Hirayama disease

Hirayama disease
Other namesJuvenile muscular atrophy of distal upper extremity, Hirayama disease, spinal muscular atrophy juvenile nonprogressive
Cervical spine

Hirayama disease, also known as monomelic amyotrophy (MMA),[1][2] is a rare motor neuron disease first described in 1959 in Japan. Its symptoms usually appear about two years after adolescent growth spurt and is significantly more common in males, with an average age of onset between 15 and 25 years. Hirayama disease is reported most frequently in Asia but has a global distribution. It is typically marked by insidious onset of muscle atrophy of an upper limb, which plateaus after two to five years from which it neither improves nor worsens. There is no pain or sensory loss. It is not believed to be hereditary.[3][4][5][6]

Both the names for the disorder and its possible causes have been evolving since first reported in 1959. It is most commonly believed the condition occurs by asymmetrical compression of the cervical spinal column by the cervical dural sac, especially when the neck is flexed. However, the disease is uncommon and diagnosis is confused by several atypical reports.[7][8][9]

  1. ^ Lay, Sara; Gudlavalleti, Aashrai; Sharma, Sandeep (2024), "Hirayama Disease", StatPearls, Treasure Island (FL): StatPearls Publishing, PMID 29763088, retrieved 2024-06-29
  2. ^ Huang, Yen-Lin; Chen, Chi-Jen (November 2011). "Hirayama disease". Neuroimaging Clinics of North America. 21 (4): 939–950, ix–x. doi:10.1016/j.nic.2011.07.009. ISSN 1557-9867. PMID 22032508.
  3. ^ "Monomelic amyotrophy | Genetic and Rare Diseases Information Center (GARD)". rarediseases.info.nih.gov. Retrieved 23 December 2016.
  4. ^ "Monomelic Amyotrophy Information Page". www.ninds.nih.gov. Retrieved 23 December 2016.
  5. ^ Ay, Halil (22 May 2017). "Hirayama disease (monomelic amyotrophy) clinically confused for carpal tunnel syndrome". Neuropsychiatric Disease and Treatment. 2017 (13): 1385–1388. doi:10.2147/NDT.S138315. PMC 5449119. PMID 28579784.
  6. ^ Liewluck, Teerin; Saperstein, David S (November 2015). "Progressive Muscular Atrophy". In Dimachkie, Mazen M.; Barohn, Richard J. (eds.). Motor Neuron Disease, An Issue of Neurologic Clinics. Elsevier Health Sciences. p. 766. ISBN 9780323413459.
  7. ^ Anuradha, S; Fanai, V (2016). "Hirayama Disease: A Rare Disease with Unusual Features". Case Reports in Neurological Medicine. 2016: 5839761. doi:10.1155/2016/5839761. PMC 5209606. PMID 28097028.
  8. ^ Turner, Martin R; Talbot, Kevin (June 2013). "Mimics and chameleons in motor neurone disease". Practical Neurology. 13 (3): 153–164. doi:10.1136/practneurol-2013-000557. PMC 3664389. PMID 23616620.
  9. ^ Hassan, KM; Sahni, H; Jha, A (April 2012). "Clinical and radiological profile of Hirayama disease: A flexion myelopathy due to tight cervical dural canal amenable to collar therapy". Annals of Indian Academy of Neurology. 15 (2): 106–112. doi:10.4103/0972-2327.94993. PMC 3345586. PMID 22566723.