link
Bookmarks
Huntington Disease
James M. Provenzale, MD
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
8
0
0

KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Huntington disease (HD), huntingtin (HTT)
            • Synonyms

              • Huntington chorea
            • Definitions

              • Autosomal dominant neurodegenerative disease
                • Loss of GABAergic neurons of basal ganglia (BG)
              • Clinical triad: Early onset dementia, choreoathetosis, psychosis

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Nuclear Medicine Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Goveas J et al: Diffusion-MRI in neurodegenerative disorders. Magn Reson Imaging. ePub, 2015
                                              2. Mason S et al: Progress in Huntington's disease: the search for markers of disease onset and progression. J Neurol. ePub, 2015
                                              3. Stroedicke M et al: Systematic interaction network filtering identifies CRMP1 as a novel suppressor of huntingtin misfolding and neurotoxicity. Genome Res. 25(5):701-13, 2015
                                              4. Valor LM: Epigenetic-based therapies in the preclinical and clinical treatment of Huntington's disease. Int J Biochem Cell Biol. ePub, 2015
                                              5. Wassef SN et al: T1ρ imaging in premanifest Huntington disease reveals changes associated with disease progression. Mov Disord. ePub, 2015
                                              6. Ahmad R et al: PET imaging shows loss of striatal PDE10A in patients with Huntington disease. Neurology. 82(3):279-81, 2014
                                              7. Cole JH et al: Test-retest reliability of diffusion tensor imaging in Huntington's disease. PLoS Curr. 6, 2014
                                              8. Georgiou-Karistianis N et al: Functional magnetic resonance imaging of working memory in Huntington's disease: cross-sectional data from the IMAGE-HD study. Hum Brain Mapp. 35(5):1847-64, 2014
                                              9. Hensman Moss DJ et al: C9orf72 expansions are the most common genetic cause of Huntington disease phenocopies. Neurology. 82(4):292-9, 2014
                                              10. Macerollo A et al: Susceptibility-weighted imaging changes suggesting brain iron accumulation in Huntington's disease: an epiphenomenon which causes diagnostic difficulty. Eur J Neurol. 21(2):e16-7, 2014
                                              11. Matsui JT et al: Diffusion weighted imaging of prefrontal cortex in prodromal huntington's disease. Hum Brain Mapp. 35(4):1562-73, 2014
                                              12. Novak MJ et al: White matter integrity in premanifest and early Huntington's disease is related to caudate loss and disease progression. Cortex. 52:98-112, 2014
                                              13. Phillips O et al: The corticospinal tract in Huntington's disease. Cereb Cortex. Epub ahead of print, 2014
                                              14. Adam OR et al: Symptomatic treatment of Huntington disease. Neurotherapeutics. 5(2):181-97, 2008
                                              15. Imarisio S et al: Huntington's disease: from pathology and genetics to potential therapies. Biochem J. 412(2):191-209, 2008
                                              16. Alberch J et al: Neurotrophic factors in Huntington's disease. Prog Brain Res. 146:195-229, 2004
                                              17. Schapiro M et al: MR imaging and spectroscopy in juvenile Huntington disease. Pediatr Radiol. 34(8):640-3, 2004
                                              18. Becker G et al: Neuroimaging in basal ganglia disorders: perspectives for transcranial ultrasound. Mov Disord. 16(1):23-32, 2001
                                              Related Anatomy
                                              Loading...
                                              Related Differential Diagnoses
                                              Loading...
                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Pathology

                                                      • Diagnostic Checklist

                                                        TERMINOLOGY

                                                        • Abbreviations

                                                          • Huntington disease (HD), huntingtin (HTT)
                                                        • Synonyms

                                                          • Huntington chorea
                                                        • Definitions

                                                          • Autosomal dominant neurodegenerative disease
                                                            • Loss of GABAergic neurons of basal ganglia (BG)
                                                          • Clinical triad: Early onset dementia, choreoathetosis, psychosis

                                                        IMAGING

                                                        • General Features

                                                          • CT Findings

                                                            • MR Findings

                                                              • Ultrasonographic Findings

                                                                • Nuclear Medicine Findings

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Staging, Grading, & Classification

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Goveas J et al: Diffusion-MRI in neurodegenerative disorders. Magn Reson Imaging. ePub, 2015
                                                                                          2. Mason S et al: Progress in Huntington's disease: the search for markers of disease onset and progression. J Neurol. ePub, 2015
                                                                                          3. Stroedicke M et al: Systematic interaction network filtering identifies CRMP1 as a novel suppressor of huntingtin misfolding and neurotoxicity. Genome Res. 25(5):701-13, 2015
                                                                                          4. Valor LM: Epigenetic-based therapies in the preclinical and clinical treatment of Huntington's disease. Int J Biochem Cell Biol. ePub, 2015
                                                                                          5. Wassef SN et al: T1ρ imaging in premanifest Huntington disease reveals changes associated with disease progression. Mov Disord. ePub, 2015
                                                                                          6. Ahmad R et al: PET imaging shows loss of striatal PDE10A in patients with Huntington disease. Neurology. 82(3):279-81, 2014
                                                                                          7. Cole JH et al: Test-retest reliability of diffusion tensor imaging in Huntington's disease. PLoS Curr. 6, 2014
                                                                                          8. Georgiou-Karistianis N et al: Functional magnetic resonance imaging of working memory in Huntington's disease: cross-sectional data from the IMAGE-HD study. Hum Brain Mapp. 35(5):1847-64, 2014
                                                                                          9. Hensman Moss DJ et al: C9orf72 expansions are the most common genetic cause of Huntington disease phenocopies. Neurology. 82(4):292-9, 2014
                                                                                          10. Macerollo A et al: Susceptibility-weighted imaging changes suggesting brain iron accumulation in Huntington's disease: an epiphenomenon which causes diagnostic difficulty. Eur J Neurol. 21(2):e16-7, 2014
                                                                                          11. Matsui JT et al: Diffusion weighted imaging of prefrontal cortex in prodromal huntington's disease. Hum Brain Mapp. 35(4):1562-73, 2014
                                                                                          12. Novak MJ et al: White matter integrity in premanifest and early Huntington's disease is related to caudate loss and disease progression. Cortex. 52:98-112, 2014
                                                                                          13. Phillips O et al: The corticospinal tract in Huntington's disease. Cereb Cortex. Epub ahead of print, 2014
                                                                                          14. Adam OR et al: Symptomatic treatment of Huntington disease. Neurotherapeutics. 5(2):181-97, 2008
                                                                                          15. Imarisio S et al: Huntington's disease: from pathology and genetics to potential therapies. Biochem J. 412(2):191-209, 2008
                                                                                          16. Alberch J et al: Neurotrophic factors in Huntington's disease. Prog Brain Res. 146:195-229, 2004
                                                                                          17. Schapiro M et al: MR imaging and spectroscopy in juvenile Huntington disease. Pediatr Radiol. 34(8):640-3, 2004
                                                                                          18. Becker G et al: Neuroimaging in basal ganglia disorders: perspectives for transcranial ultrasound. Mov Disord. 16(1):23-32, 2001