Monomelic amyotrophy: non progressive atrophy of the upper limb

Monomelic amyotrophy is a rare clinical entity, resulting in wasting and weakness localized to the hand and forearm unilaterally, in the absence of any sensory or long tract signs. The onset of the disease is insidious, occurring in males before the age of 30 years, with a clinical course marked by...

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Bibliographic Details
Published in:Journal of clinical neuroscience 1999-07, Vol.6 (4), p.353-355
Main Authors: Kiernan, Matthew C, Lethlean, A.Keith, Blum, Peter W
Format: Article
Language:English
Subjects:
Biological and medical sciences
Diseases of striated muscles. Neuromuscular diseases
Medical sciences
monomelic amyotrophy
Neurology
non-progressive atrophy
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Summary:Monomelic amyotrophy is a rare clinical entity, resulting in wasting and weakness localized to the hand and forearm unilaterally, in the absence of any sensory or long tract signs. The onset of the disease is insidious, occurring in males before the age of 30 years, with a clinical course marked by non-progression. The case of a 19-year-old Indonesian male patient is presented, with a one year history of right upper limb weakness. Nerve conduction studies were normal, without evidence of conduction block. Electromyography showed changes of chronic partial denervation. Magnetic resonance imaging scans revealed an asymmetry of the spinal cord. Possible aetiological mechanisms for these changes are discussed.
ISSN:0967-5868
1532-2653
DOI:10.1016/S0967-5868(99)90066-8