肌萎缩侧索硬化症与神经结节病：一例报告
Amyotrophic lateral sclerosis and neurosarcoidosis: a case report.
作者信息
Chen I-Hweii Amy, Mitsumoto Hiroshi, Vonsattel Jean P, Hays Arthur P
机构信息
Department of Neurology and the Eleanor and Lou Gehrig MDA/ALS Research Center, Columbia University, New York, New York, USA.
出版信息
Muscle Nerve. 2009 Feb;39(2):234-8. doi: 10.1002/mus.21159.
Abstract
Amyotrophic lateral sclerosis (ALS) remains a clinical diagnosis without definable biomarkers. The pathomechanism of motor neuron degeneration in ALS has yet to be elucidated. Here we present a case of limb-onset ALS, with autopsy findings of Bunina bodies and skein-like inclusions, as well as sarcoid granulomas predominating among motor neurons. The targeting of the motor neurons by the sarcoid inflammation raises questions regarding the role of cellular immunity in the pathomechanisms for ALS.
摘要
肌萎缩侧索硬化症（ALS）仍然是一种没有明确生物标志物的临床诊断疾病。ALS中运动神经元变性的发病机制尚未阐明。在此，我们报告一例肢体起病的ALS病例，尸检发现有布尼亚小体和丝状包涵体，以及运动神经元中以结节病肉芽肿为主。结节病炎症对运动神经元的靶向作用引发了关于细胞免疫在ALS发病机制中作用的疑问。
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