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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Synonyms
- Syntelencephaly
- Middle interhemispheric variant (MIH) of holoprosencephaly (HPE)
Definitions
- Variant of HPE characterized by lack of separation of midportion of hemispheres
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
Ultrasonographic Findings
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
Selected References
- Calloni SF et al: Disorders of ventral induction/spectrum of holoprosencephaly. Neuroimaging Clin N Am. 29(3):411-21, 2019
- Özdemir M et al: Middle interhemispheric variant of holoprosencephaly in an asymptomatic adult. BJR Case Rep. 5(4):20190035, 2019
- Dubourg C et al: Recent advances in understanding inheritance of holoprosencephaly. Am J Med Genet C Semin Med Genet. 178(2):258-69, 2018
- Kousa YA et al: Prenatal diagnosis of holoprosencephaly. Am J Med Genet C Semin Med Genet. 178(2):206-13, 2018
- Yang E et al: A practical approach to supratentorial brain malformations: what radiologists should know. Radiol Clin North Am. 55(4):609-27, 2017
- Akyol Y et al: Syntelencephaly associated with cystic cochleovestibular malformations. Neurology. 86(2):198-9, 2016
- Bulakbasi N et al: The middle interhemispheric variant of holoprosencephaly: magnetic resonance and diffusion tensor imaging findings. Br J Radiol. 89(1063):20160115, 2016
- Griffiths PD et al: In utero MR imaging of fetal holoprosencephaly: a structured approach to diagnosis and classification. AJNR Am J Neuroradiol. 37(3):536-43, 2016
- Winter TC et al: Holoprosencephaly: a survey of the entity, with embryology and fetal imaging. Radiographics. 35(1):275-90, 2015
- Vinurel N et al: Distortion of the anterior part of the interhemispheric fissure: significance and implications for prenatal diagnosis. Ultrasound Obstet Gynecol. 43(3):346-52, 2014
- Arora A et al: Teaching neuroimages: syntelencephaly: middle interhemispheric fusion. Neurology. 79(10):e86, 2012
- Solomon BD et al: Genotypic and phenotypic analysis of 396 individuals with mutations in Sonic Hedgehog. J Med Genet. 49(7):473-9, 2012
- Mercier S et al: New findings for phenotype-genotype correlations in a large European series of holoprosencephaly cases. J Med Genet. 48(11):752-60, 2011
- Dheen ST et al: Recent studies on neural tube defects in embryos of diabetic pregnancy: an overview. Curr Med Chem. 16(18):2345-54, 2009
- Cheng X et al: Central roles of the roof plate in telencephalic development and holoprosencephaly. J Neurosci. 26(29):7640-9, 2006
- Picone O et al: Prenatal diagnosis of a possible new middle interhemispheric variant of holoprosencephaly using sonographic and magnetic resonance imaging. Ultrasound Obstet Gynecol. 28(2):229-31, 2006
- Hahn JS et al: Endocrine disorders associated with holoprosencephaly. J Pediatr Endocrinol Metab. 18(10):935-41, 2005
- Biancheri R et al: Middle interhemispheric variant of holoprosencephaly: a very mild clinical case. Neurology. 63(11):2194-6, 2004
- Lewis AJ et al: Middle interhemispheric variant of holoprosencephaly: a distinct cliniconeuroradiologic subtype. Neurology. 59(12): 1860-5, 2002
- Simon EM et al: The middle interhemispheric variant of holoprosencephaly. AJNR Am J Neuroradiol. 23(1):151-6, 2002
- Barkovich AJ et al: Middle interhemispheric fusion: an unusual variant of holoprosencephaly. AJNR Am J Neuroradiol. 14(2):431-40, 1993
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Synonyms
- Syntelencephaly
- Middle interhemispheric variant (MIH) of holoprosencephaly (HPE)
Definitions
- Variant of HPE characterized by lack of separation of midportion of hemispheres
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
Ultrasonographic Findings
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
Selected References
- Calloni SF et al: Disorders of ventral induction/spectrum of holoprosencephaly. Neuroimaging Clin N Am. 29(3):411-21, 2019
- Özdemir M et al: Middle interhemispheric variant of holoprosencephaly in an asymptomatic adult. BJR Case Rep. 5(4):20190035, 2019
- Dubourg C et al: Recent advances in understanding inheritance of holoprosencephaly. Am J Med Genet C Semin Med Genet. 178(2):258-69, 2018
- Kousa YA et al: Prenatal diagnosis of holoprosencephaly. Am J Med Genet C Semin Med Genet. 178(2):206-13, 2018
- Yang E et al: A practical approach to supratentorial brain malformations: what radiologists should know. Radiol Clin North Am. 55(4):609-27, 2017
- Akyol Y et al: Syntelencephaly associated with cystic cochleovestibular malformations. Neurology. 86(2):198-9, 2016
- Bulakbasi N et al: The middle interhemispheric variant of holoprosencephaly: magnetic resonance and diffusion tensor imaging findings. Br J Radiol. 89(1063):20160115, 2016
- Griffiths PD et al: In utero MR imaging of fetal holoprosencephaly: a structured approach to diagnosis and classification. AJNR Am J Neuroradiol. 37(3):536-43, 2016
- Winter TC et al: Holoprosencephaly: a survey of the entity, with embryology and fetal imaging. Radiographics. 35(1):275-90, 2015
- Vinurel N et al: Distortion of the anterior part of the interhemispheric fissure: significance and implications for prenatal diagnosis. Ultrasound Obstet Gynecol. 43(3):346-52, 2014
- Arora A et al: Teaching neuroimages: syntelencephaly: middle interhemispheric fusion. Neurology. 79(10):e86, 2012
- Solomon BD et al: Genotypic and phenotypic analysis of 396 individuals with mutations in Sonic Hedgehog. J Med Genet. 49(7):473-9, 2012
- Mercier S et al: New findings for phenotype-genotype correlations in a large European series of holoprosencephaly cases. J Med Genet. 48(11):752-60, 2011
- Dheen ST et al: Recent studies on neural tube defects in embryos of diabetic pregnancy: an overview. Curr Med Chem. 16(18):2345-54, 2009
- Cheng X et al: Central roles of the roof plate in telencephalic development and holoprosencephaly. J Neurosci. 26(29):7640-9, 2006
- Picone O et al: Prenatal diagnosis of a possible new middle interhemispheric variant of holoprosencephaly using sonographic and magnetic resonance imaging. Ultrasound Obstet Gynecol. 28(2):229-31, 2006
- Hahn JS et al: Endocrine disorders associated with holoprosencephaly. J Pediatr Endocrinol Metab. 18(10):935-41, 2005
- Biancheri R et al: Middle interhemispheric variant of holoprosencephaly: a very mild clinical case. Neurology. 63(11):2194-6, 2004
- Lewis AJ et al: Middle interhemispheric variant of holoprosencephaly: a distinct cliniconeuroradiologic subtype. Neurology. 59(12): 1860-5, 2002
- Simon EM et al: The middle interhemispheric variant of holoprosencephaly. AJNR Am J Neuroradiol. 23(1):151-6, 2002
- Barkovich AJ et al: Middle interhemispheric fusion: an unusual variant of holoprosencephaly. AJNR Am J Neuroradiol. 14(2):431-40, 1993
