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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
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
- Autosomal dominant neurodegenerative disease
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
- Chen L et al: Altered brain iron content and deposition rate in Huntington's disease as indicated by quantitative susceptibility MRI. J Neurosci Res. 97(4):467-79., 2018
- Liu Y et al: Progress in the diagnosis and management of chorea-acanthocytosis. Chin Med Sci J. 33(1):53-9, 2018
- Sobue G et al: Pathogenesis of frontotemporal lobar degeneration: insights from loss of function theory and early involvement of the caudate nucleus. Front Neurosci. 12:473, 2018
- Goveas J et al: Diffusion-MRI in neurodegenerative disorders. Magn Reson Imaging. 33(7):853-76, 2015
- Mason S et al: Progress in Huntington's disease: the search for markers of disease onset and progression. J Neurol. 262(8):1990-5, 2015
- 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
- Valor LM: Epigenetic-based therapies in the preclinical and clinical treatment of Huntington's disease. Int J Biochem Cell Biol. Oct;67:45-8, 2015
- Wassef SN et al: T1ρ imaging in premanifest Huntington disease reveals changes associated with disease progression. Mov Disord. ePub, 2015
- Ahmad R et al: PET imaging shows loss of striatal PDE10A in patients with Huntington disease. Neurology. 82(3):279-81, 2014
- Cole JH et al: Test-retest reliability of diffusion tensor imaging in Huntington's disease. PLoS Curr. 6, 2014
- 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
- Hensman Moss DJ et al: C9orf72 expansions are the most common genetic cause of Huntington disease phenocopies. Neurology. 82(4):292-9, 2014
- 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
- Matsui JT et al: Diffusion weighted imaging of prefrontal cortex in prodromal huntington's disease. Hum Brain Mapp. 35(4):1562-73, 2014
- 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
- Phillips O et al: The corticospinal tract in Huntington's disease. Cereb Cortex. 25(9):2670-82, 2014
- Adam OR et al: Symptomatic treatment of Huntington disease. Neurotherapeutics. 5(2):181-97, 2008
- Imarisio S et al: Huntington's disease: from pathology and genetics to potential therapies. Biochem J. 412(2):191-209, 2008
- Alberch J et al: Neurotrophic factors in Huntington's disease. Prog Brain Res. 146:195-229, 2004
- Schapiro M et al: MR imaging and spectroscopy in juvenile Huntington disease. Pediatr Radiol. 34(8):640-3, 2004
- Becker G et al: Neuroimaging in basal ganglia disorders: perspectives for transcranial ultrasound. Mov Disord. 16(1):23-32, 2001
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
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
- Autosomal dominant neurodegenerative disease
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
- Chen L et al: Altered brain iron content and deposition rate in Huntington's disease as indicated by quantitative susceptibility MRI. J Neurosci Res. 97(4):467-79., 2018
- Liu Y et al: Progress in the diagnosis and management of chorea-acanthocytosis. Chin Med Sci J. 33(1):53-9, 2018
- Sobue G et al: Pathogenesis of frontotemporal lobar degeneration: insights from loss of function theory and early involvement of the caudate nucleus. Front Neurosci. 12:473, 2018
- Goveas J et al: Diffusion-MRI in neurodegenerative disorders. Magn Reson Imaging. 33(7):853-76, 2015
- Mason S et al: Progress in Huntington's disease: the search for markers of disease onset and progression. J Neurol. 262(8):1990-5, 2015
- 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
- Valor LM: Epigenetic-based therapies in the preclinical and clinical treatment of Huntington's disease. Int J Biochem Cell Biol. Oct;67:45-8, 2015
- Wassef SN et al: T1ρ imaging in premanifest Huntington disease reveals changes associated with disease progression. Mov Disord. ePub, 2015
- Ahmad R et al: PET imaging shows loss of striatal PDE10A in patients with Huntington disease. Neurology. 82(3):279-81, 2014
- Cole JH et al: Test-retest reliability of diffusion tensor imaging in Huntington's disease. PLoS Curr. 6, 2014
- 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
- Hensman Moss DJ et al: C9orf72 expansions are the most common genetic cause of Huntington disease phenocopies. Neurology. 82(4):292-9, 2014
- 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
- Matsui JT et al: Diffusion weighted imaging of prefrontal cortex in prodromal huntington's disease. Hum Brain Mapp. 35(4):1562-73, 2014
- 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
- Phillips O et al: The corticospinal tract in Huntington's disease. Cereb Cortex. 25(9):2670-82, 2014
- Adam OR et al: Symptomatic treatment of Huntington disease. Neurotherapeutics. 5(2):181-97, 2008
- Imarisio S et al: Huntington's disease: from pathology and genetics to potential therapies. Biochem J. 412(2):191-209, 2008
- Alberch J et al: Neurotrophic factors in Huntington's disease. Prog Brain Res. 146:195-229, 2004
- Schapiro M et al: MR imaging and spectroscopy in juvenile Huntington disease. Pediatr Radiol. 34(8):640-3, 2004
- Becker G et al: Neuroimaging in basal ganglia disorders: perspectives for transcranial ultrasound. Mov Disord. 16(1):23-32, 2001
