Juvenile muscular atrophy of distal upper extremities.
نویسندگان
چکیده
منابع مشابه
Non familial juvenile distal spinal muscular atrophy of upper extremity.
An uncommon variety of non familial, juvenile onset, spinal muscular atrophy with asymmetric distal upper extremity affection is described. One hundred and two patients with a one to 14 year follow up are analysed. Spinal muscular atrophies with a distal distribution are rare. However, in the past three decades, previously unrecognised varieties of neurogenic muscular atrophy have been describe...
متن کاملDistal hereditary upper limb muscular atrophy.
OBJECTIVES To identify the clinical, electrophysiological, and genetic characteristics of a family with an unusual form of hereditary motor neuron disease. METHODS Surviving members of a pedigree in which affected members presented with weakness and atrophy of distal musculature in the upper limbs were examined clinically and electrophysiologically, and had genetic testing. RESULTS The dise...
متن کاملHyperIgEaemia in patients with juvenile muscular atrophy of the distal upper extremity (Hirayama disease).
BACKGROUND Juvenile muscular atrophy of the distal upper extremity (Hirayama disease) is characterised by anterior horn cell loss in the lower cervical cord, presumably caused by anterior displacement of the dural sac during neck flexion. A recent report suggests that atopy and IgE may contribute to anterior horn damage. OBJECTIVE To investigate whether IgE is a contributing factor in Hirayam...
متن کاملJuvenile muscular atrophy of the distal upper limb (Hirayama disease) associated with atopy.
Juvenile muscular atrophy of the distal upper limb (Hirayama disease) is a rare disease predominantly affecting the anterior horn cells of the cervical spinal cord in young men. Although the disease is considered to be a type of cervical myelopathy, the mechanism remains unknown. An immunological study of five consecutive patients with this disorder who were examined in the neurology clinic at ...
متن کاملFocal cervical poliopathy causing juvenile muscular atrophy of distal upper extremity: a pathological study.
A new clinical entity under the name of "juvenile muscular atrophy of unilateral upper extremity" was first described in 1959. Although about 150 cases in Japan, and several additional cases in other countries, have been clinically reported in the literature, the pathology has remained unknown because of the benign course of the disease. The first necropsy findings are reported, obtained from a...
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ژورنال
عنوان ژورنال: Journal of Neurology, Neurosurgery & Psychiatry
سال: 1985
ISSN: 0022-3050
DOI: 10.1136/jnnp.48.3.285