Progress in clinical related research of Hirayama disease

Abstract

Hirayama disease (HD), also known as juvenile muscular atrophy of the distal upper extremity, is featured by concealed onset clinically and often occurs in male adolescence. In most cases, one or both of the asymmetrical distal muscles of the upper extremities are weak and atrophied without objective sensory abnormalities and this disease naturally stops developing after several years. Early Hirayama disease is similar to motor neuron diseases in clinical symptoms like amyotrophic lateral sclerosis. The limitation of medical diagnostic techniques and the lack of understanding by clinicians lead to a higher rate of misdiagnosis of Hirayama disease, making their treatment plan and prognosis completely different. Therefore, a timely and accurate diagnosis of Hirayama disease is essential. In recent years, domestic and foreign scholars conducted researches in the diagnosis of Hirayama disease mainly through clinical manifestation combined with the magnetic resonance imaging of the cervical flexion position and the application of various neurophysiological detection techniques. As the number of clinical cases gradually increases, Hirayama disease draws more attention of clinicians and there are more and more reports and researches on related cases. With the development of research and application of imaging and neuroelectrophysiological techniques, the importance and effectiveness of early diagnosis and treatment of the disease have been affirmed and emphasized, thus people acquire a better understanding of its pathogenesis, clinical diagnosis and treatment. Especially for those suspected patients with juvenile muscular atrophy of the distal upper extremity, it is more important to be vigilant against the possibility of Hirayama disease. Based on this reality, this paper will discuss the new understanding and research progress of pathogenesis, clinical diagnosis and treatment of Hirayama disease, which is very important for clinicians to better understand and pay more attention to this disease.