{"id":"3f976b72-edca-417d-9bc0-f7260b272d07","slug":"hirayama-disease-early-mri-diagnosis-of-subacute-medullary-ischemia-a-case-report","title":"Hirayama disease – Early MRI diagnosis of subacute medullary ischemia: A case report","authors":["Karol Galletta","Michele Gaeta","Concetta Alafaci","Sergio Vinci","Marcello Longo","Giovanni Grasso","Francesca Granata"],"abstract":"Background: Hirayama disease (HD) is a rare, benign, and self-limiting motor neuron disorder that results in selective motor impairment of the C7-T1 myotomes. It is characterized by progressive, unilateral, or bilateral asymmetric muscle atrophy of the distal upper extremities and myelopathy. Case Description: A 23-year-old male presented with bilateral atrophy of the thenar/hypothenar eminences/ interosseous muscles, plus left-hand weakness. The cervical MRI documented subacute ischemic damage of the distal cervical cord. To rule out a tumor and reduce questionable cord compression, the patient underwent a C5–C6 anterior cervical discectomy and fusion (ACDF) immediately followed by a laminectomy with durotomy and to obtain a spinal cord biopsy. When the histology confirmed focal cord ischemia consistent with HD, it was clear that both operations were unnecessary. Conclusion: Establishing the diagnosis of HD is based on clinical findings and MRI/flexion MR features which include the demonstration of an increased T2-weighted intramedullary cord signal, enlargement of the posterior epidural space, and segmental spinal cord atrophy. The presence of HD should be recognized as a “nonsurgical entity,” and conservative nonsurgical management should be employed.","thumbnailUrl":"https://sni-digital-videos.s3.amazonaws.com/articles/3f976b72-edca-417d-9bc0-f7260b272d07/featured/hero-1781563456754.png","publishDate":"2020-05-16T00:00:00.000Z","doi":"10.25259/SNI_151_2020","categories":["Spine","Case Report"],"fullTextUrl":"https://surgicalneurologyint.com/wp-content/uploads/2020/05/10031/SNI-11-115.pdf"}