The clinical characteristics of Hirayama disease in females

Wang, Hongwei; Wei, Lei; Tian, Ye; Wu, Jianwei; Ma, Xin; Lyu, Feizhou; Xia, Xinlei; Liang, Jingjuan; Jiang, Jianyuan; Wang, Hongli

doi:10.1016/j.nrleng.2022.06.004

Cited by 2 publications

(1 citation statement)

References 33 publications

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“…The male-to-female ratio varies considerably between countries, with a strong male predominance (2.8:1 in India). 16 The disease affects patients aged 15-25 years. 8 Hirayama noted that the peak age of disease onset was 2 years after the longitudinal growth curve peak of adolescents.…”

Section: History and Epidemiologymentioning

confidence: 99%

Hirayama Disease: Review on Pathophysiology, Clinical Features, Diagnosis and Treatment

Gomathy¹,

Agarwal²,

Garg³

et al. 2022

US Neurology

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Hirayama disease is a relatively unusual cause of cervical myelopathy characterized by uni- or bilateral asymmetric weakness, fasciculations and atrophy of the distal upper limbs, mostly seen in young males. The insidious weakness progresses over 3–5 years, after which it stops. Hirayama disease results from the abnormal anterior shifting of the posterior cervical dura that occurs during cervical flexion, leading to cord impingement. Electrophysiologic studies reveal denervation of C7–T1 myotomes and absence of sensory involvement. Dynamic magnetic resonance imaging of the cervical spine is essential in confirming the diagnosis. Treatment is aimed at primarily avoiding neck flexion, which is achieved with the help of a cervical brace; however, progressive disease calls for surgical intervention. Although the disease is self-limiting, it causes many functional impairments in affected individuals. In this review, we describe the epidemiology, pathophysiology, clinical manifestations, imaging characteristics, electrophysiologic findings, differential diagnoses and updates in the treatment of Hirayama disease. This review also aims to improve the awareness of this disease among clinicians, enabling early suspicion, diagnosis and management.

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Section: History and Epidemiologymentioning

confidence: 99%

Hirayama Disease: Review on Pathophysiology, Clinical Features, Diagnosis and Treatment

Gomathy¹,

Agarwal²,

Garg³

et al. 2022

US Neurology

View full text Add to dashboard Cite

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A Case Report and Review of Literature on Hirayama Disease

Baddam,

Chadalavada,

Ambati

2024

Cureus

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Hirayama disease (HD) is a rare, benign, self-limiting condition that typically affects individuals in their 20s. Although the disease is self-limiting, it can result in functional impairment in those affected. The most common presentation is an asymmetrical, unilateral, or bilateral upper limb weakness with wasting. With an interesting pathogenesis and lack of definitive treatment, HD is an interesting neurological conundrum. Mild symptoms in patients often lead to underreporting of the disease, as individuals may not seek medical attention or may not recognize their symptoms. Most case reports in the literature are from Asia and the Middle East. We report a case of HD in a male patient in his 20s with gradual bilateral upper limb weakness and wasting, confirmed by imaging and nerve conduction studies.

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The clinical characteristics of Hirayama disease in females

Cited by 2 publications

References 33 publications

Hirayama Disease: Review on Pathophysiology, Clinical Features, Diagnosis and Treatment

Hirayama Disease: Review on Pathophysiology, Clinical Features, Diagnosis and Treatment

A Case Report and Review of Literature on Hirayama Disease

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