在进行性脊髓病中进行脊髓萎缩成像：人类嗜 T 淋巴细胞病毒 I 型相关的神经系统疾病（HAM/TSP）和多发性硬化症（MS）。
Imaging spinal cord atrophy in progressive myelopathies: HTLV-I-associated neurological disease (HAM/TSP) and multiple sclerosis (MS).
机构信息
Viral Immunology Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health (NIH), Bethesda, MD.
Translational Neuroradiology Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health (NIH), Bethesda, MD.
出版信息
Ann Neurol. 2017 Nov;82(5):719-728. doi: 10.1002/ana.25072. Epub 2017 Nov 8.
OBJECTIVE
Previous work measures spinal cord thinning in chronic progressive myelopathies, including human T-lymphotropic virus 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) and multiple sclerosis (MS). Quantitative measurements of spinal cord atrophy are important in fully characterizing these and other spinal cord diseases. We aimed to investigate patterns of spinal cord atrophy and correlations with clinical markers.
METHODS
Spinal cord cross-sectional area was measured in individuals (24 healthy controls [HCs], 17 asymptomatic carriers of HTLV-1 (AC), 47 HAM/TSP, 74 relapsing-remitting MS [RRMS], 17 secondary progressive MS [SPMS], and 40 primary progressive MS [PPMS]) from C1 to T10. Clinical disability scores, viral markers, and immunological parameters were obtained for patients and correlated with representative spinal cord cross-sectional area regions at the C2 to C3, C4 to C5, and T4 to T9 levels. In 2 HAM/TSP patients, spinal cord cross-sectional area was measured over 3 years.
RESULTS
All spinal cord regions are thinner in HAM/TSP (56 mm [standard deviation, 10], 59 [10], 23 [5]) than in HC (76 [7], 83 [8], 38 [4]) and AC (71 [7], 78 [9], 36 [7]). SPMS (62 [9], 66 [9], 32 [6]) and PPMS (65 [11], 68 [10], 35 [7]) have thinner cervical cords than HC and RRMS (73 [9], 77 [10], 37 [6]). Clinical disability scores (Expanded Disability Status Scale [p = 0.009] and Instituto de Pesquisas de Cananeia [p = 0.03]) and CD8 T-cell frequency (p = 0.04) correlate with T4 to T9 spinal cord cross-sectional area in HAM/TSP. Higher cerebrospinal fluid HTLV-1 proviral load (p = 0.01) was associated with thinner spinal cord cross-sectional area. Both HAM/TSP patients followed longitudinally showed thoracic thinning followed by cervical thinning.
INTERPRETATION
Group average spinal cord cross-sectional area in HAM/TSP and progressive MS show spinal cord atrophy. We further hypothesize in HAM/TSP that is possible that neuroglial loss from a thoracic inflammatory process results in anterograde and retrograde degeneration of axons, leading to the temporal progression of thoracic to cervical atrophy described here. Ann Neurol 2017;82:719-728.
目的
先前的研究已经测量了慢性进行性脊髓病（包括人类 T 淋巴细胞病毒 1（HTLV-1）相关性脊髓病/热带痉挛性截瘫（HAM/TSP）和多发性硬化症（MS））中的脊髓变薄。脊髓萎缩的定量测量对于充分描述这些疾病和其他脊髓疾病非常重要。我们旨在研究脊髓萎缩的模式及其与临床标志物的相关性。
方法
对 24 名健康对照者（HCs）、17 名无症状 HTLV-1 携带者（AC）、47 名 HAM/TSP 患者、74 名复发缓解型 MS（RRMS）患者、17 名继发进展型 MS（SPMS）患者和 40 名原发进展型 MS（PPMS）患者的 C1 至 T10 脊髓进行了脊髓横截面积测量。为患者获得了临床残疾评分、病毒标志物和免疫学参数，并与 C2 至 C3、C4 至 C5 和 T4 至 T9 水平的代表性脊髓横截面积区域相关联。在 2 名 HAM/TSP 患者中，测量了 3 年的脊髓横截面积。
结果
与 HCs（76 [7]、83 [8]、38 [4]）和 AC（71 [7]、78 [9]、36 [7]）相比，所有脊髓区域在 HAM/TSP 中均变薄（56 [10]、59 [10]、23 [5]）。SPMS（62 [9]、66 [9]、32 [6]）和 PPMS（65 [11]、68 [10]、35 [7]）的颈段脊髓比 HCs 和 RRMS 更薄（73 [9]、77 [10]、37 [6]）。HAM/TSP 的临床残疾评分（扩展残疾状况量表[Expanded Disability Status Scale，p=0.009]和 Instituto de Pesquisas de Cananeia，p=0.03）和 CD8 T 细胞频率（p=0.04）与 T4 至 T9 脊髓横截面积相关。更高的脑脊液 HTLV-1 前病毒载量（p=0.01）与脊髓横截面积变薄相关。两名进行纵向随访的 HAM/TSP 患者均表现为胸段变薄，随后是颈段变薄。
结论
HAM/TSP 和进行性 MS 的组平均脊髓横截面积显示脊髓萎缩。我们进一步假设，在 HAM/TSP 中，可能是由于胸段炎症过程中的神经胶质丧失导致轴突的顺行和逆行变性，导致这里描述的胸段到颈段萎缩的时间进展。
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