Hirayama Disease: a Case Report with Late Onset

Main Article Content

Antônio Marco Duarte de Albuquerque
https://orcid.org/0000-0001-5143-6953
Sarah Câmara Ferreira
Sophia Alves de França Silva
Rodrigo Araújo de Melo

Abstract

The etiology of Hirayama's disease (HD) is still controversial, but the most accepted mechanism is the repetition of sudden neck flexion movements, which causes a flattening of the cervical spinal cord. It reaches the cells of the anterior tip of the medulla in the C7-T1 segments. 45-year-old patient with HD, diagnosed 1 year ago with typical atrophy of the left upper limb (LUL) associated with fasciculation and decreased osteotendinous reflexes in upper limbs (ULL). Due to the rarity of the disease, there are many underdiagnosed cases, which results in low epidemiological data reported. We are aware that the prevalence in males is explained by accelerated growth at puberty.

Article Details

How to Cite
Albuquerque, A. M. D. de, Ferreira, S. C., Silva, S. A. de F., & Melo, R. A. de. (2024). Hirayama Disease: a Case Report with Late Onset. Brazilian Journal of Case Reports, 4(4), 40–42. https://doi.org/10.52600/2763-583X.bjcr.2024.4.4.40-42
Section
Clinical Case Reports
Author Biographies

Antônio Marco Duarte de Albuquerque, Faculdade de Medicina de Olinda – FMO

Faculdade de Medicina de Olinda – FMO, Olinda, PE, Brazil.

Universidade Federal de Pernambuco, Recife, PE, Brazil.

Universidade de Pernambuco, Recife, PE, Brazil.

Sarah Câmara Ferreira, Faculdade de Medicina de Olinda – FMO

Faculdade de Medicina de Olinda – FMO, Olinda, PE, Brazil.

Sophia Alves de França Silva, Faculdade de Medicina de Olinda – FMO

Faculdade de Medicina de Olinda – FMO, Olinda, PE, Brazil.

Rodrigo Araújo de Melo, Faculdade de Medicina de Olinda

Faculdade de Medicina de Olinda – FMO, Olinda, PE, Brazil.

References

Saranya BG, Ayashi A, Ajay G. Hyariama Disease: Review on pathophysiology, clinical features, diagnosis and treatment. Rev Neurol. 2022;18(2):109-116. doi: 10.17925/UN.2022.18.2.109.

Gourie-Devi M, Suresh TG, Shankar SK. Monomelic amyotrophy. Arch Neuro. 1984;41:388-94.

Iyotsna Y, Dhanush A, Iyotsna, Sriram J, Afishan JS. Role of dynamic magnetic resonance imaging in Hirayama disease, a rare motor neuron disease. Cureus. [Date of Publication]. doi: 10.7759/cureus.31099.

Srivbastava SK, Marathe N, Raj A, Bhosale S, Dhole K. Surgical management of Hirayama disease. A rare entity with unusual clinical features. Asian J Neurosurg. 2022;15:405-08. doi: 10.4103/ajns.AJNS_291_19.

Singh R, Hudson M, Meyer JH, Neal MT, Pate NP. Surgical treatment of spinal cord compression due to Hirayama disease. Illustrative case. J Neurosurg Case Lessons. 2022;3:10. doi: 10.3171/CASE21697.

Wang XN, Cui LY, Liu MS, et al. A clinical neurophysiology study of Hirayama disease. Chin Med J. 2012;125(06):1115-1120.