Characterized by ↓ or disorganized growth, injury, or ↑ apoptosis
IMAGING
General Features
CT Findings
MR 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
Gao SS et al: [Two cases with lissencephaly associated cerebellar hypoplasia related to RELN variation.] Zhonghua Er Ke Za Zhi. 58(3):238-40, 2020
Saugier-Veber P et al: Pontocerebellar hypoplasia with rhombencephalosynapsis and microlissencephaly expands the spectrum of PCH type 1B. Eur J Med Genet. 63(4):103814, 2020
Shaikh M et al: Unilateral cerebellar hypoplasia: a rare cause of childhood seizures. J Pediatr Neurosci. 14(4):236-7, 2019
Bosemani T et al: Congenital abnormalities of the posterior fossa. Radiographics. 35(1):200-20, 2015
Poretti A et al: Differential diagnosis of cerebellar atrophy in childhood: an update. Neuropediatrics. 46(6):359-70, 2015
Adachi Y et al: Posterior fossa in primary microcephaly: relationships between forebrain and mid-hindbrain size in 110 patients. Neuropediatrics. 45(2):93-101, 2014
Bahi-Buisson N et al: The wide spectrum of tubulinopathies: what are the key features for the diagnosis? Brain. 137(Pt 6):1676-700, 2014
Poretti A et al: Cerebellar hypoplasia: differential diagnosis and diagnostic approach. Am J Med Genet C Semin Med Genet. 166(2):211-26, 2014
Doherty D et al: Midbrain and hindbrain malformations: advances in clinical diagnosis, imaging, and genetics. Lancet Neurol. 12(4):381-93, 2013
González G et al: Location of periventricular nodular heterotopia is related to the malformation phenotype on MRI. AJNR Am J Neuroradiol. 34(4):877-83, 2013
Massoud M et al: Prenatal unilateral cerebellar hypoplasia in a series of 26 cases: significance and implications for prenatal diagnosis. Ultrasound Obstet Gynecol. 44(4):447-54, 2013
Tam EW: Potential mechanisms of cerebellar hypoplasia in prematurity. Neuroradiology. 55 Suppl 2:41-6, 2013
Pisano T et al: Peritrigonal and temporo-occipital heterotopia with corpus callosum and cerebellar dysgenesis. Neurology. 79(12):1244-51, 2012
Ventura P et al: Mental retardation and epilepsy in patients with isolated cerebellar hypoplasia. J Child Neurol. 21(9):776-81, 2006
Adamsbaum C et al: MRI of the fetal posterior fossa. Pediatr Radiol. 35(2):124-40, 2005
Glass HC et al: Autosomal recessive cerebellar hypoplasia in the Hutterite population. Dev Med Child Neurol. 47(10):691-5, 2005
Messerschmidt A et al: Disruption of cerebellar development: potential complication of extreme prematurity. AJNR Am J Neuroradiol. 26(7):1659-67, 2005
Ozgen HM et al: Cerebellar hypoplasia-endosteal sclerosis: a long term follow-up. Am J Med Genet A. 134(2):215-9, 2005
Yapici Z et al: Non-progressive congenital ataxia with cerebellar hypoplasia in three families. Acta Paediatr. 94(2):248-53, 2005
Boltshauser E. Related Articles et al: Cerebellum-small brain but large confusion: a review of selected cerebellar malformations and disruptions. Am J Med Genet A. 126(4):376-85, 2004
Soto Ares G et al: [Cerebellar cortical dysplasia: MRI aspects and significance] J Radiol. 85(6 Pt 1):729-40, 2004
Soto-Ares G: Analysis and classification of cerebellar malformations. AJNR Am J Neuroradiol. 24(1):153; author reply 153, 2003
Wassmer E et al: Clinical spectrum associated with cerebellar hypoplasia. Pediatr Neurol. 28(5):347-51, 2003
Boltshauser E et al: Vanishing cerebellum in myelomeningocoele. Eur J Paediatr Neurol. 6(2):109-13, 2002
Patel S et al: Analysis and classification of cerebellar malformations. AJNR Am J Neuroradiol. 23(7):1074-87, 2002
Ramaekers VT et al: Genetic disorders and cerebellar structural abnormalities in childhood. Brain. 120 ( Pt 10):1739-51, 1997
al Shahwan SA et al: Non-progressive familial congenital cerebellar hypoplasia. J Neurol Sci. 128(1):71-7, 1995
Boycott KM et al: VLDLR Cerebellar Hypoplasia 1993
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
Synonyms
Microcerebellum, cerebellar hypogenesis
Definitions
Spectrum of malformative or disruptive conditions
There can be overlap
Characterized by ↓ or disorganized growth, injury, or ↑ apoptosis
IMAGING
General Features
CT Findings
MR 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
Gao SS et al: [Two cases with lissencephaly associated cerebellar hypoplasia related to RELN variation.] Zhonghua Er Ke Za Zhi. 58(3):238-40, 2020
Saugier-Veber P et al: Pontocerebellar hypoplasia with rhombencephalosynapsis and microlissencephaly expands the spectrum of PCH type 1B. Eur J Med Genet. 63(4):103814, 2020
Shaikh M et al: Unilateral cerebellar hypoplasia: a rare cause of childhood seizures. J Pediatr Neurosci. 14(4):236-7, 2019
Bosemani T et al: Congenital abnormalities of the posterior fossa. Radiographics. 35(1):200-20, 2015
Poretti A et al: Differential diagnosis of cerebellar atrophy in childhood: an update. Neuropediatrics. 46(6):359-70, 2015
Adachi Y et al: Posterior fossa in primary microcephaly: relationships between forebrain and mid-hindbrain size in 110 patients. Neuropediatrics. 45(2):93-101, 2014
Bahi-Buisson N et al: The wide spectrum of tubulinopathies: what are the key features for the diagnosis? Brain. 137(Pt 6):1676-700, 2014
Poretti A et al: Cerebellar hypoplasia: differential diagnosis and diagnostic approach. Am J Med Genet C Semin Med Genet. 166(2):211-26, 2014
Doherty D et al: Midbrain and hindbrain malformations: advances in clinical diagnosis, imaging, and genetics. Lancet Neurol. 12(4):381-93, 2013
González G et al: Location of periventricular nodular heterotopia is related to the malformation phenotype on MRI. AJNR Am J Neuroradiol. 34(4):877-83, 2013
Massoud M et al: Prenatal unilateral cerebellar hypoplasia in a series of 26 cases: significance and implications for prenatal diagnosis. Ultrasound Obstet Gynecol. 44(4):447-54, 2013
Tam EW: Potential mechanisms of cerebellar hypoplasia in prematurity. Neuroradiology. 55 Suppl 2:41-6, 2013
Pisano T et al: Peritrigonal and temporo-occipital heterotopia with corpus callosum and cerebellar dysgenesis. Neurology. 79(12):1244-51, 2012
Ventura P et al: Mental retardation and epilepsy in patients with isolated cerebellar hypoplasia. J Child Neurol. 21(9):776-81, 2006
Adamsbaum C et al: MRI of the fetal posterior fossa. Pediatr Radiol. 35(2):124-40, 2005
Glass HC et al: Autosomal recessive cerebellar hypoplasia in the Hutterite population. Dev Med Child Neurol. 47(10):691-5, 2005
Messerschmidt A et al: Disruption of cerebellar development: potential complication of extreme prematurity. AJNR Am J Neuroradiol. 26(7):1659-67, 2005
Ozgen HM et al: Cerebellar hypoplasia-endosteal sclerosis: a long term follow-up. Am J Med Genet A. 134(2):215-9, 2005
Yapici Z et al: Non-progressive congenital ataxia with cerebellar hypoplasia in three families. Acta Paediatr. 94(2):248-53, 2005
Boltshauser E. Related Articles et al: Cerebellum-small brain but large confusion: a review of selected cerebellar malformations and disruptions. Am J Med Genet A. 126(4):376-85, 2004
Soto Ares G et al: [Cerebellar cortical dysplasia: MRI aspects and significance] J Radiol. 85(6 Pt 1):729-40, 2004
Soto-Ares G: Analysis and classification of cerebellar malformations. AJNR Am J Neuroradiol. 24(1):153; author reply 153, 2003
Wassmer E et al: Clinical spectrum associated with cerebellar hypoplasia. Pediatr Neurol. 28(5):347-51, 2003
Boltshauser E et al: Vanishing cerebellum in myelomeningocoele. Eur J Paediatr Neurol. 6(2):109-13, 2002
Patel S et al: Analysis and classification of cerebellar malformations. AJNR Am J Neuroradiol. 23(7):1074-87, 2002
Ramaekers VT et al: Genetic disorders and cerebellar structural abnormalities in childhood. Brain. 120 ( Pt 10):1739-51, 1997
al Shahwan SA et al: Non-progressive familial congenital cerebellar hypoplasia. J Neurol Sci. 128(1):71-7, 1995
Boycott KM et al: VLDLR Cerebellar Hypoplasia 1993
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