Hirayama’s disease associated with cervical deformity and spinal cord compression: a case report from Sweden

Hirayama's disease (HD) is a rare condition primarily affecting young males, characterized by muscle weakness and atrophy in the upper extremities. The exact cause of HD remains unknown, but a prevailing theory suggests that forward bending of the neck can compress the spinal cord, leading to the development of myelopathy. While conservative treatments like cervical collars are often the initial approach, surgical interventions have demonstrated promising outcomes for patients experiencing neurological progression.

• 1
  Surgical intervention (ACDF) in a 17-year-old male with HD and cervical deformity improved cervical lordosis but led to a transient C5 palsy.
• 2
  Reciprocal changes in the sub-axial spine occurred post-surgery, leading to deterioration in cervical alignment and myelomalacia at C4 after 3 years.
• 3
  Despite the complications, the patient reported less pain and stable neurological function, leading him to decline further surgical correction.