The clinical characteristics of Hirayama disease in females

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

doi:10.1016/j.nrleng.2022.06.004

The clinical characteristics of Hirayama disease in females

Neurologia (Engl Ed). 2022 Aug 10:S2173-5808(22)00086-4. doi: 10.1016/j.nrleng.2022.06.004. Online ahead of print.

Authors

Affiliations

¹ Department of Orthopedics, Huashan Hospital, Fudan University, Shanghai, 200040, China; Spine Center Fudan University, Shanghai, 200040, China.
² Huashan Hospital, Fudan University, Shanghai, 200040, China.
³ Department of Orthopedics, Huashan Hospital, Fudan University, Shanghai, 200040, China; Spine Center Fudan University, Shanghai, 200040, China; Department of Orthopedics, Shanghai Fifth People's Hospital, Fudan University, Shanghai 200240, China.
⁴ Department of Orthopedics, Huashan Hospital, Fudan University, Shanghai, 200040, China; Spine Center Fudan University, Shanghai, 200040, China. Electronic address: wanghongli0212@163.com.

PMID: 35963535
DOI: 10.1016/j.nrleng.2022.06.004

Abstract

Introduction: To characterize Hirayama disease in female patients, and increase awareness among clinicians regarding the specifics of this disease.

Methods: Baseline data, clinical manifestations, characteristics of cervical-flexion magnetic resonance imaging, and electromyography were collected and compared among females and males with Hirayama disease. In addition, the literature on Hirayama disease in females up to October, 2021 was searched in PubMed and the relevant data were compared with the data from our study.

Results: Twenty female and 40 male patients were included in this study. The average ages of onset and menarche were 14.65 and 12.75 years old. All patients suffered from muscular weakness and atrophy of the upper limb(s), with flattening and/or atrophy of the lower cervical spinal cords in cervical-flexion magnetic resonance imaging, and neurogenic patterns in the atrophic muscles as determined using electromyography. The age of onset in females was about 2 years later than the age of menarche, and the age of onset in females was 2 years earlier than that in males. There were no obvious differences in clinical presentation between males and females.

Discussion: Although females presented with Hirayama disease two years earlier than males, no other clinical differences were observed. Hirayama disease is likely associated with growth and development in puberty, and early identification, regardless of whether patients are male or female, is critical to optimizing prognosis.

Keywords: Clinical manifestations; Electromyography; Enfermedad de Hirayama; Female; Hirayama disease; Medical imaging; Pathogenesis; electromiografía; imagenología médica; manifestaciones clínicas; mujer/femenina; patogénesis.