A Clinical, Magnetic Resonance Imaging, and Survival Motor Neuron Gene Deletion Study of Hirayama Disease

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A clinical, magnetic resonance imaging, and survival motor neuron gene deletion study of Hirayama disease.

BACKGROUND Hirayama disease (HD) is a segmental nonprogressive spinal muscular atrophy found in male patients. OBJECTIVE To report the results of a comprehensive evaluation of clinical, magnetic resonance imaging (MRI), electromyography (EMG), and survival motor neuron (SMN) gene analysis of HD. DESIGN Clinical, MRI, and SMN gene deletion study. SETTING Tertiary care teaching hospital. ...

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Magnetic resonance imaging in Hirayama disease.

Hirayama disease (HD) is a rare type of cervical myelopathy related to flexion of the neck characterized by progressive muscular weakness and atrophy of the distal upper limbs most frequently seen in young males. HD is thought to be secondary to an abnormal anterior displacement of the posterior dura with secondary compression of the lower cervical spinal cord and chronic injury to the anterior...

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Clinical Experiences of Uncommon Motor Neuron Disease: Hirayama Disease

Hirayama disease, juvenile muscular atrophy of the distal upper limb, is a rare disease predominantly affecting the anterior horn cells of the cervical spinal cord in young men. This cervical myelopathy is associated with neck flexion. It should be suspected in young male patients with a chronic history of weakness and atrophy involving the upper extremities followed by clinical stability in fe...

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Bimelic symmetric Hirayama disease: Spectrum of magnetic resonance imaging findings and comparative evaluation with classical monomelic amyotrophy and other motor neuron disease

Background: The aim of the study was to evaluate the magnetic resonance imaging (MRI) findings in bilateral symmetrical Hirayama disease and find out MRI features which are probably more indicative of symmetrical Hirayama disease, thereby help in differentiating this entity from other motor neuron disease (MND). Methods: This prospective as well as retrospective study was carried out from Decem...

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ژورنال

عنوان ژورنال: Archives of Neurology

سال: 2005

ISSN: 0003-9942

DOI: 10.1001/archneur.62.1.120