monomelic amyotrophy


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monomelic amyotrophy

Abbreviation: MMA
A pure movement disorder characterized by degeneration and death of motor neurons, resulting in atrophy of a limb, typically one arm.
Synonym: Hirayama syndrome
See also: amyotrophy
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Its other synonyms are juvenile muscular atrophy of the distal upper extremity (JMADUE)3 or monomelic amyotrophy (MMA)4 and juvenile asymmetric segmental spinal atrophy.5 Although the cause of cervical myelopathy remains unclear, neuropathologic and neuroradiologic findings suggest an abnormal compression or flattening of the anterior cord against the vertebral bodies during neck flexion, causing compression of the cervical cord, and resulting in atrophic and ischaemic changes in the anterior horn.
Benign monomelic amyotrophy of lower limb: report of three cases.
Benign monomelic amyotrophy lower limb: a rare entity with a characteristic muscular CT.
Benign monomelic amyotrophy: a study of twenty-one cases.