Bookmarks
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Wilson disease (WD)
Synonyms
- Progressive hepatolenticular degeneration
Definitions
- Autosomal recessive copper metabolism disorder with abnormal accumulation of copper in various tissues
IMAGING
General Features
CT Findings
MR 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
Image Interpretation Pearls
Selected References
- Kalita J et al: Predictors of seizure in Wilson disease: a clinico-radiological and biomarkers study. Neurotoxicology. 71:87-92, 2018
- Dusek P et al: Brain iron accumulation in Wilson disease: a post mortem 7 Tesla MRI - histopathological study. Neuropathol Appl Neurobiol. 43(6):514-32, 2017
- Wu F et al: Wilson's disease: a comprehensive review of the molecular mechanisms. Int J Mol Sci. 16(3):6419-31, 2015
- Fritzsch D et al: Seven-tesla magnetic resonance imaging in Wilson disease using quantitative susceptibility mapping for measurement of copper accumulation. Invest Radiol. 49(5):299-306, 2014
- Dusek P et al: The neurotoxicity of iron, copper and manganese in Parkinson's and Wilson's diseases. J Trace Elem Med Biol. 31:193-203, 2014
- Gupta S: Cell therapy to remove excess copper in Wilson's disease. Ann N Y Acad Sci. 1315:70-80, 2014
- Hermann W: Morphological and functional imaging in neurological and non-neurological Wilson's patients. Ann N Y Acad Sci. 1315:24-9, 2014
- Kozić DB et al: Reversible lesions in the brain parenchyma in Wilson's disease confirmed by magnetic resonance imaging: earlier administration of chelating therapy can reduce the damage to the brain. Neural Regen Res. 9(21):1912-6, 2014
- Krishna SH et al: Congenital genetic inborn errors of metabolism presenting as an adult or persisting into adulthood: neuroimaging in the more common or recognizable disorders. Semin Ultrasound CT MR. 35(2):160-91, 2014
- Parekh JR et al: Wilson's disease: 'face of giant panda' and 'trident' signs together. Oxf Med Case Reports. 2014(1):16-7, 2014
- Patell R et al: Atypical neuroimaging in Wilson's disease. BMJ Case Rep. 2014, 2014
- Pulai S et al: Clinical features, MRI brain, and MRS abnormalities of drug-naïve neurologic Wilson's disease. Neurol India. 62(2):153-8, 2014
- Zhou XX et al: Characterizing brain mineral deposition in patients with Wilson disease using susceptibility-weighted imaging. Neurol India. 62(4):362-6, 2014
- Trocello JM et al: Extensive striatal, cortical, and white matter brain MRI abnormalities in Wilson disease. Neurology. 81(17):1557, 2013
- Lorincz MT: Recognition and treatment of neurologic Wilson's disease. Semin Neurol. 32(5):538-43, 2012
- Ala A et al: Wilson's disease. Lancet. 369(9559):397-408, 2007
- Kim TJ et al: MR imaging of the brain in Wilson disease of childhood: findings before and after treatment with clinical correlation. AJNR Am J Neuroradiol. 27(6):1373-8, 2006
- Sinha S et al: Wilson's disease: cranial MRI observations and clinical correlation. Neuroradiology. 48(9):613-21, 2006
- Jacobs DA et al: The "double panda sign" in Wilson's disease. Neurology. 61(7):969, 2003
Related Anatomy
Loading...
Related Differential Diagnoses
Loading...
References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Wilson disease (WD)
Synonyms
- Progressive hepatolenticular degeneration
Definitions
- Autosomal recessive copper metabolism disorder with abnormal accumulation of copper in various tissues
IMAGING
General Features
CT Findings
MR 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
Image Interpretation Pearls
Selected References
- Kalita J et al: Predictors of seizure in Wilson disease: a clinico-radiological and biomarkers study. Neurotoxicology. 71:87-92, 2018
- Dusek P et al: Brain iron accumulation in Wilson disease: a post mortem 7 Tesla MRI - histopathological study. Neuropathol Appl Neurobiol. 43(6):514-32, 2017
- Wu F et al: Wilson's disease: a comprehensive review of the molecular mechanisms. Int J Mol Sci. 16(3):6419-31, 2015
- Fritzsch D et al: Seven-tesla magnetic resonance imaging in Wilson disease using quantitative susceptibility mapping for measurement of copper accumulation. Invest Radiol. 49(5):299-306, 2014
- Dusek P et al: The neurotoxicity of iron, copper and manganese in Parkinson's and Wilson's diseases. J Trace Elem Med Biol. 31:193-203, 2014
- Gupta S: Cell therapy to remove excess copper in Wilson's disease. Ann N Y Acad Sci. 1315:70-80, 2014
- Hermann W: Morphological and functional imaging in neurological and non-neurological Wilson's patients. Ann N Y Acad Sci. 1315:24-9, 2014
- Kozić DB et al: Reversible lesions in the brain parenchyma in Wilson's disease confirmed by magnetic resonance imaging: earlier administration of chelating therapy can reduce the damage to the brain. Neural Regen Res. 9(21):1912-6, 2014
- Krishna SH et al: Congenital genetic inborn errors of metabolism presenting as an adult or persisting into adulthood: neuroimaging in the more common or recognizable disorders. Semin Ultrasound CT MR. 35(2):160-91, 2014
- Parekh JR et al: Wilson's disease: 'face of giant panda' and 'trident' signs together. Oxf Med Case Reports. 2014(1):16-7, 2014
- Patell R et al: Atypical neuroimaging in Wilson's disease. BMJ Case Rep. 2014, 2014
- Pulai S et al: Clinical features, MRI brain, and MRS abnormalities of drug-naïve neurologic Wilson's disease. Neurol India. 62(2):153-8, 2014
- Zhou XX et al: Characterizing brain mineral deposition in patients with Wilson disease using susceptibility-weighted imaging. Neurol India. 62(4):362-6, 2014
- Trocello JM et al: Extensive striatal, cortical, and white matter brain MRI abnormalities in Wilson disease. Neurology. 81(17):1557, 2013
- Lorincz MT: Recognition and treatment of neurologic Wilson's disease. Semin Neurol. 32(5):538-43, 2012
- Ala A et al: Wilson's disease. Lancet. 369(9559):397-408, 2007
- Kim TJ et al: MR imaging of the brain in Wilson disease of childhood: findings before and after treatment with clinical correlation. AJNR Am J Neuroradiol. 27(6):1373-8, 2006
- Sinha S et al: Wilson's disease: cranial MRI observations and clinical correlation. Neuroradiology. 48(9):613-21, 2006
- Jacobs DA et al: The "double panda sign" in Wilson's disease. Neurology. 61(7):969, 2003
