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Chin Med J (Taipei) 1998;61:S156.

Juvenile Lower Cervical Spinal Muscular Atrophy (Hirayama Disease)

Ko-Pei Kao

Associate Professor Neurological Center. Veterans General Hospital-Taipei, Taipei, Taiwan R.O.C.

Abstract

A unique and self- limiting variant of lower motor neuron disease affecting the upper limbs with juvenile onset and a marked male preponderance has been reported for more than 30 years. With a few exceptions most cases have been reported in Asia.

In this presentation, the clinical observations on this island (Neuroepidemiology 1993;12:331-335, European Neurology 1994;34:103-106, J Neurol 1993;240:284-286, Brain & Dev 1993(15):219-221, etc) and "tight dura canal in flexion (flexion myelopathy)" the likely patho-mechanism raised recently, will be reviewed.

[Chin Med J (Taipei) 1998;61:S156.]

Copyright: 1998, Chinese Medical Association (Taipei)