Hirayama Disease: A Case Report and Literatures Review_West China Medical Journal

Authors：

 LOUJin-he ¹ , YANGJuan ² ,  WANGJian ² , ZHANGBei ²

1. Department of Neurology, the First Affiliated Hospital, Chongqing Medical University, Chongqing 630014, P. R. China;
2. Department of Neurology, the Second People's Hospital of Chengdu, Chengdu, Sichuan 610017, P. R. China;

Corresponding author：

WANGJian, Email: jian.wx@163.com

Keywords：

Hirayama disease; Electromyography; Magnetic resonance imaging

DOI：

10.7507/1002-0179.20150181

Video：

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Objective To investigate the clinical features of Hirayama disease (HD). Methods We analyzed the clinical manifestations and assistant examination results of one patient with HD diagnosed in November, 2010. In addition, we reviewed the related literatures. Results The patient was a young man, and the main symptoms were bilateral hand weakness with muscular atrophy. The muscle bundles trembled when he stretched his fingers. The electromyography showed neurogenic damage of both the thenar muscles and the first interosseous muscles. Brain magnetic resonance imaging (MRI) showed normal; cervical spine flexion MRI showed thinning cervical spinal cord with spinal canal narrowing at C₅-C₇ plane. In addition, epidural fat gap widened at C₄-T₁ plane, and enhanced scan showed cord-like changes, patchy strengthening and blood flow void shadow. Conclusion When asymmetric distal upper limb muscle weakness with muscle atrophy appears in adolescents, HD should be considered. The combination of neuroelectrophysiological examination and cervical spine flexion MRI scan is helpful for diagnosis. Wearing cervical collar may slow disease progression.

Citation： LOUJin-he, YANGJuan, WANGJian, ZHANGBei. Hirayama Disease: A Case Report and Literatures Review. West China Medical Journal, 2015, 30(4): 622-625. doi: 10.7507/1002-0179.20150181 Copy

1.	唐江威, 赵丽冰, 郭鹏. 双侧上肢同时受累的平山病的诊断分析[J]. 齐齐哈尔医学院学报, 2008, 29(22):2745.
2.	Hirayamak K. Juvenile muscular atrophy of distal upper extremity (Hirayama disease)[J]. Intern Med, 2000, 39(4):283-290.
3.	Atchayaram N, Vasudev MK, Goel G. Familial monomelic amyotrophy (Hirayama disease):two brothers with classical flexion induced dynamic changes of the cervical dural sac[J]. Neurol India, 2010, 57(6):810-812.
4.	Chen CJ, Hsu HL, Tseng YC, et al. Hirayama flexion myelopathy:neutral-position Mr imaging findings-importance of loss of attachment[J]. Radiology, 2004, 231(1):39-44.
5.	Jakhere S, Wagh V. Hirayama's disease:the importance of flexion magnetic resonance imaging[J]. J Postgrad Med, 2011, 57(1):48-50.
6.	Misra UK, Kalita J, Mishra VN, et al. A clinical, magnetic resonance imaging, and survival motor neuron gene deletion study of Hirayama disease[J]. Arch Neurol, 2005, 62(1):120-123.
7.	Toma S, Shiozawa Z. Amyotrophic cervical myelopathy in adolescence[J]. J Neurol Neurosurg Psychiatry, 1995, 58(1):56-64.
8.	Hirayama K, Tokumaru Y. Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity[J]. Neurology, 2000, 54(10):1922-1926.
9.	Konno S, Goto S, Murakami M, et al. Juvenile amyotrophy of the distal upper extremity:pathologic findings of the dura mater and surgical management[J]. Spine (Phila Pa 1976), 1997, 22(5):486-492.
10.	郁冰冰, 张殿星, 姜庆军, 等. 自然位与过屈位磁共振成像对平山病的诊断价值[J]. 医学影像学杂志, 2009, 19(1):31-33.
11.	Sobue I, Saito N, Iida M, et al. Juvenile type of distal and segmental muscular atrophy of upper extremities[J]. Ann Neurol, 1978, 3(5):429-432.
12.	Schlegel U, Jerusalem F, Tackmann W, et al. Benign juvenile focal muscular atrophy of upper extremities:a familial case[J]. J Neurol Sci, 1987, 80(2/3):351-353.
13.	Tandan R, Sharma KR, Bradley WG, et al. Chronic segmental spinal muscular atrophy of upper extremities in identical twins[J]. Neurology, 1990, 40(2):236-239.
14.	Nalini A, Lokesh L, Ratnavalli E. Familial monomelic amyotrophy:a case report from India[J]. J Neurol Sci, 2004, 220(1/2):95-98.
15.	Yagihashi T, Hatori K, Ishii K, et al. Juvenile muscular atrophy of a unilateral upper extremity (Hirayama disease) in a patient with CHARGE syndrome[J]. Mol Syndromol, 2010, 1(2):91-94.
16.	Ito S, Kuwabara S, Fukutake T, et al. HyperIgEaemia in patients with juvenile muscular atrophy of the distal upper extremity (Hirayama disease)[J]. J Neurol Neurosurg Psychiatry, 2005, 76(1):132-134.
17.	Hou C, Han H, Yang X, et al. How does the neck flexion affect the cervical MRI features of Hirayama disease?[J]. Neurol Sci, 2012, 33(5):1101-1105.
18.	Lai V, Wong YC, Poon WL, et al. 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[J]. Eur J Radiol, 2011, 80(3):724-728.
19.	Gotkine M, Abraham A, Drory VE, et al. Dynamic MRI testing of the cervical spine has prognostic significance in patients with progressive upper-limb distal weakness and atrophy[J]. J Neurol Sci, 2014, 345(1/2):168-171.
20.	郭剑, 陈逊文, 曹正霖. 平山病的诊治进展[J]. 海南医学, 2010, 21(14):129-131.
21.	Tokumaru Y, Hirayama K. Cervical collar therapy for juvenile muscular atrophy of distal upper extremity (Hirayama disease):results from 38 cases[J]. Rinsho Shinkeigaku, 2001, 41(4/5):173-178.
22.	Watanabe K, Hasegawa K, Hirano T, et al. Anterior spinal decompression and fusion for cervical flexion myelopathy in young patients[J]. J Neurosurg Spine, 2005, 3(2):86-91.
23.	彭茂秀, 汤呈宣, 林利兴, 等. 非融合内固定手术治疗平山病的临床初步研究[J]. 临床骨科杂志, 2011, 14(4):378-380.

1. 唐江威, 赵丽冰, 郭鹏. 双侧上肢同时受累的平山病的诊断分析[J]. 齐齐哈尔医学院学报, 2008, 29(22):2745.
2. Hirayamak K. Juvenile muscular atrophy of distal upper extremity (Hirayama disease)[J]. Intern Med, 2000, 39(4):283-290.
3. Atchayaram N, Vasudev MK, Goel G. Familial monomelic amyotrophy (Hirayama disease):two brothers with classical flexion induced dynamic changes of the cervical dural sac[J]. Neurol India, 2010, 57(6):810-812.
4. Chen CJ, Hsu HL, Tseng YC, et al. Hirayama flexion myelopathy:neutral-position Mr imaging findings-importance of loss of attachment[J]. Radiology, 2004, 231(1):39-44.
5. Jakhere S, Wagh V. Hirayama's disease:the importance of flexion magnetic resonance imaging[J]. J Postgrad Med, 2011, 57(1):48-50.
6. Misra UK, Kalita J, Mishra VN, et al. A clinical, magnetic resonance imaging, and survival motor neuron gene deletion study of Hirayama disease[J]. Arch Neurol, 2005, 62(1):120-123.
7. Toma S, Shiozawa Z. Amyotrophic cervical myelopathy in adolescence[J]. J Neurol Neurosurg Psychiatry, 1995, 58(1):56-64.
8. Hirayama K, Tokumaru Y. Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity[J]. Neurology, 2000, 54(10):1922-1926.
9. Konno S, Goto S, Murakami M, et al. Juvenile amyotrophy of the distal upper extremity:pathologic findings of the dura mater and surgical management[J]. Spine (Phila Pa 1976), 1997, 22(5):486-492.
10. 郁冰冰, 张殿星, 姜庆军, 等. 自然位与过屈位磁共振成像对平山病的诊断价值[J]. 医学影像学杂志, 2009, 19(1):31-33.
11. Sobue I, Saito N, Iida M, et al. Juvenile type of distal and segmental muscular atrophy of upper extremities[J]. Ann Neurol, 1978, 3(5):429-432.
12. Schlegel U, Jerusalem F, Tackmann W, et al. Benign juvenile focal muscular atrophy of upper extremities:a familial case[J]. J Neurol Sci, 1987, 80(2/3):351-353.
13. Tandan R, Sharma KR, Bradley WG, et al. Chronic segmental spinal muscular atrophy of upper extremities in identical twins[J]. Neurology, 1990, 40(2):236-239.
14. Nalini A, Lokesh L, Ratnavalli E. Familial monomelic amyotrophy:a case report from India[J]. J Neurol Sci, 2004, 220(1/2):95-98.
15. Yagihashi T, Hatori K, Ishii K, et al. Juvenile muscular atrophy of a unilateral upper extremity (Hirayama disease) in a patient with CHARGE syndrome[J]. Mol Syndromol, 2010, 1(2):91-94.
16. Ito S, Kuwabara S, Fukutake T, et al. HyperIgEaemia in patients with juvenile muscular atrophy of the distal upper extremity (Hirayama disease)[J]. J Neurol Neurosurg Psychiatry, 2005, 76(1):132-134.
17. Hou C, Han H, Yang X, et al. How does the neck flexion affect the cervical MRI features of Hirayama disease?[J]. Neurol Sci, 2012, 33(5):1101-1105.
18. Lai V, Wong YC, Poon WL, et al. 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[J]. Eur J Radiol, 2011, 80(3):724-728.
19. Gotkine M, Abraham A, Drory VE, et al. Dynamic MRI testing of the cervical spine has prognostic significance in patients with progressive upper-limb distal weakness and atrophy[J]. J Neurol Sci, 2014, 345(1/2):168-171.
20. 郭剑, 陈逊文, 曹正霖. 平山病的诊治进展[J]. 海南医学, 2010, 21(14):129-131.
21. Tokumaru Y, Hirayama K. Cervical collar therapy for juvenile muscular atrophy of distal upper extremity (Hirayama disease):results from 38 cases[J]. Rinsho Shinkeigaku, 2001, 41(4/5):173-178.
22. Watanabe K, Hasegawa K, Hirano T, et al. Anterior spinal decompression and fusion for cervical flexion myelopathy in young patients[J]. J Neurosurg Spine, 2005, 3(2):86-91.
23. 彭茂秀, 汤呈宣, 林利兴, 等. 非融合内固定手术治疗平山病的临床初步研究[J]. 临床骨科杂志, 2011, 14(4):378-380.

West China Medical Journal

Hirayama Disease: A Case Report and Literatures Review

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