A cervical myelopathy with a Hirayama disease-like phenotype

Chiara Cerami, Francesca Valentino, Federico Piccoli, Vincenzo La Bella

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)451-454
Number of pages4
JournalNeurological Sciences
Issue number6
Publication statusPublished - 2008


  • Cervical myelopathy
  • Hirayama disease
  • MRI
  • Muscular atrophy

ASJC Scopus subject areas

  • Dermatology
  • Clinical Neurology
  • Psychiatry and Mental health


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