This is the first report of Hirayama disease after COVID-19 mRNA vaccination in a female adolescent. Hirayama disease occurs in young people in their 20s and 30s and much more often in male than in female patients at a ratio of 20:1 [
1]. It shows an insidious onset and slow progression of muscular atrophy in the distal upper limbs, including the thenar, hypothenar, interossei muscles, and wrist flexors and extensors, but it spares the brachioradialis muscles [
6]. The amyotrophy is unilateral in most patients, but it can be bilateral. Most patients show “cold paresis” symptoms, in which the finger muscles weaken in cold environments. Paresthesia is uncommon; however, in some patients, local sensation is lost in both hands. Most (73%) patients stop developing symptoms within 5 years [
1]. Amyotrophic lateral sclerosis, spinal muscle atrophy, C8-T1 radiculopathy, compressive myelopathy, cervical spondylotic myelopathy, syringomyelia, post-polio syndrome, multifocal motor neuropathy, and toxic neuropathy should be considered in the differential diagnosis [
7]. To differentiate Hirayama disease from the above-mentioned diseases, nerve conduction velocity, electromyography (EMG), motor-evoked potentials (MEPs), and cerebrospinal fluid (CSF) tapping can be performed, and MRI yields the most characteristic findings. In previous research, EMG showed acute and chronic denervation of the atrophied muscles [
8], while the findings of nerve conduction velocity and CSF tapping analyses were almost normal [
2]. If MEPs are measured after transcranial magnetic stimulation is performed with neck flexion, the latency increases and the amplitude decreases, suggesting the pathogenic role of neck flexion in this disease [
9]. Neck flexion on MRI is diagnostic for Hirayama disease, with characteristic findings of focal hyperintensities on T2-weighted images of the anterior caudal segments of the cervical spinal cord [
10]. Our patient had no symptoms of muscle atrophy, but MRI showed a typical pattern of Hirayama disease, including an enlarged posterior epidural space with epidural venous plexus engorgement, enhancement at the cervicothoracic junction to the T8 level, and prominent posterior epidural vessels in the flexion view.
In response to the current COVID-19 pandemic, vaccines are being administered to minimize complications. However, neurological complications can occur from the vaccination itself, including headache, GBS, VST, transverse osteomyelitis, facial neuropathy, small nerve neuropathy, and multiple sclerosis, as mentioned earlier [
3]. In our patient, spinal cord damage occurred due to the immune response to the vaccination; the diagnosis of Hirayama disease was made based on the MRI findings, despite the lack of full consistency with the clinical features. Given the observation of an excessively increased COVID-19 antibody load, this overreaction may have affected the blood vessels of the spine via thrombosis, hyperviscosity of the blood, or other unknown mechanisms by forming an immune complex.
Further research is needed, but considering the findings of the current case, attention should be paid to the possibility of Hirayama disease in the current circumstances of widespread Pfizer-BioNTech COVID-19 vaccination among Asian adolescents. Although no cases have been reported, if the above symptoms occur after vaccination, the possibility of Hirayama disease should be considered.