Oct 19.
A viral etiology of amyotrophic lateral sclerosis (ALS) has been proposed because of the selective vulnerability of motor neurons to certain viruses. During the last 20 years, at least 19 cases of ALS or ALS-like disease have been reported in HIV-1 (HIV) seropositive individuals.
To describe two cases of clinically definite ALS in patients with HIV infection and to review the previously reported cases of HIV-associated ALS syndrome.
A multidisciplinary ALS center and Neuro-AIDS clinic at a tertiary care university hospital.
PATIENTS/DESIGN: We investigated and prospectively monitored two patients who had developed clinically definite ALS by El Escorial criteria several years after acquiring the HIV infection. The previously reported cases of ALS or ALS-like disease in patients with HIV infection were reviewed for comparison and contrast with the characteristics of sporadic ALS.
The clinical course of ALS in our two HIV seropositive individuals mirrored that of classical sporadic ALS. A review of previously described 19 patients with ALS syndrome revealed clinically definite ALS in 4 cases and clinically probable or possible ALS in 15. ALS commenced at different stages of the HIV disease; in 7 patients, HIV infection was discovered contemporaneously with diagnosis of ALS. CD4+ T cell count ranged from 2 to 560 cells/mm3. Three (1 definite ALS) of the fatal cases were studied at autopsy and all exhibited pathology outside the motor neuron pool. Unlike our patients, 7 of 8 patients with HIV-associated ALS syndrome receiving HAART demonstrated at least partial recovery of their motor deficit.
ALS-like syndrome can occur in association with HIV infection; however, the causal relationship remains uncertain. Patients with ALS syndrome related to HIV infection are generally younger in age and often demonstrate pathology outside the motor neuron system. Patients with HIV-associated ALS syndrome may improve following antiretroviral therapy. An aggressive HAART regimen to reduce viral load should be pursued in all such cases.
由于运动神经元对某些病毒具有选择性易感性，有人提出肌萎缩侧索硬化症（ALS）存在病毒病因。在过去20年中，至少有19例ALS或ALS样疾病在HIV-1（HIV）血清反应阳性个体中被报道。
描述2例HIV感染患者的临床确诊ALS病例，并回顾先前报道的HIV相关ALS综合征病例。
一所三级护理大学医院的多学科ALS中心和神经艾滋病诊所。
患者/设计：我们调查并前瞻性监测了2例在感染HIV数年之后依据埃尔埃斯科里亚尔标准发展为临床确诊ALS的患者。回顾先前报道的HIV感染患者的ALS或ALS样疾病病例，以便与散发性ALS的特征进行比较和对照。
我们的2例HIV血清反应阳性个体的ALS临床病程与典型散发性ALS相似。对先前描述的19例ALS综合征患者的回顾显示，4例为临床确诊ALS，15例为临床很可能或可能ALS。ALS在HIV疾病的不同阶段开始；7例患者在诊断ALS时同时发现HIV感染。CD4 + T细胞计数范围为2至560个细胞/mm³。3例（1例确诊ALS）死亡病例进行了尸检，所有病例均在运动神经元池外表现出病理改变。与我们的患者不同，接受高效抗逆转录病毒治疗（HAART）的8例HIV相关ALS综合征患者中有7例至少部分恢复了运动功能障碍。
ALS样综合征可与HIV感染相关；然而，因果关系仍不确定。与HIV感染相关的ALS综合征患者通常年龄较轻，且常表现出运动神经元系统外的病理改变。HIV相关ALS综合征患者在抗逆转录病毒治疗后可能改善。在所有此类病例中应采用积极的HAART方案以降低病毒载量。
