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
Related Anatomy
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References
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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
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