Abstract
The fourteen cases of Hirayama disease (HD) are presented in this article. HD is seldom disease characterized by juvenile muscular atrophy of upper extremities and benign course. All cases were diagnosed in the Research Center of Neurology (Moscow, Russia) during the year 2015–2017. Such methods as MRI (magnetic resonance imaging), EMG (electromyography), and NCS (nerve conduction studies) have been used to confirm diagnosis of HD. Transcranial magnetic stimulation was used to exclude upper motor neuron involvement in two cases. The original scale of neurological disturbances in HD has been proposed by authors to reveal correlations of HD severity with age of patients and duration of disease.
Most of patients with HD are young males with common clinical signs. Detected MRI and EMG data were also comparable with previous publications. Independence of HD severity from age and duration of the disease may be the result of individual physical characteristics of dura mater and other structures of the cervical vertebra. In some our cases, amyotrophic lateral sclerosis and other neurological disorders were misdiagnosed before. In view of different prognosis in these pathologies and possible correction of HD, early diagnosis is very important.
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Hirayama K, Toyokura YTT (1959) Juvenile muscular atrophy of unilateral upper extremity: a new clinical entity. PsychiatrNeurolJpn 61:2190–2198
Hirayama K, Tomonaga M, Kitano K, Yamada T, Kojima S, Arai K (1987) Focal cervical poliopathy causing juvenile muscular atrophy of distal upper extremity: a pathological study. J Neurol Neurosurg Psychiatry 50(3):285–290
Mukai E, Matsuo T, Muto T, Takahashi A, Sobue I (1987) Magnetic resonance imaging of juvenile-type distal and segmental muscular atrophy of the upper extremities. RinshoShinkeigaku 27(1):99–107
Kikuchi S, Tashiro K, Kitagawa M, Iwasaki Y, Abe H (1987) A mechanism of juvenile muscular atrophy localized in the hand and forearm (Hirayama’s disease)--flexion myelopathy with tight dural canal in flexion. RinshoShinkeigaku 27(4):412–419
Tashiro K, Kikuchi S, Itoyama Y, Tokumaru Y, Sobue G, Mukai E, Akiguchi I, Nakashima K, Kira JI, Hirayama K (2006) Nationwide survey of juvenile muscular atrophy of distal upper extremity (Hirayama disease) in Japan. Amyotroph Lateral Scler 7(1):38–45. https://doi.org/10.1080/14660820500396877
Raval M, Kumari R, Dung AAD, Guglani B, Gupta N, Gupta R (2010) MRI findings in Hirayama disease. Indian J Radiol Imaging 20(4):245–249. https://doi.org/10.4103/0971-3026.73528
Kang J-S, Jochem-Gawehn S, Laufs H, Ferbert A, Vieregge P, Ziemann U (2011) Hirayama disease in Germany: case reports and review of the literature. Nervenarzt 82(10):1264–1272. https://doi.org/10.1007/s00115-011-3320-9
Finsterer J, Löscher W, Wanschitz J, Baumann M, Quasthoff S, Grisold W (2013) Hirayama disease in Austria. Joint Bone Spine 80(5):503–507. https://doi.org/10.1016/j.jbspin.2012.10.013
Correia de Sá M, Costa H, Castro S, Vila Real M (2013) A Portuguese case of Hirayama disease. BMJ Case Rep 2013:bcr2013200645. https://doi.org/10.1136/bcr-2013-200645
Foster E, Tsang BK-T, Kam A, Storey E, Day B (2015) Hill A. Hirayama disease. J ClinNeurosci 22(6):951–954. https://doi.org/10.1016/j.jocn.2014.11.025
Lehman VT, Luetmer PH, Sorenson EJ, Carter RE, Gupta V, Fletcher GP, Hu LS, Kotsenas AL (2013) Cervical spine MR imaging findings of patients with Hirayama disease in North America: a multisite study. AJNR Am J Neuroradiol 34(2):451–456. https://doi.org/10.3174/ajnr.A3277
Zhou B, Chen L, Fan D, Zhou D (2010) Clinical features of Hirayama disease in mainland China 11(1–2):133–9. https://doi.org/10.3109/17482960902912407
Yoo SD, Kim H-S, Yun DH, Kim DH, Chon J, Lee SA, Lee SY, Han YJ (2015) Monomelicamyotrophy (hirayama disease) with upper motor neuron signs: a case report. Ann Rehabil Med 39(1):122–127. https://doi.org/10.5535/arm.2015.39.1.122
Nalini A, Gourie-Devi M, Thennarasu K, Ramalingaiah AH (2014) Monomelic amyotrophy: clinical profile and natural history of 279 cases seen over 35 years (1976–2010). Amyotroph Lateral Scler Front Degener 15(5–6):457–465
Hou C, Han H, Yang X, Xu X, Gao H, Fan D, Fu Y, Sun Y, Liu B (2012) How does the neck flexion affect the cervical MRI features of Hirayama disease? Neurol Sci 33(5):1101–1105. https://doi.org/10.1007/s10072-011-0912-x
Kraushaar G, Patel R, Stoneham GW (2005) West Nile virus: a case report with flaccid paralysis and cervical spinal cord: MR imaging findings. Am J Neuroradiol 26:26–29
Masson C, Pruvo JP, Meder JF et al (2004) Spinal cord infarction: clinical and magnetic resonance imaging findings and short term outcome. J Neurol Neurosurg Psychiatry 75(10):1431–1435
Novy J, Carruzzo A, Maeder P et al (2006) Spinal cord ischemia: clinical and imaging patterns, pathogenesis, and outcomes in 27 patients. Arch Neurol 63(8):1113–1120
Zheng C, Zhu D, Lu F, Zhu Y, Ma X, Xia X, Weber R, Jiang J (2017) Compound muscle action potential decrement to repetitive nerve stimulation between Hirayama disease and amyotrophic lateral sclerosis. J ClinNeurophysiol 34(2):119–125. https://doi.org/10.1097/WNP.0000000000000336
Goel A, Dhar A, Shah A (2017) Multilevel spinal stabilization as a treatment for Hirayama disease: report of an experience with five cases. World Neurosurg 99:186–191. https://doi.org/10.1016/j.wneu.2016.11.143
Singh RJ, Preethish-Kumar V, Polavarapu K, Vengalil S, Prasad C, Nalini A (2017) Reverse split hand syndrome: dissociated intrinsic hand muscle atrophy pattern in Hirayama disease/brachial monomelicamyotrophy. Amyotroph Lateral Scler Frontotemporal Degener 18(1–2):10–16. https://doi.org/10.1080/21678421.2016.1223140
Liao MF, Chang HS, Chang KH, Ro LS, Chu CC, Kuo HC, Lyu RK (2016) Correlations of clinical, neuroimaging, and electrophysiological features in Hirayama disease. Medicine (Baltimore) 95(28):e4210. https://doi.org/10.1097/MD.0000000000004210
Agundez M, Rouco I, Barcena J, Mateos B, Barredo J, Zarranz JJ (2015) Hirayama disease: is surgery an option? Neurologia 30(8):502–509. https://doi.org/10.1016/j.nrl.2013.05.005
Ding Y, Rong D, Wang X, Li C (2015) To evaluate the cervical spine curvature and growth rate for studying the pathogenesis of Hirayama disease in adolescents. ZhonghuaNeiKeZaZhi 54(8):721–724
Khadilkar S, Patel B, Bhutada A, Chaudhari C (2015) Do longer necks predispose to Hirayama disease? A comparison with mimics and controls. J NeurolSci 359(1–2):213–216. https://doi.org/10.1016/j.jns.2015.11.005
Shao M, Yin J, Lu F, Zheng C, Wang H, Jiang J (2015) The quantitative assessment of imaging features for the study of Hirayama disease progression. Biomed Res Int 2015:803148. https://doi.org/10.1155/2015/803148
Foster E, Tsang BK, Kam A, Storey E, Day B, Hill A (2015) Hirayama disease. J Clin Neurosci 22(6):951–954. https://doi.org/10.1016/j.jocn.2014.11.025
Abraham A, Gotkine M, Drory VE, Blumen SC (2013) Effect of neck flexion on somatosensory and motor evoked potentials in Hirayama disease. J Neurol Sci 334(1–2):102–105. https://doi.org/10.1016/j.jns.2013.07.2519
Matamala JM, Geevasinga N, Huynh W, Dharmadasa T, Howells J, Simon NG, Menon P, Vucic S, Kiernan MC (2017) Cortical function and corticomotoneuronal adaptation in monomelic amyotrophy. Clin Neurophysiol 128(8):1488–1495
Brooks BR, Miller RG, Swash M, Munsat TL (2000) El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 1(5):293–299
de Carvalho M, Dengler R, Eisen A, England JD, Kaji R, Kimura J, Mills K, Mitsumoto H, Nodera H, Shefner J, Swash M (2008) Electrodiagnostic criteria for diagnosis of ALS. Clin Neurophysiol 119(3):497–503
Boelmans K, Kaufmann J, Schmelzer S, Vielhaber S, Kornhuber M, Münchau A, Zierz S, Gaul C (2013) Hirayama disease is a pure spinal motor neuron disorder—a combined DTI and transcranial magnetic stimulation study. J Neurol 260(2):540–548
Hirayama K (2000) Juvenile muscular atrophy of distalupper extremity (Hirayama disease). Intern Med 39:283–290. https://doi.org/10.2169/internalmedicine.39.283
Cortese R, Gerevini S, Dicuonzo F, Zoccolella S, Simone IL (2015) Hirayama disease: the importance of an early diagnosis. Neurol Sci 36(6):1049–1050. https://doi.org/10.1007/s10072-015-2080-x
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Rosliakova, A., Zakroyshchikova, I., Bakulin, I. et al. Hirayama disease: analysis of cases in Russia. Neurol Sci 40, 105–112 (2019). https://doi.org/10.1007/s10072-018-3588-7
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DOI: https://doi.org/10.1007/s10072-018-3588-7