Iranian Journal of Neurology 2017. 16(3):136-145.

Bimelic symmetric Hirayama disease: Spectrum of magnetic resonance imaging findings and comparative evaluation with classical monomelic amyotrophy and other motor neuron disease
Deb Kumar Boruah, Shantiranjan Sanyal, Arjun Prakash, Sashidhar Achar, Dhabal D. Dhingani, Binod Sarma


Background: The aim of the study was to evaluate the magnetic resonance imaging (MRI) findings in bilateral symmetrical Hirayama disease and find out MRI features which are probably more indicative of symmetrical Hirayama disease, thereby help in differentiating this entity from other motor neuron disease (MND).

Methods: This prospective as well as retrospective study was carried out from December 2010 to September 2016 in a tertiary care center of northeast India on 92 patients with Hirayama disease. Only 19 patients having bilateral symmetric upper limb involvement at the time of presentation were included in this study sample.

Results: Nineteen patients, who constituted 20.6% of 92 patients of clinical and flexion MRI confirmed Hirayama disease were found to have bilateral symmetrical wasting and weakness of distal upper limb muscles at the time of presentation. Mean ± standard deviation (SD) age of onset of the disease process was 21.7 ± 3.8 years with mean ± SD duration of illness of 3.6 ± 1.3 years. MRI revealed lower cervical cord flattening in 13 (68.4%) patients which was symmetrical in 6 (31.6%) patients and asymmetrical in 7 (36.8%) patients. In the majority of these patients, T2-weighted images (T2WI) cervical cord hyperintensities were found extending from C5 to C6 vertebral level. Seven (36.8%) patients in our study showed bilateral symmetric T2WI hyperintensities in anterior horn cells (AHC).

Conclusion: Bilateral symmetrical involvement of Hirayama disease is an uncommon presentation. Symmetrical cervical cord flattening, T2WI cord and/or bilateral AHC hyperintensities were the major MRI findings detected. Flexion MRI demonstrated similar findings in both bimelic amyotrophy and classical unilateral amyotrophy. However, flexion MRI produced some distinguishing features more typical for bilateral symmetrical Hirayama disease which help to differentiate it from other MNDs.


Monomelic Amyotrophy; Wasting; Lamino-Dural Space; Anterior Horn Cells; Amyotrophic Lateral Sclerosis

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Hirayama K, Toyokura Y, Tsubaki T. Juvenile muscular atrophy unilateral upper extremity a new clinical entity. Psychiatr Neurol Jpn 1959; 61: 2190-2197

Biondi A, Dormont D, Weitzner I Jr, Bouche P, Chaine P, Bories J. AJNR Am J Neuroradiol 1989;10:263‑8.

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

Di Muzio A, Delli Pizzi C. Lugaresi A, Ragno M, Uncini A. Benign monomelic amyotrophy of lower limb: a rare entity with a characteristic muscular CT. J Neurol Sci.1994; 126:153-61.

Guo XM, Qin XY, Huang C. Neuroelectrophysiological characteristics of Hirayama disease: report of 14 cases.Chin Med J (Engl) 2012;125:2440-3.

Pradhan S. Bilaterlly symmetric form of Hirayama disease. Neurology 2009;72:2083-9

Tataroglu C, Bagdatoglu Cl, Apaydin Demir F, Celikbas H and Koksel T. 'Hirayama's disease: a case report', Amyotrophic Lateral Sclerosis, 2003; 4:4,264- 265.

Nascimento OJM, De Freitas MRG. Non-progressive juvenile spinal muscular atrophy of the distal upper limb (Hirayama’s disease): a clinical variant of the benign monomelic amyotrophy. Arq. Neuro-Psiquiatr 2000; 58: 814–819.

Tashiro K, Kikuchi S, Itoyama Y, Tokumaru Y, Sobue G, Mukai E et al. Nationwide survey of juvenile muscular atrophy of distal upper extremity (Hirayama disease) in Japan. Amyotroph Lateral Scler. 2006;7(1):38-45.

Chen TH, Hung CH, Hsieh TJ, Lu SR, Yang SN, Jong YJ.Symmetric Atrophy of Bilateral Distal Upper Extremities and HyperIgEaemia in a Male Adolescent With Hirayama Disease. Journal of Child Neurology 2010 ; 25 (3): 371-374

Donofrio PD. Monomelic amyotrophy. AAEM case report; Muscle Nerve 1994; 17: 1129–1134.

Preethish-Kumar V, Nalini A, Singh RJ, Saini J, Prasad C, Polavarapu K et al. Distal bimelic amyotrophy (DBMA): Phenotypically distinct but identical on cervical spine MR imaging with brachial monomelic amyotrophy/Hirayama disease. Amyotroph Lateral Scler Frontotemporal Degener. 2015;16(5-6):338-44.

Gourie-Devi M, Suresh TG, Shankar SK. Monomelic amyotrophy. Arch Neurol 1994;41: 388-394.

Nalini A, Gourie-Devi M, Thennarasu K, Ramalingaiah AH. Monomelic amyotrophy: clinical profile and natural history of 279 cases seen over 35 years (1976-2010). Amyotroph Lateral Scler Frontotemporal Degener.2014;15: 457-65.

Chen CJ, Hsu HL, Tseng YC, Lyu RK, Chen CM, Huang YC, et al. Hirayama flexion myelopathy: neutral-position MR imaging findings-importance of loss of attachment. Radiology 2004; 231(1): 39-44.

Foster E, Tsang BK, Kam A, Stark RJ.Mechanisms of upper limb amyotrophy in spinal disorders. J Clin Neurosci 2014;21(7): 1209-14.

Gotkine M, Abraham A, Drory VE, Argov Z, Gomori JM, Blumen SC.Dynamic MRI testing of the cervical spine has prognostic significance in patients with progressive upper-limbdistal weakness and atrophy. J Neurol Sci 2014; 345(1-2): 168-71.

Lehman VT, Luetmer PH, Sorenson EJ, Carter RE, Gupta V, Fletcher GP, et al.Cervical spine MR imaging findings of patients with Hirayama disease in North America: A multis ite study. AJNR Am J Neuroradiol 2013; 34(2): 451-6.

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;80(3): 724-8.

Zhou B, Chen L, Fan D, Zhou D. Clinical features of Hirayama disease in mainland China. Amyotroph Lateral Scler 2010;11(1-2): 133-9.

Waragai M. MRI and clinical features in amyotrophic lateral sclerosis.Neuroradiology 1997; 39(12): 847-51.

Agosta F, Chio A, Cosottini M, De SN, Falini A, Mascalchi M, et al. The present and the future of neuroimaging in amyotrophic lateral sclerosis. AJNR Am J Neuroradiol 2010; 31(10): 1769-77.

Kolb SJ, Kissel JT. Spinal muscular atrophy: A timely review. Arch Neurol 2011; 68(8): 979-84.

Tokumaru Y, Hirayama K. [Cervical collar therapy for juvenile muscular atrophy of distal upper extremity (Hirayama disease): Results from 38 cases]. Rinsho Shinkeigaku 2001; 41(4-5): 173-8.

Chiba S, Yonekura K, Nonaka M, Imai T, Matumoto H, Wada T. Advanced Hirayama disease with successful improvement of activities of daily living by operative reconstruction. Intern Med2004; 43(1): 79-81.

Ito H, Takai K, Taniguchi M. Cervical duraplasty with tenting sutures via laminoplasty for cervical flexion myelopathy in patients with Hirayama disease: Successful decompression of a "tight dural canal in flexion" without spinal fusion. J Neurosurg Spine 2014;21(5): 743-52.


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