A cervical myelopathy with a Hirayama disease-like phenotype

Neurol Sci. 2008 Dec;29(6):451-4. doi: 10.1007/s10072-008-1058-3. Epub 2008 Dec 4.

Abstract

A 21-year-old man with a muscular atrophy of the left distal upper extremity is presented. The disorder had been progressive over a few years, showing an exacerbation of the hand's weakness when the patient worked in a chilled environment (i.e., in a cold room). The patient's diagnostic work-up was extensive and the MRI documented the presence of a cervical myelopathy, associated to an inversion of the physiological lordosis at the C5-C6 level, with a phenotype highly resembling Hirayama disease. This case indirectly supports the debated hypothesis that juvenile amyotrophy of the upper limb (Hirayama disease) is actually a type of cervical myelopathy, with a likely ischaemic pathogenesis of the ventral horns.

Publication types

  • Case Reports

MeSH terms

  • Arm / innervation
  • Arm / pathology
  • Arm / physiopathology
  • Cervical Vertebrae / pathology*
  • Cervical Vertebrae / physiopathology
  • Cold Temperature / adverse effects
  • Cumulative Trauma Disorders / complications
  • Cumulative Trauma Disorders / pathology*
  • Cumulative Trauma Disorders / physiopathology
  • Disease Progression
  • Functional Laterality / physiology
  • Humans
  • Ischemia / etiology
  • Ischemia / pathology
  • Ischemia / physiopathology
  • Kyphosis / complications
  • Kyphosis / pathology*
  • Kyphosis / physiopathology
  • Magnetic Resonance Imaging
  • Male
  • Muscle Weakness / etiology
  • Muscle Weakness / pathology
  • Muscle Weakness / physiopathology
  • Muscle, Skeletal / innervation
  • Muscle, Skeletal / pathology
  • Muscle, Skeletal / physiopathology
  • Muscular Atrophy, Spinal / etiology
  • Muscular Atrophy, Spinal / pathology*
  • Muscular Atrophy, Spinal / physiopathology
  • Neural Pathways / injuries
  • Neural Pathways / pathology
  • Neural Pathways / physiopathology
  • Occupational Diseases / complications
  • Occupational Diseases / pathology*
  • Occupational Diseases / physiopathology
  • Spinal Cord / blood supply
  • Spinal Cord / pathology
  • Spinal Cord / physiopathology
  • Spinal Cord Compression / etiology
  • Spinal Cord Compression / pathology*
  • Spinal Cord Compression / physiopathology
  • Syndrome
  • Young Adult