2015
DOI: 10.1016/j.jocn.2014.11.025
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Hirayama disease

Abstract: This article discusses three patients with likely Hirayama disease. They have no other significant past medical history and no personal or family history of other neurological disorders. Hirayama disease is a form of cervical myelopathy attributed to forward displacement of the posterior cervical dural sac on neck flexion with resultant cord compression and/or venous congestion. It is characterized by a pure motor focal amyotrophy in the distribution of C7, C8 and T1 spinal segmental-innervated muscles and dif… Show more

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Cited by 26 publications
(45 citation statements)
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“…To achieve real dynamic spinal imaging, specialized equipment such as multi-positional MRI scanners is required. However, if these devices are not available, optimizing neutral and flexion cervical spine MRI scan can be performed by using standard MRI equipment 14) . In our cases, upon suspicion of For patients with Hirayama disease, the most effective treatment is avoiding neck flexion by using neck collars, which significantly halts the disease progression 15) .…”
Section: Discussionmentioning
confidence: 99%
“…To achieve real dynamic spinal imaging, specialized equipment such as multi-positional MRI scanners is required. However, if these devices are not available, optimizing neutral and flexion cervical spine MRI scan can be performed by using standard MRI equipment 14) . In our cases, upon suspicion of For patients with Hirayama disease, the most effective treatment is avoiding neck flexion by using neck collars, which significantly halts the disease progression 15) .…”
Section: Discussionmentioning
confidence: 99%
“…Here, we suspected monomelic amyotrophy based on the fact that patient had no other forms of existing disease or family history, exhibited symptoms localized to the right lower limb, and had normal electrophysiological findings with regard to other lower and upper limbs and paraspinal muscles. Yet, because monomelic amyotrophy generally affects the upper extremities and frequently presents in male adolescents [7][8][9], additional observation is required before a definitive diagnosis can be made. Nerve compression induces edema within the endoneurium and thickening of the internal epineurium and perineurium, which also become enlarged due to microvascular proliferation.…”
Section: Discussionmentioning
confidence: 99%
“…Hirayama disease is a rare form of monomelic myopathy that can mimic amyotrophic lateral sclerosis, predominantly occurs in young males in their teenage years, and most often presents as unilateral weakness and atrophy limited to the hand and forearm in the C7‐T1 distribution . This may be related to anterior horn damage from compression of the lower cervical spinal cord against the vertebral bodies during flexion secondary to an abnormally short thecal sac . MRI of the cervical spine may be normal in the neutral position (Fig A).…”
Section: Epidural Venous Plexus Variations and Related Pathologymentioning
confidence: 99%