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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Abbreviations
- Rhombencephalosynapsis (RS)
- Lhermitte-Duclos disease (LDD)
- Joubert syndrome & related disorders (JSRD)
Synonyms
- LDD: Cowden syndrome, dysplastic cerebellar gangliocytoma, diffuse hypertrophy of cerebellar cortex
- JSRD: Molar tooth malformation
Definitions
- RS: Incomplete separation of cerebellar hemispheres with absence or hypoplasia of vermis
- LDD: Focal enlargement (probably hamartomatous) of cerebellar cortex
- JSRD: Absence of decussation of superior cerebellar peduncles leading to molar tooth sign
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
Selected References
- Whitehead MT et al: Rhombencephalosynapsis as a cause of aqueductal stenosis: an under-recognized association in hydrocephalic children. Pediatr Radiol. 44(7):849-56, 2014
- Barkovich AJ et al: Pediatric neuroimaging. 5th e. Philadelphia, PA. Lippincott Williams & Wilkins. 473-83, 2012
- Poretti A et al: Joubert syndrome and related disorders: spectrum of neuroimaging findings in 75 patients. AJNR Am J Neuroradiol. 32(8):1459-63, 2011
- Shinagare AB et al: Case 144: Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease). Radiology. 251(1):298-303, 2009
- Kumar R et al: Lhermitte-Duclos disease. Childs Nerv Syst. 23(7):729-32, 2007
- van Beek EJ et al: Case 25: Joubert syndrome. Radiology. 216(2):379-82, 2000
- Quisling RG et al: Magnetic resonance imaging features and classification of central nervous system malformations in Joubert syndrome. J Child Neurol. 14(10):628-35; discussion 669-72, 1999
- Utsunomiya H et al: Rhombencephalosynapsis: cerebellar embryogenesis. AJNR Am J Neuroradiol. 19(3):547-9, 1998
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Abbreviations
- Rhombencephalosynapsis (RS)
- Lhermitte-Duclos disease (LDD)
- Joubert syndrome & related disorders (JSRD)
Synonyms
- LDD: Cowden syndrome, dysplastic cerebellar gangliocytoma, diffuse hypertrophy of cerebellar cortex
- JSRD: Molar tooth malformation
Definitions
- RS: Incomplete separation of cerebellar hemispheres with absence or hypoplasia of vermis
- LDD: Focal enlargement (probably hamartomatous) of cerebellar cortex
- JSRD: Absence of decussation of superior cerebellar peduncles leading to molar tooth sign
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
Selected References
- Whitehead MT et al: Rhombencephalosynapsis as a cause of aqueductal stenosis: an under-recognized association in hydrocephalic children. Pediatr Radiol. 44(7):849-56, 2014
- Barkovich AJ et al: Pediatric neuroimaging. 5th e. Philadelphia, PA. Lippincott Williams & Wilkins. 473-83, 2012
- Poretti A et al: Joubert syndrome and related disorders: spectrum of neuroimaging findings in 75 patients. AJNR Am J Neuroradiol. 32(8):1459-63, 2011
- Shinagare AB et al: Case 144: Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease). Radiology. 251(1):298-303, 2009
- Kumar R et al: Lhermitte-Duclos disease. Childs Nerv Syst. 23(7):729-32, 2007
- van Beek EJ et al: Case 25: Joubert syndrome. Radiology. 216(2):379-82, 2000
- Quisling RG et al: Magnetic resonance imaging features and classification of central nervous system malformations in Joubert syndrome. J Child Neurol. 14(10):628-35; discussion 669-72, 1999
- Utsunomiya H et al: Rhombencephalosynapsis: cerebellar embryogenesis. AJNR Am J Neuroradiol. 19(3):547-9, 1998
