Tag Archive for: Monomelic amyotrophy

Volume 3 | Issue 2 | October 2022-March 2023 | page: 109-112 | Sharvin K Sheth, Amit C Jhala, Jay V Shah

DOI: https://doi.org/10.13107/bbj.2022.v03i02.048

Authors: Sharvin K Sheth [1], Amit C Jhala [1], Jay V Shah [2]

[1] Department of Spine Surgery, Chirayu Orthopaedic and Spine Hospital, Ahmedabad, Gujarat, India.
[2] Department of Radiology, Medimax Advance Radio Imaging, Ahmedabad, Gujarat, India.

Address of Correspondence

Dr. Amit C Jhala,
Consultant Spine Surgeon and Chief of Department of Spine Surgery, Chirayu Orthopaedic and Spine Hospital, Ahmedabad, Gujarat, India.
E-mail: acjhala@gmail.com

Abstract

Hirayama disease is a rare neurological condition and is characterized by a sporadic juvenile muscular atrophy of distal upper extremity in young males. The disease is more prevalent in Japan and other Asian countries, though a few cases have been reported in Western countries as well. It manifests as a self-limiting, gradually progressive atrophic weakness of forearm and hand. The anterior displacement of posterior dura during neck flexion leading to cervical cord atrophy has been hypothesized. We discuss a case of a 21-year-old male patient with progressive distal upper extremity weakness, diagnosed with Hirayama disease, and literature review for the same.
Keywords: Hirayama Disease, Juvenile muscular atrophy, Monomelic amyotrophy

References

1. Hirayama K, Tsubaki T, Toyokura Y, Okinaka S. Juvenile muscular atrophy of unilateral upper extremity. Neurology. 1963;13:373-80.
2. Sobue I, Saito M, Iida M, Ando K. Juvenile type of distal and segmental muscular atrophy of upper extremities. Ann Neurol. 1978;3:429–32.
3. Gourie-Devi M, Suresh TG, Shankar SK. Monomelic amyotrophy. Arch Neurol. 1984;4:388–94.
4. Pilgaard S. Unilateral juvenile muscular atrophy of upper limbs. Acta Orthop Scand 1968;39:327-31.
5. Compernolle T. A case of juvenile muscular atrophy confined to one upper limb. Eur Neurol. 1973;10(4):237-42.
6. Oryema J, Ashby P, Spiegel S. Monomelic atrophy. Can J Neurol Sci. 1990;17:124–30.
7. Donofrio PD. Monomelic amyotrophy. Muscle Nerve. 1994;17:1129–34.
8. Hirayama K. Juvenile muscular atrophy of distal upper extremity (Hirayama disease). Intern Med. 2000;39:283–90.
9. Hirayama K, Tokumaru Y. Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity. Neurology. 2000;54:1922–6.
10. Ojha R, Shahi S, Nepal G, Shakya A, Gajurel BP, Karn R, Rajbhandari R, Gautam N. The diagnostic quandary of magnetic resonance imaging-negative Hirayama disease: a case report. J Med Case Rep. 2020 Aug 21;14(1):133.
11. S. D. Yoo, H.-S. Kim, D. H. Yun et al., “Monomelic amyotrophy (Hirayama disease) with upper motor neuron signs: a case report,” Annals of Rehabilitation Medicine (2015);39(1):122-127.
12. Anuradha S, Fanai V. Hirayama Disease: A Rare Disease with Unusual Features. Case Rep Neurol Med. 2016;2016:5839761.
13. K. M. Hassan, H. Sahni, and A. Jha, “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 (2012); 15(2): 106-112.
14. K. Tashiro, S. Kikuchi, Y. Itoyama et al., “Nationwide survey of juvenile muscular atrophy of distal upper extremity (Hirayama disease) in Japan,” Amyotrophic Lateral Sclerosis (2006); 7(1):38-45.
15. Shruti Thakur, R.G. Sood, Anupam Jhobta, Dinesh Sharma, Sushma Makhaik, Neeti Aggarwal, Charu S. Thakur. “Hirayama disease in unilateral and bilateral forms-3 case reports”. The Egyptian Journal of Radiology and Nuclear Medicine (2013); 44(2):291-295.
16. Hirayama K, Tomonaga M, Kitano K, Yamada T, Kojima S, Arai K. Focal cervical poliopathy causing juvenile muscular atrophy of distal upper extremity: a pathological study. J Neurol Neurosurg Psychiatry. 1987 Mar;50(3):285-90.
17. Virmani V, Mohan PK. Non-familial, spinal segmental muscular atrophy in juvenile and young subjects. Acta Neurol Scand. 1985;72:336–40.
18. Tanaka M, Ishizu T, Ochi H, et al. Intrathecal upregulation of IFN- gamma and MIP-1beta in juvenile muscular atrophy of the distal upper extremity. J Neurol Sci 2008;275:74 –77.
19. Kikuchi S, Tashiro K, Kitagawa M, Iwasaki Y, Abe H. A mechanism of juvenile muscular atrophy localized in the hand and forearm (Hirayama’s disease): flexion myelopathy with tight dural canal in flexion (in Japanese). Clin Neurol (Tokyo). 1987 Apr;27(4):412-9.
20. Narayana Gowda BS, Mohan Kumar J, Basim PK. Hirayama’s Disease – A Rare Case Report with Review of Literature. J Orthop Case Rep. 2013 Jul-Sep;3(3):11-4.
21. Kato Y, Kataoka H, Ichihara K, Imajo Y, Kojima T, Kawano S, Hamanaka D, Yaji K, Taguchi T. Biomechanical study of cervical flexion myelopathy using a three-dimensional finite element method. J Neurosurg Spine. 2008 May;8(5):436-41.
22. Lai V, Wong YC, Poon WL, Yuen MK, Fu YP, Wong OW. Forward shifting of posterior dural sac during flexion cervical magnetic resonance imaging in Hirayama disease: an initial study on normal subjects compared to patients with Hirayama disease. Eur J Radiol. 2011 Dec;80(3):724-8.
23. Boruah DK, Prakash A, Gogoi BB, Yadav RR, Dhingani DD, Sarma B. The Importance of Flexion MRI in Hirayama Disease with Special Reference to Laminodural Space Measurements. AJNR Am J Neuroradiol. 2018 May;39(5):974-980.
24. Xu H, Shao M, Zhang F, Nie C, Wang H, Zhu W, Xia X, Ma X, Lu F, Jiang J. Snake-Eyes Appearance on MRI Occurs during the Late Stage of Hirayama Disease and Indicates Poor Prognosis. Biomed Res Int. 2019 Jan 13;2019:9830243.
25. Mizuno J, Nakagawa H, Inoue T, Hashizume Y. Clinicopathological study of “snake-eye appearance” in compressive myelopathy of the cervical spinal cord. J Neurosurg. 2003 Sep;99(2 Suppl):162-8.
26. Al-Hashel JY, Abdelnabi EA, Ibrahim Ismail I. Monomelic Amyotrophy (Hirayama Disease): A Rare Case Report and Literature Review. Case Rep Neurol. 2020 Sep 17;12(3):291-298.
27. Jin X, Jiang JY, Lu FZ, Xia XL, Wang LX, Zheng CJ. Electrophysiological differences between Hirayama disease, amyotrophic lateral sclerosis and cervical spondylotic amyotrophy. BMC Musculoskelet Disord. 2014 Oct 16;15:349.
28. MARIE, P., and FOIX, c.: L’atrophie isolhe non pro- gressive des petits muscles de la main (Tephromala- cie antCieure). Nouv. iconogr. de la SalpbriBre 25: 353 and 427, 1912.

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