Early-onset Hirayama disease in a female

Baumann, Matthias; Finsterer, Josef; Gizewski, Elke R.; Löscher, Wolfgang N.

doi:10.1177/2050313x16686710

Cited by 2 publications

(1 citation statement)

References 16 publications

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“…The results of these studies most often display high amplitude polyphasic action potentials in the tested nerves. 14 A flexion MRI is perhaps the most conclusive diagnostic tool in HD workup. With the patient’s neck flexed forward, MRI reveals the pathognomonic anterior displacement of the lower cervical dural sac with flattening of the cervical cord.…”

Section: Discussionmentioning

confidence: 99%

Surgical treatment of spinal cord compression due to Hirayama disease: illustrative case

Singh

Hudson

Meyer

et al. 2022

Journal of Neurosurgery: Case Lessons

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BACKGROUND Hirayama disease (HD), also known as juvenile spinal muscular atrophy, is a rare condition in which flexion of the cervical neck causes compression and ischemic changes to the anterior horns of the spinal cord. Here the authors presented the first reported case of HD in North America that was successfully treated via surgical intervention. OBSERVATIONS The patient was a 15-year boy with insidious onset upper limb weakness and atrophy. His findings were a classic presentation of HD although his complex history and relative rarity of the disease caused him to remain undiagnosed for months. After conservative management via cervical collar failed, the patient was successfully treated via C5-C7 anterior cervical discectomy and fusion. The patient’s symptoms stabilized by the 3-month follow-up. LESSONS The diagnosis of HD is easy to miss because of the lack of reporting and widespread knowledge of this condition in North America. Thus, when presented with a case of insidious onset limb weakness in a juvenile patient, HD should be placed on the differential list and verified with cervical flexion magnetic resonance imaging. Additionally, surgical intervention should be considered a safe and effective option for HD when conservative methods have failed.

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Section: Discussionmentioning

confidence: 99%

Surgical treatment of spinal cord compression due to Hirayama disease: illustrative case

Singh

Hudson

Meyer

et al. 2022

Journal of Neurosurgery: Case Lessons

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show abstract

Do patients with Hirayama disease require surgical treatment? A review of the literature

Gao

Sun

et al. 2022

IRDR

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Hirayama disease, posterior cervical surgery, anterior cervical surgeryThe main clinic characteristic of Hirayama disease (HD) is atrophy of the distal muscles in the upper limbs. Recently, an increasing number of HD cases have been reported. Many HD patients have persistently progressive symptoms and conservative treatments failed. This article aims to review the current status of the field and summarizes the main surgical treatment options for patients with HD. A comprehensive search of the PubMed and the Web of Science databases was conducted from their inception to September 15th, 2022. Search terms included "juvenile muscular atrophy of upper extremity", "Hirayama disease" and "surgery". A total of 169 relevant publications were identified and 29 articles were finally reviewed. Current surgical treatments for HD are either anterior cervical surgery or posterior cervical surgery. The two approaches can effectively stop the disease. However, no studies have compared the advantages and limitations of the two surgical methods. The previous view that HD can be improved with conservative treatment has been challenged. In many studies, surgical treatment has been shown to improve the hand function in patients with HD. However, there is still controversy about the methods of anterior and posterior cervical surgery. Future research could focus on exploring the advantages and limitations of different surgeries.Cases HD, Hirayama disease; ACDF, anterior cervical discectomy and fusion. Treatment Collar therapy Collar therapy Collar therapy Collar therapy Conservative treatment No treatment Physical therapy ACDF Not reported Conservative treatment ACDF ACDF Conservative treatment Conservative treatment Conservative treatment Conservative treatment Conservative treatment Collar therapy Conservative treatment ACDF Not reported Conservative treatment Rehabilitation treatment Not reported Posterior surgery Nerve transfer Tendon transfer Lu X, et al.

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Early-onset Hirayama disease in a female

Cited by 2 publications

References 16 publications

Surgical treatment of spinal cord compression due to Hirayama disease: illustrative case

Surgical treatment of spinal cord compression due to Hirayama disease: illustrative case

Do patients with Hirayama disease require surgical treatment? A review of the literature

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