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首页> 外文期刊>Iranian Journal of Neurology >Bimelic symmetric Hirayama disease: Spectrum of magnetic resonance imaging findings and comparative evaluation with classical monomelic amyotrophy and other motor neuron disease
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Bimelic symmetric Hirayama disease: Spectrum of magnetic resonance imaging findings and comparative evaluation with classical monomelic amyotrophy and other motor neuron disease

机译：双峰对称平山病：磁共振成像发现的光谱和与经典单体肌萎缩症和其他运动神经元疾病的比较评估

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Background: The aim of the study was to evaluate the magnetic resonance imaging (MRI) findings in bilateral symmetrical Hirayama disease and find out MRI features which are probably more indicative of symmetrical Hirayama disease, thereby help in differentiating this entity from other motor neuron disease (MND).Methods: This prospective as well as retrospective study was carried out from December 2010 to September 2016 in a tertiary care center of northeast India on 92 patients with Hirayama disease. Only 19 patients having bilateral symmetric upper limb involvement at the time of presentation were included in this study sample.Results: Nineteen patients, who constituted 20.6% of 92 patients of clinical and flexion MRI confirmed Hirayama disease were found to have bilateral symmetrical wasting and weakness of distal upper limb muscles at the time of presentation. Mean ± standard deviation (SD) age of onset of the disease process was 21.7 ± 3.8 years with mean ± SD duration of illness of 3.6 ± 1.3 years. MRI revealed lower cervical cord flattening in 13 (68.4%) patients which was symmetrical in 6 (31.6%) patients and asymmetrical in 7 (36.8%) patients. In the majority of these patients, T2-weighted images (T2WI) cervical cord hyperintensities were found extending from C5 to C6 vertebral level. Seven (36.8%) patients in our study showed bilateral symmetric T2WI hyperintensities in anterior horn cells (AHC).Conclusion: Bilateral symmetrical involvement of Hirayama disease is an uncommon presentation. Symmetrical cervical cord flattening, T2WI cord and/or bilateral AHC hyperintensities were the major MRI findings detected. Flexion MRI demonstrated similar findings in both bimelic amyotrophy and classical unilateral amyotrophy. However, flexion MRI produced some distinguishing features more typical for bilateral symmetrical Hirayama disease which help to differentiate it from other MNDs.
机译：背景：这项研究的目的是评估双侧对称平山疾病的磁共振成像（MRI）结果，并找出可能更能表明平山对称疾病的MRI特征，从而帮助将该实体与其他运动神经元疾病区分开来（方法：这项前瞻性及回顾性研究于2010年12月至2016年9月在印度东北部的三级医疗中心对92例平山病患者进行。结果：本研究样本仅包括19例出现双侧对称上肢受累的患者。结果：19例患者（占92例临床和屈曲MRI确诊的平山病患者的20.6％）被发现患有双侧对称性消瘦和无力出现时的上肢远端肌肉。发病过程的平均±标准差（SD）年龄为21.7±3.8年，平均±SD病程为3.6±1.3年。 MRI显示13例（68.4％）的患者颈椎脐带变平，其中6例（31.6％）的患者对称，7例（36.8％）的患者不对称。在大多数这些患者中，发现T2加权图像（T2WI）颈椎高信号从C5延伸到C6椎骨。本研究中有七名（36.8％）患者显示前角细胞（AHC）出现双侧对称性T2WI高强度。结论：平山病双侧对称性受累并不常见。对称的颈椎颈椎扁平化，T2WI脊髓和/或双侧AHC高强度是检测到的主要MRI表现。屈曲MRI在双体性肌萎缩症和经典单侧肌萎缩症中均显示出相似的发现。然而，屈曲MRI产生了一些双侧对称性平山病更典型的区别特征，有助于将其与其他MND区别开来。

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