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Hirayama Disease and Other Focal Amyotrophies

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Neuromuscular Disorders

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Abstract

Hirayama disease results in focal amyotrophy of upper limbs. It is seen in young Asian males. Distal upper limbs are usually involved in an asymmetric manner and patients can have cold paresis. Neck MRI in flexion shows dural changes and spinal cord signals in a proportion of patients. Cervical segments can be relatively long in such patients. The disease burns itself out after initial progression of months and patients stabilize. Cervical collar therapy and, in some selected patients, surgical interventions have been known to reduce the impact of the disease.

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Authors and Affiliations

  1. Department of Neurology, Bombay Hospital Institute of Medical Sciences, Mumbai, Maharashtra, India

    Satish V. Khadilkar

  2. Consultant Neurologist, Suyog Neurology Clinic, NH Multispeciality Hospital, Ahmedabad, Gujarat, India

    Rakhil S. Yadav

  3. Consultant Neurologist, TN Medical College and Nair Hospital, Mumbai, Maharashtra, India

    Bhagyadhan A. Patel

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  1. Satish V. Khadilkar
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  2. Rakhil S. Yadav
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  3. Bhagyadhan A. Patel
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Khadilkar, S.V., Yadav, R.S., Patel, B.A. (2018). Hirayama Disease and Other Focal Amyotrophies. In: Neuromuscular Disorders. Springer, Singapore. https://doi.org/10.1007/978-981-10-5361-0_3

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  • DOI: https://doi.org/10.1007/978-981-10-5361-0_3

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