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Diagnosis of Benign Monomelic Amyotrophy

양성 국소 근위축증의 진단

  • Byun, Justin (Department of Rehabilitation Medicine, Gangnam Severance Hospital, Rehabilitation Institute of Neuromuscular Disease, Yonsei University College of Medicine) ;
  • Bang, Meyong Hwan (Department of Physical Medicine and Rehabilitation, National Health Insurance Service Ilsan Hospital) ;
  • Park, Jung Hyun (Department of Rehabilitation Medicine, Gangnam Severance Hospital, Rehabilitation Institute of Neuromuscular Disease, Yonsei University College of Medicine)
  • 변저스틴 (연세대학교 의과대학 재활의학교실) ;
  • 방명환 (국민건강보험 일산병원 재활의학과) ;
  • 박중현 (연세대학교 의과대학 재활의학교실)
  • Received : 2019.10.16
  • Accepted : 2020.06.01
  • Published : 2020.12.31

Abstract

Benign monomelic amyotrophy (BMA) is a benign motor neuron disease in which amyotrophic change is confined to either the upper or the lower extremities. Numerous cases of BMA have been reported from Japan and India. However, only a few cases have been reported from other regions, including South Korea. Here we report a rare case of late-onset BMA in Korean male using conventional diagnostic approach with magnetic resonance imaging and electromyography. The patient received ten sessions of manual therapy, which focused on strengthening of the left ankle. At two-month follow up, weakness was still isolated to the patient's left ankle. There were no signs of disease progression.

Keywords

References

  1. Biondi A, Dormont D, Weitzner I Jr, Bouche P, Chaine P, Bories J. MR Imaging of the cervical cord in juvenile amyotrophy of distal upper extremity. AJNR Am J Neuroradiol. 1989; 10: 263-268
  2. Gourie-Devi M, Suresh TG, Shankar SK. Monomelic amyotrophy. Arch Neurol. 1984; 41: 388-394 https://doi.org/10.1001/archneur.1984.04050160050015
  3. Di Muzio A, Delli PC, 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: 153-161 https://doi.org/10.1016/0022-510X(94)90266-6
  4. Hirayama K, Tohocura Y, Tsubaki T. Juvenile muscular atrophy of unilateral extremity: a new entity. Jpn J Psychiatry Neurol 1959; 61: 2190-2197
  5. Boruah DK, Sanyal S, Prakash A, et al. Bimelic symmetric Hirayama disease: Spectrum of magnetic resonance imaging findings and comparative evaluation with classical monomelic amyotrophy and other motor neuron disease. Iran J Neurol 2017; 16: 136-145
  6. Peiris JB, Seneviratne KN, Wickremasinghe HR, Gunatilake SB, Gamage R. Non-familial juvenile distal spinal atrophy of the upper extremity. J Neurol Neurosurg Psychiatry 1989; 52: 314-319 https://doi.org/10.1136/jnnp.52.3.314
  7. Marcos RG, De Freitas MR, Osvaldo J, Nascimento OJ. Benign monomelic amyotrophy: a study of twenty-one cases. Arq Neuropsiquiatr 2000; 58: 808-813 https://doi.org/10.1590/S0004-282X2000000500003
  8. Xu X, Han H, Gao H, Hou C, Fan D, Fu Y, et al. The increased range of cervical flexed motion detected by radiographs in Hirayama disease. Eur J Radiol 2011; 78: 82-86 https://doi.org/10.1016/j.ejrad.2010.08.012
  9. Mulder DW, Rosenbaum RA, Layton DD Jr. Late progression of poliomyelitis or form fruste of Amyotrophic lateral sclerosis. Mayo Clinic Proc 1972; 47: 756-761
  10. Konno S, Goto S, Murakami M, Mochizuki M, Motegi H, Moriya H. Juvenile amyotrophy of the distal upper extremity: pathologic findings of the dura mater and surgical management. Spine 1997; 22: 486-492 https://doi.org/10.1097/00007632-199703010-00004