Pathologic Staging of White Matter Lesions in Adult-Onset Leukoencephalopathy/Leukodystrophy With Axonal Spheroids
Pathologic Staging of White Matter Lesions in Adult-Onset Leukoencephalopathy/Leukodystrophy With Axonal Spheroids
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DOI：
10.1097/nen.0000000000000168
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发表时间：
2015-03-01
影响因子：
3.2
通讯作者：
Ang, Lee-Cyn
中科院分区：
文献类型：
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作者：
Alturkustani, Murad;Keith, Julia;Ang, Lee-Cyn
成人发病的脑白质病/脑白质营养不良伴轴突球体（ALAS）的病理特征是多变的，这导致了关于主要脱髓鞘或轴突病变是否可能是这种疾病的基础的不同假设。典型的ALAS病理很少伴有局灶性多发性硬化症（MS）样斑块。在ALAS病理伴局灶性多发性硬化症（MS）样斑块的病例中，其病理特征无法与伴有弥漫性白质异常的进展性MS区分开来。为了澄清这些问题，我们检查了来自5例ALAS病例（3男2女，年龄39-61岁）的159个代表性样本和来自3例慢性MS病例（1男2女，年龄50-73岁）的95个代表性样本的神经病理特征。ALAS病例的白质异常可分为3个发展阶段：1)白质伴大量球体，背景为有髓鞘的纤维；2)髓鞘纤维中度损失，球体稀疏到中等数量；3)汇合性轴突和髓磷脂损失样脑白质营养不良。这种分期系统的应用表明，在ALAS中髓磷脂损失发生在轴突病变之前。在进展性MS病例中，弥漫性白质异常病理可归因于原发性脱髓鞘和轴突病。一些以轴索病变为主的病例很难与ALAS病例区分。
The pathologic features of adult-onset leukoencephalopathy/leukodystrophy with axonal spheroids (ALAS) are variable, and this has led to different hypotheses as to whether primarily demyelination or axonopathy may underlie this disorder. Typical ALAS pathology is rarely accompanied by focal multiple sclerosis (MS)-like plaques. In ALAS pathology accompanied by focal multiple sclerosis (MS)-like plaques cases, the pathologic features cannot be distinguished from those of progressive MS with diffusely abnormal white matter. To clarify these issues, we examined neuropathologic features in 159 representative samples from 5 ALAS cases (3 men and 2 women aged 39-61 years) and in 95 representative samples from 3 chronic MS cases (1 man and 2 women aged 50-73 years). The white matter abnormalities in ALAS cases were characterized by 3 evolving stages: 1) white matter with numerous spheroids in a background of well-myelinated fibers; 2) moderate loss of myelinated fibers with sparse to moderate number of spheroids; and 3) leukodystrophy-like pattern of confluent axonal and myelin loss. The application of this staging system suggests that myelin loss in ALAS is preceded by axonopathy. In progressive MS cases, the diffusely abnormal white matter pathology could be attributed to both primary demyelination and axonopathy. Some cases with predominant axonopathy are difficult to distinguish from cases with ALAS.