spinal muscular atrophy type III

(redirected from juvenile muscular atrophy)

spinal mus·cu·lar at·ro·phy type III

[MIM*253400]
the juvenile form, with onset in childhood or adolescence; characterized by progressive proximal muscular weakness and wasting, primarily in the pelvic girdle, followed by distal muscle involvement; caused by degeneration of motor neurons in the anterior horns of the spinal cord; autosomal recessive inheritance, caused by mutation in the SMN1 gene on 5q.
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Juvenile muscular atrophy of distal upper extremity (Hirayama disease).
Juvenile muscular atrophy of distal upper extremity (Hirayama disease): focal cervical ischemic poliomyelopathy.
Nationwide survey of juvenile muscular atrophy of distal upper extremity (Hirayama disease) in Japan.
Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity.
Keywords: Juvenile muscular atrophy, Monomelic amyotrophy, Ulnar entrapment.
Hirayama Disease (HD), also known as juvenile muscular atrophy or monomelic amyotrophy, was reported first by Hirayama et al.
Hirayama disease (HD), a rare neurological condition, is a sporadic juvenile muscular atrophy of the distal upper extremities, which predominantly affects the lower cervical cord.
and was reported under the name of "juvenile muscular atrophy of unilateral upper extremity" [2].
HD, also known as juvenile muscular atrophy of the distal upper extremity, is characterized by slowly progressive distal amyotrophy of the forearm ulnar muscles.[sup][6] HD mainly affects males early in their lives, and the progression of amyotrophy in HD usually arrests within a few years.
Its other synonyms are juvenile muscular atrophy of the distal upper extremity(JMADUE) or monomelic amyotrophy (MMA).
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.
Juvenile Asymmetric Segmental spino-muscular Atrophy (JASSMA) of Distal Upper Extremity also called: Juvenile muscular atrophy of distal upper extremity (Hirayama disease) is a cervical myelopathy.

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