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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Canavan disease (CD)
Synonyms
- Spongiform leukodystrophy, aspartoacylase deficiency
- Spongy degeneration of CNS, Canavan-van Bogaert-Bertrand disease, ASPA deficiency, ASP deficiency, aminoacylase 2 deficiency, ACY2 deficiency
Definitions
- Progressive fatal autosomal-recessive spongiform leukodystrophy
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic 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
- Jauhari P et al: Juvenile Canavan disease: a leukodystrophy without white matter changes. Neuropediatrics. 49(6):420-1, 2018
- Guo F et al: Ablating N-acetylaspartate prevents leukodystrophy in a Canavan disease model. Ann Neurol. 77(5):884-8, 2015
- Baslow MH et al: Canavan disease, a rare early-onset human spongiform leukodystrophy: insights into its genesis and possible clinical interventions. Biochimie. 95(4):946-56, 2013
- van der Knaap MS et al: Defining and categorizing leukoencephalopathies of unknown origin: MR imaging approach. Radiology. 213(1):121-33, 1999
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
- Canavan disease (CD)
Synonyms
- Spongiform leukodystrophy, aspartoacylase deficiency
- Spongy degeneration of CNS, Canavan-van Bogaert-Bertrand disease, ASPA deficiency, ASP deficiency, aminoacylase 2 deficiency, ACY2 deficiency
Definitions
- Progressive fatal autosomal-recessive spongiform leukodystrophy
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic 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
- Jauhari P et al: Juvenile Canavan disease: a leukodystrophy without white matter changes. Neuropediatrics. 49(6):420-1, 2018
- Guo F et al: Ablating N-acetylaspartate prevents leukodystrophy in a Canavan disease model. Ann Neurol. 77(5):884-8, 2015
- Baslow MH et al: Canavan disease, a rare early-onset human spongiform leukodystrophy: insights into its genesis and possible clinical interventions. Biochimie. 95(4):946-56, 2013
- van der Knaap MS et al: Defining and categorizing leukoencephalopathies of unknown origin: MR imaging approach. Radiology. 213(1):121-33, 1999
