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A Case Presentation on a Patient with the Familial Form of Monomelic Amyotrophy

Author

Listed:
  • Binod Wagle
  • MBBS
  • Hafsa Lodhi
  • Margaret Finn

    (University of Missouri-Kansas City, USA)

Abstract

Monomelic Amyotrophy, also known as Hirayama disease, is a rare motor neuron disease that causes a painless, asymmetric weakness and atrophy in the distal upper extremities. The cause is unknown and it typically affects Asian males in their second or third decade. We report a case of a 21-year-old Bhutanese refugee with a three-year history of weakness and atrophy in his left upper extremity associated with a hand tremor and significant family history of similar findings in a sibling. Usually the disease is sporadic in nature, but the case we report is a familial presentation. This presentation was consistent with the clinical diagnosis and was accompanied by diagnostic imaging. Magnetic resonance imaging showed asymmetric atrophy of the spinal cord. With flexion positioning on both axial and sagittal planes, tightening of the dura along dorsal aspect of thecal sac from C3 to thoracic segments was observed.

Suggested Citation

  • Binod Wagle & MBBS & Hafsa Lodhi & Margaret Finn, 2017. "A Case Presentation on a Patient with the Familial Form of Monomelic Amyotrophy," Open Access Journal of Neurology & Neurosurgery, Juniper Publishers Inc., vol. 5(4), pages 47-49, August.
    • Handle: RePEc:adp:joajnn:v:5:y:2017:i:4:p:47-49
    DOI: 10.19080/OAJNN.2017.05.555666
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