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Two Cases of Hirayama Disease in a Pediatric Clinic

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주제분류신경과학

초록

We report two pediatric cases with Hirayama disease—a 16-year-old boy with a left wrist drop and a 14-year-old-boy with weakness and muscle atrophy of right hand. Motor nerve conduction study revealed decreased motor nerve action potential amplitudes in the ulnar nerve and radial nerve of the affected hands. The former patient showed normal magnetic resonance imaging (MRI) of the cervical spine, but the latter showed mild, asymmetric thinning of the anterior spinal cord at levels C5 to C7. Following active rehabilitation and avoidance of neck flexion, no further progression of neurological findings was noticed. These clinical findings were typical of Hirayama disease. We show that timely and accurate diagnosis for Hirayama disease is possible with awareness of disease history, careful physical examination, and the use of neurophysiological studies and MRI studies.

Abstract

We report two pediatric cases with Hirayama disease—a 16-year-old boy with a left wrist drop and a 14-year-old-boy with weakness and muscle atrophy of right hand. Motor nerve conduction study revealed decreased motor nerve action potential amplitudes in the ulnar nerve and radial nerve of the affected hands. The former patient showed normal magnetic resonance imaging (MRI) of the cervical spine, but the latter showed mild, asymmetric thinning of the anterior spinal cord at levels C5 to C7. Following active rehabilitation and avoidance of neck flexion, no further progression of neurological findings was noticed. These clinical findings were typical of Hirayama disease. We show that timely and accurate diagnosis for Hirayama disease is possible with awareness of disease history, careful physical examination, and the use of neurophysiological studies and MRI studies.

참고문헌 (19)

  1. "Psychiatr Neurol Jpn", 1959, 2190~2198
  2. Hirayama K, "Non-progressive juvenile spinal muscular atrophy f the distal upper limb (Hirayama's disease)", pp.107~120 (1991), Elsevier
  3. Huang YC, "A clinical study of Hirayama disease in Taiwan", Muscle Nerve, 37, pp.576~582 (2008)[DOI]
  4. Zhou B, "Clinical features of Hirayama disease in mainland China", Amyotroph Lateral Scler, 11, pp.133~139 (2010)[DOI]
  5. Chen CJ, "Hirayama disease: MR diagnosis", AJNR Am J Neuroradiol, 19, pp.365~368 (1998)
  6. Pradhan S, "Bilaterally symmetric form of Hirayama disease", Neurology, 72, pp.2083~2089 (2009)[DOI]
  7. 김진일, "Hirayama Disease with Proximal Involvement", Journal of Korean Medical Science, 31(10), pp.1664~1667 (2016), 대한의학회
  8. Hirayama K, "Juvenile muscular atrophy of distal upper extremity (Hirayama disease): focal cervical ischemic poliomyelopathy", Neuropathology, 20(Suppl), pp.S91~S94 (2000)
  9. Kikuchi S, "A mechanism of juvenile muscular atrophy localized in the hand and forearm (Hirayama's disease)--flexion myelopathy with tight dural canal in flexion", Rinsho Shinkeigaku, 27, pp.412~419 (1987)
  10. Lyu RK, "Electrophysiological features of Hirayama disease", Muscle Nerve, 44, pp.185~190 (2011)[DOI]
  11. Desai JA, "Teaching neuroimages: anterior horn cell hyperintensity in Hirayama disease", Neurology, 77, pp.e73~ (2011)[DOI]
  12. Hirayama K, "Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity", Neurology, 54, pp.1922~1926 (2000)[DOI]
  13. Toma S, "Amyotrophic cervical myelopathy in adolescence", J Neurol Neurosurg Psychiatry, 58, pp.56~64 (1995)[DOI]
  14. Foster E, "Hirayama disease", J Clin Neurosci, 22, pp.951~954 (2015)[DOI]
  15. Gandhi D, "Case 68: Hirayama disease", Radiology, 230, pp.692~696 (2004)[DOI]
  16. 유승돈, "Monomelic Amyotrophy (Hirayama Disease) With Upper Motor Neuron Signs: A Case Report", Annals of Rehabilitation Medicine, 39(1), pp.122~127 (2015), 대한재활의학회
  17. Jung HJ, "Hirayama disease presenting as isolated triceps atrophy", J Clin Neurosci, 20, pp.1450~1451 (2013)[DOI]
  18. 이경희, "Clinical Experiences of Uncommon Motor Neuron Disease: Hirayama Disease", Neurospine, 13(3), pp.170~172 (2016), 대한척추신경외과학회[DOI]
  19. 권오현, "A Korean Case of Juvenile Muscular Atrophy of Distal Upper Extremity (Hirayama Disease) with Dynamic Cervical Cord Compression", Journal of Korean Medical Science, 19(5), pp.768~771 (2004), 대한의학회

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