Objective

To characterize the clinical, radiological, and pathological manifestations of 18 cases showing neurogenic calf amyotrophy with creatine kinase (CK) elevation by entrapment radiculopathy (NCACKEER).

Methods

We retrospectively reviewed and evaluated the medical records of patients who complained of weakness or atrophy of the calf muscles in our department between 2004 and 2019. We identified 18 cases fulfilling the proposed criteria of NCACKEER. We extracted neurological, laboratory, neurophysiological, and neuroradiological data from all cases. Moreover, we evaluated biopsy specimens from the gastrocnemius in four cases.

Results

Eighteen NCACKEER cases exhibited the characteristic findings that can discriminate previously known myopathies or polyneuropathies affecting distal legs. We noticed male predominance (72%) with an average age at diagnosis of 65.6 years. Muscle weakness or atrophy was localized in the distal legs, with Achilles tendon reflexes absent in all cases. We observed elevated serum CK levels with a range from 237 to 2294 IU/L. All electromyography (EMG) studies showed neurogenic changes in the affected muscles. Lumbar spinal MRI exhibited either spinal canal stenosis at various vertebral levels or intervertebral foraminal stenosis at L4/5 and L5/S1 in all cases with significant straightening spinal and sacral alignments. All muscle biopsy specimens showed findings of neurogenic muscular degeneration with no inflammatory infiltrations. Cases with higher CK elevation had more necrotic muscle fibers.

Conclusion

We established the clinical characteristics of NCACKEER. Evaluations of serum CK level and skeletal muscle CT imaging are useful for screening, and lumbar spinal MRI, EMG and/or muscle biopsy are necessary for diagnostic confirmation.

中文翻译：

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神经性小腿肌萎缩伴神经根病引起的CK升高；临床，放射和病理分析18例。

目的

表征18例表现为神经源性小腿肌萎缩症并伴有神经根病（NCACKEER）的肌酸激酶（CK）升高的临床，放射学和病理学表现。

方法

我们回顾性回顾和评估了2004年至2019年间本部门抱怨小腿肌肉无力或萎缩的患者的病历。我们确定了18例符合拟议NCACKEER标准的病例。我们从所有病例中提取了神经，实验室，神经生理和神经放射学数据。此外，我们评估了四例腓肠肌的活检标本。

结果

18例NCACKEER病例表现出特征性发现，可以区分出先前已知的影响远端腿的肌病或多发性神经病。我们发现男性占主导地位（72％），平均年龄为65.6岁。肌肉无力或萎缩位于远端腿部，所有病例均无跟腱反射。我们观察到血清CK水平升高，范围为237至2294 IU / L。所有肌电图（EMG）研究均显示受影响肌肉的神经源性变化。在所有情况下，腰椎MRI表现为各种椎管水平的椎管狭窄或椎间孔狭窄，椎管和骨排列明显变直，L4 / 5和L5 / S1。所有肌肉活检标本均显示神经源性肌肉变性，无炎性浸润。

结论

我们建立了NCACKEER的临床特征。血清CK水平和骨骼肌CT成像的评估可用于筛查，而腰椎MRI，EMG和/或肌肉活检对于诊断确认必不可少。