Hirayama-like disease in the thoracic spine

• Type of Material: Article
• Authors: Maria Braileanu, Massachusetts General Hospital; Michael Hoch, University of Pennsylvania; Sandra Rincon, Massachusetts General Hospital; Brent Weinberg, Emory University
• Language: English
• Date: 2021-05-01
• Publisher: Elsevier
• Publication Version: Final Published Version
• Copyright Statement: © 2021 The Authors. Published by Elsevier Inc. on behalf of University of Washington.
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1016/j.radcr.2021.02.039
• Title of Journal or Parent Work: Radiology Case Reports
• Volume: 16
• Issue: 5
• Start Page: 1216
• End Page: 1219
• Grant/Funding Information: None declared
• Keywords: Thoracic surgery; Hirayama disease; Spinal cord injury; Radiology, Nuclear Medicine & Medical Imaging
• Research Categories: Health Sciences, Epidemiology; Health Sciences, Medicine and Surgery; Health Sciences, Radiology

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.