早期肌萎缩侧索硬化症的临床及电诊断异常模式
Patterns of clinical and electrodiagnostic abnormalities in early amyotrophic lateral sclerosis.
作者信息
Simon Neil G, Lomen-Hoerth Catherine, Kiernan Matthew C
机构信息
Department of Neurology, University of California at San Francisco, San Francisco, California, USA; Prince of Wales Clinical School and Neuroscience Research Australia, University of New South Wales, Sydney, New South Wales, Australia.
出版信息
Muscle Nerve. 2014 Dec;50(6):894-9. doi: 10.1002/mus.24244. Epub 2014 Oct 31.
INTRODUCTION
The distribution of clinical and neurophysiological abnormalities in patients with early amyotrophic lateral sclerosis (ALS) was investigated in an attempt to delineate patterns of disease spread.
METHODS
Clinical and electrodiagnostic data were collected from 150 ALS patients and analyzed based on the clinical region of onset.
RESULTS
Asymmetry of clinical and neurophysiological abnormalities was more marked in upper limb-onset than lower limb-onset disease. Significant rostral-caudal gradients of clinical weakness were identified in bulbar- and lower limb-onset disease. Neurophysiological evidence of the ALS "split-hand" pattern was evident irrespective of the region of disease onset. Limbs with and without evidence of clinical weakness demonstrated similar rates of abnormality on electromyography.
CONCLUSIONS
These findings suggest a pattern of disease spread in ALS. This study may serve to guide ongoing development of disease quantitation biomarkers and the targeting of future neuroprotective strategies.
引言
对早期肌萎缩侧索硬化症（ALS）患者的临床和神经生理学异常分布进行了研究，以试图描绘疾病传播模式。
方法
收集了150例ALS患者的临床和电诊断数据，并根据临床发病部位进行分析。
结果
上肢起病的疾病中，临床和神经生理学异常的不对称性比下肢起病的疾病更为明显。在延髓和下肢起病的疾病中，发现了明显的临床肌无力头-尾梯度。无论疾病发病部位如何，ALS“分裂手”模式的神经生理学证据都很明显。有和没有临床肌无力证据的肢体在肌电图上显示出相似的异常率。
结论
这些发现提示了ALS的疾病传播模式。本研究可能有助于指导疾病定量生物标志物的持续开发以及未来神经保护策略的靶向治疗。
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