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Hirayama Disease
Jeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Hirayama flexion myelopathy, juvenile spinal muscular atrophy, monomelic amyotrophy, juvenile asymmetric segmental spinal muscular atrophy
              • Definitions

                • Cervical myelopathy related to anterior displacement of posterior cervical dura with flexion

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Image Interpretation Pearls

                                      Selected References

                                      1. Paredes I et al: A severe case of Hirayama disease successfully treated by anterior cervical fusion. J Neurosurg Spine. 20(2):191-5, 2014
                                      2. Lehman VT et al: Cervical spine MR imaging findings of patients with Hirayama disease in North America: a multisite study. AJNR Am J Neuroradiol. 34(2):451-6, 2013
                                      3. Patel TR et al: Lack of epidural pressure change with neck flexion in a patient with Hirayama disease: case report. Neurosurgery. 64(6):E1196-7; discussion E1197, 2009
                                      4. Zhou B et al: Clinical features of Hirayama disease in mainland China. Amyotroph Lateral Scler. Epub ahead of print, 2009
                                      5. Cerami C et al: A cervical myelopathy with a Hirayama disease-like phenotype. Neurol Sci. 29(6):451-4, 2008
                                      6. Hirayama K: [Juvenile muscular atrophy of unilateral upper extremity (Hirayama disease)--half-century progress and establishment since its discovery.] Brain Nerve. 60(1):17-29, 2008
                                      7. Huang YC et al: A clinical study of Hirayama disease in Taiwan. Muscle Nerve. 37(5):576-82, 2008
                                      8. Misra UK et al: A clinical, magnetic resonance imaging, and survival motor neuron gene deletion study of Hirayama disease. Arch Neurol. 62(1):120-3, 2005
                                      9. Chen CJ et al: Hirayama flexion myelopathy: neutral-position MR imaging findings--importance of loss of attachment. Radiology. 231(1):39-44, 2004
                                      10. Hirayama K: Juvenile muscular atrophy of distal upper extremity (Hirayama disease). Intern Med. 39(4):283-90, 2000
                                      11. Hirayama K: Juvenile muscular atrophy of distal upper extremity (Hirayama disease): focal cervical ischemic poliomyelopathy. Neuropathology. 20 Suppl:S91-4, 2000
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Top Differential Diagnoses

                                            • Pathology

                                              • Clinical Issues

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Synonyms

                                                    • Hirayama flexion myelopathy, juvenile spinal muscular atrophy, monomelic amyotrophy, juvenile asymmetric segmental spinal muscular atrophy
                                                  • Definitions

                                                    • Cervical myelopathy related to anterior displacement of posterior cervical dura with flexion

                                                  IMAGING

                                                  • General Features

                                                    • CT Findings

                                                      • MR Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Gross Pathologic & Surgical Features

                                                                CLINICAL ISSUES

                                                                • Presentation

                                                                  • Demographics

                                                                    • Natural History & Prognosis

                                                                      • Treatment

                                                                        DIAGNOSTIC CHECKLIST

                                                                        • Image Interpretation Pearls

                                                                          Selected References

                                                                          1. Paredes I et al: A severe case of Hirayama disease successfully treated by anterior cervical fusion. J Neurosurg Spine. 20(2):191-5, 2014
                                                                          2. Lehman VT et al: Cervical spine MR imaging findings of patients with Hirayama disease in North America: a multisite study. AJNR Am J Neuroradiol. 34(2):451-6, 2013
                                                                          3. Patel TR et al: Lack of epidural pressure change with neck flexion in a patient with Hirayama disease: case report. Neurosurgery. 64(6):E1196-7; discussion E1197, 2009
                                                                          4. Zhou B et al: Clinical features of Hirayama disease in mainland China. Amyotroph Lateral Scler. Epub ahead of print, 2009
                                                                          5. Cerami C et al: A cervical myelopathy with a Hirayama disease-like phenotype. Neurol Sci. 29(6):451-4, 2008
                                                                          6. Hirayama K: [Juvenile muscular atrophy of unilateral upper extremity (Hirayama disease)--half-century progress and establishment since its discovery.] Brain Nerve. 60(1):17-29, 2008
                                                                          7. Huang YC et al: A clinical study of Hirayama disease in Taiwan. Muscle Nerve. 37(5):576-82, 2008
                                                                          8. Misra UK et al: A clinical, magnetic resonance imaging, and survival motor neuron gene deletion study of Hirayama disease. Arch Neurol. 62(1):120-3, 2005
                                                                          9. Chen CJ et al: Hirayama flexion myelopathy: neutral-position MR imaging findings--importance of loss of attachment. Radiology. 231(1):39-44, 2004
                                                                          10. Hirayama K: Juvenile muscular atrophy of distal upper extremity (Hirayama disease). Intern Med. 39(4):283-90, 2000
                                                                          11. Hirayama K: Juvenile muscular atrophy of distal upper extremity (Hirayama disease): focal cervical ischemic poliomyelopathy. Neuropathology. 20 Suppl:S91-4, 2000