Hirayama-like disease in the thoracic spine

Abstract

Hirayama disease is a cervical flexion myelopathy that typically causes upper extremity weakness in young male patients. We present two male patients (age 15 and 29) with MRI findings of thoracic ligamentous laxity similar in appearance to Hirayama disease. However, patients presented with atypical symptoms, specifically back pain and paresthesia of the upper and/or lower extremities, likely correlating to the abnormal thoracic spinal levels involved. Flexion/extension MRI sequences demonstrated the forward displacement of the dorsal dura and compression the thoracic cord with prominence of the posterior epidural space and venous plexus. Follow-up MRAs were negative for a spinal vascular malformation. Patients were managed conservatively with no surgical intervention. Clinical history, thoracic MRI, and follow-up flexion and angiographic imaging sequences may help confirm a diagnosis of Hirayama-like thoracic ligamentous laxity.

Keywords

• Thoracic spine
• Dorsal ligamentous laxity
• Hirayama disease
• Spine MRI
• Spine MRA
• Spinal vascular malformation