Neurological Sciences

, Volume 40, Issue 1, pp 181–186 | Cite as

The reversible effect of neck flexion on the somatosensory evoked potentials in patients with Hirayama disease: a preliminary study

  • Jin-Sung Park
  • Jin Young Ko
  • Donghwi ParkEmail author
Original Article


The aim of this study was to examine and characterize the reversibility of the cervical somatosensory electrophysiological pathways during neutral and flexed neck positions. The parameters of somatosensory evoked potentials (SEPs) during neutral and flexed neck positions (N9, N13, and N20 SEP latencies; N9–N13 and N13–N20 inter-peak latencies; and the changes in N9–N13 and N13–N20 inter-peak latency during neutral and flexed neck positions) were measured in the patients with Hirayama disease (HD) and also in the healthy controls. In patients with HD, there was a significant difference in the mean value of N13–N20 inter-peak latency during the flexed neck position compared to that of the healthy controls (p < 0.05). In a multivariate logistic regression analysis, N13–N20 inter-peak latency during the flexed neck position significantly correlated with the presence of HD (p < 0.05). Collectively, in this cohort of patients with HD, the neck flexion of patients with HD showed a reversible effect on the SEP parameter, especially in N13–N20 inter-peak latency. Conventional diagnosis of HD is based on nerve conduction studies and electromyography along with a cervical flexion MRI, and our study suggests the possibility of an additional and cost-effective electrophysiological marker that may be helpful in the diagnosis of HD.


Hirayama Sensory evoked potentials Neck flexion MRI Motor neuron disease 


Funding information

This research was supported by Basic Science Research Program through the National Research Foundation of Korea (NRF) funded by the Ministry of Science, ICT and Future Planning (NRF-2017R1D1A1B03033127). This work was supported by the National Research Foundation of Korea (NRF) grant funded by the Korea government (Ministry of Science and ICT) (No. 2017R1C1B5076264).

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


  1. 1.
    Park JS, Park SP, Seo JG (2014) Hirayama disease with juvenile myoclonic epilepsy: a case report. Ann Indian Acad Neurol 17(3):358–360CrossRefGoogle Scholar
  2. 2.
    Gamez J, Pradhan S (2010) Bilaterally symmetric form of Hirayama disease. Neurology 74(4):345 author reply −6CrossRefGoogle Scholar
  3. 3.
    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):102–105CrossRefGoogle Scholar
  4. 4.
    Hirayama K, Tokumaru Y (2000) Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity. Neurology 54(10):1922–1926CrossRefGoogle Scholar
  5. 5.
    Shizukawa H, Imai T, Kobayashi N, Chiba S, Matsumoto H (1994) Cervical flexion-induced changes of motor evoked potentials by transcranial magnetic stimulation in a patient with Hirayama disease--juvenile muscular atrophy of unilateral upper extremity. Rinsho shinkeigaku = Clinical neurology 34(5):500–503PubMedGoogle Scholar
  6. 6.
    Lin MS, Kung WM, Chiu WT, Lyu RK, Chen CJ, Chen TY (2010) Hirayama disease. J Neurosurg Spine 12(6):629–634CrossRefGoogle Scholar
  7. 7.
    Elsheikh B, Kissel JT, Christoforidis G, Wicklund M, Kehagias DT, Chiocca EA, Mendell JR (2009) Spinal angiography and epidural venography in juvenile muscular atrophy of the distal arm "Hirayama disease". Muscle Nerve 40(2):206–212CrossRefGoogle Scholar
  8. 8.
    Hirayama K (2008) Juvenile muscular atrophy of unilateral upper extremity (Hirayama disease)--half-century progress and establishment since its discovery. Brain and nerve = Shinkei kenkyu no shinpo 60(1):17–29PubMedGoogle Scholar
  9. 9.
    Restuccia D, Rubino M, Valeriani M, Mirabella M, Sabatelli M, Tonali P (2003) Cervical cord dysfunction during neck flexion in Hirayama’s disease. Neurology 60(12):1980–1983CrossRefGoogle Scholar
  10. 10.
    Nalini A, Praveen-Kumar S, Ebenezer B, Ravishankar S, Subbakrishna DK (2008) Multichannel somato sensory evoked potential study demonstrated abnormalities in cervical cord function in brachial monomelic amyotrophy. Neurol India 56(3):368–373CrossRefGoogle Scholar
  11. 11.
    Polo A, Curro’ Dossi M, Fiaschi A, Zanette GP, Rizzuto N (2003) Peripheral and segmental spinal abnormalities of median and ulnar somatosensory evoked potentials in Hirayama’s disease. J Neurol Neurosurg Psychiatry 74(5):627–632CrossRefGoogle Scholar
  12. 12.
    Ammendola A, Gallo A, Iannaccone T, Tedeschi G (2008) Hirayama disease: three cases assessed by F wave, somatosensory and motor evoked potentials and magnetic resonance imaging not supporting flexion myelopathy. Neurol Sci 29(5):303–311CrossRefGoogle Scholar
  13. 13.
    Misra UK, Kalita J, Mishra VN, Phadke RV, Hadique A (2006) Effect of neck flexion on F wave, somatosensory evoked potentials, and magnetic resonance imaging in Hirayama disease. J Neurol Neurosurg Psychiatry 77(5):695–698CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Italia S.r.l., part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of NeurologySchool of Medicine, Kyungpook National UniversityDaeguKorea
  2. 2.Department of Rehabilitation MedicineSeoul National University Bundang HospitalSeongnam-siSouth Korea
  3. 3.Department of Rehabilitation MedicineDaegu Fatima HospitalDaeguSouth Korea

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