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Neurogenic calf amyotrophy with CK elevation by entrapment radiculopathy; clinical, radiological, and pathological analyses of 18 cases

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Abstract

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.

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Data availability

Anonymized data can be made available by request to qualified investigators.

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Acknowledgements

The authors sincerely thank all patients for their participation in this study.

Funding

This study was supported in part by Grants-in-Aid for Scientific Research (C) (18K07491 to MI, 19K07813 to YI) from the Ministry of Education, Culture, Sports, Science and Technology, Japan.

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Authors and Affiliations

  1. Department of Neurology, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi, Gunma, 371-8511, Japan

    Makoto Shibata, Hiroo Kasahara, Kouki Makioka, Masaki Ikeda, Kazuaki Nagashima, Yukio Fujita & Yoshio Ikeda

Authors
  1. Makoto Shibata
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  2. Hiroo Kasahara
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  3. Kouki Makioka
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  4. Masaki Ikeda
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  5. Kazuaki Nagashima
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  6. Yukio Fujita
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  7. Yoshio Ikeda
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Contributions

MS and YI contributed to the conception and design of this study. MS, HK and YF reviewed the muscle pathology. HK, KM, MI, KN, YF and YI contributed to the interpretation and critical revision of the manuscript.

Corresponding author

Correspondence to Yoshio Ikeda.

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Conflicts of interest

The authors report no disclosures.

Ethical approval

The ethical committee of research on human subjects at the Gunma University Graduate School of Medicine approved the protocol of this study (HS2019-210).

Patient consent for publication

Written consent was obtained from Case 3 and the case presented in Fig. 3d.

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Shibata, M., Kasahara, H., Makioka, K. et al. Neurogenic calf amyotrophy with CK elevation by entrapment radiculopathy; clinical, radiological, and pathological analyses of 18 cases. J Neurol 267, 3528–3540 (2020). https://doi.org/10.1007/s00415-020-10021-3

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  • DOI: https://doi.org/10.1007/s00415-020-10021-3

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