Hirayama Disease: An Important Cause of Focal Hand Weakness in Young Adults

This article describes three cases of Hirayama disease, a rare condition causing hand weakness in young adults due to spinal cord compression. Early recognition is crucial to avoid unnecessary tests and treatments that could worsen the condition. The cases highlight how delayed diagnosis can lead to unnecessary surgery and genetic testing, potentially affecting clinical outcomes. Conversely, early diagnosis and conservative treatment can lead to improvement. Key indicators for Hirayama disease include onset in adolescence, male sex, hand weakness, and specific findings on MRI with neck flexion. Recognizing these signs can aid in timely diagnosis and management.

• 1
  Delayed diagnosis of Hirayama disease can lead to unnecessary interventions, such as surgery and genetic testing, potentially worsening the patient's condition.
• 2
  Early diagnosis of Hirayama disease, coupled with conservative measures like cervical collar immobilization and physical therapy, can lead to symptom improvement.
• 3
  Specific findings on cervical MRI with neck flexion, including focal cervical cord atrophy and enlarged epidural venous enhancement, are crucial for diagnosing Hirayama disease.