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Hirayama disease: analysis of cases in Russia

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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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References

  1. Hirayama K, Toyokura YTT (1959) Juvenile muscular atrophy of unilateral upper extremity: a new clinical entity. PsychiatrNeurolJpn 61:2190–2198

    Google Scholar 

  2. 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

    Article  CAS  Google Scholar 

  3. 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

    CAS  Google Scholar 

  4. 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

    CAS  Google Scholar 

  5. 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

    Article  PubMed  Google Scholar 

  6. 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

    Article  PubMed  PubMed Central  Google Scholar 

  7. 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

    Article  PubMed  Google Scholar 

  8. 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

    Article  PubMed  Google Scholar 

  9. 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

    Article  PubMed  PubMed Central  Google Scholar 

  10. 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

    Article  Google Scholar 

  11. 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

    Article  CAS  PubMed  Google Scholar 

  12. 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

  13. 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

    Article  PubMed  PubMed Central  Google Scholar 

  14. 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

    Article  Google Scholar 

  15. 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

    Article  PubMed  Google Scholar 

  16. 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

    PubMed  Google Scholar 

  17. 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

    Article  CAS  Google Scholar 

  18. 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

    Article  Google Scholar 

  19. 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

    Article  Google Scholar 

  20. 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

    Article  PubMed  Google Scholar 

  21. 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

    Article  PubMed  Google Scholar 

  22. 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

    Article  Google Scholar 

  23. 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

    Article  CAS  PubMed  Google Scholar 

  24. 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

    Google Scholar 

  25. 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

    Article  Google Scholar 

  26. 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

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. 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

    Article  PubMed  Google Scholar 

  28. 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

    Article  CAS  PubMed  Google Scholar 

  29. 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

    Article  Google Scholar 

  30. 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

    Article  CAS  Google Scholar 

  31. 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

    Article  Google Scholar 

  32. 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

    Article  Google Scholar 

  33. Hirayama K (2000) Juvenile muscular atrophy of distalupper extremity (Hirayama disease). Intern Med 39:283–290. https://doi.org/10.2169/internalmedicine.39.283

    Article  CAS  PubMed  Google Scholar 

  34. 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

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Research Center of Neurology, Moscow, Russia

    Anna Rosliakova, Inessa Zakroyshchikova, Ilya Bakulin, Rodion Konovalov, Elena Kremneva, Marina Krotenkova, Natalya Suponeva & Mariya Zakharova

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  1. Anna Rosliakova
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  2. Inessa Zakroyshchikova
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Correspondence to Anna Rosliakova.

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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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