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Midline interhemispheric variant of holoprosencephaly

MedGen UID:
798407
•Concept ID:
CN206692
•
Disease or Syndrome
Synonyms: Middle interhemispheric fusion variant; Middle interhemispheric variant of holoprosencephaly; MIH; MIH type HPE; MIHF; MIHV; Syntelencephaly
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Non-Mendelian inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
Orphanet: ORPHA93926

Definition

Midline interhemispheric variant of holoprosencephaly (MIH) or syntelencephaly is a form of holoprosencephaly (HPE; see this term) characterized by non-separation of the posterior frontal and parietal lobes, normally-formed callosal genu and splenium, absence of the callosal body, normally-separated hypothalamus and lentiform nucleus, and frequent heterotopic gray matter. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMidline interhemispheric variant of holoprosencephaly
Follow this link to review classifications for Midline interhemispheric variant of holoprosencephaly in Orphanet.

Professional guidelines

PubMed

Prenatal diagnosis of middle interhemispheric variant of holoprosencephaly: review of literature and prenatal case series.
Tavano I, De Keersmaecker B, Aertsen M, De Catte L
J Matern Fetal Neonatal Med 2022 Dec;35(25):4976-4984. Epub 2021 Jan 17 doi: 10.1080/14767058.2021.1873942. PMID: 33455493
Evaluation and management of children with holoprosencephaly.
Hahn JS, Plawner LL
Pediatr Neurol 2004 Aug;31(2):79-88. doi: 10.1016/j.pediatrneurol.2004.03.006. PMID: 15301825

Recent clinical studies

Etiology

The wide spectrum of ultrasound diagnosis of holoprosencephaly.
Ionescu CA, Vladareanu S, Tudorache S, Ples L, Herghelegiu C, Neacsu A, Navolan D, Dragan I, Oprescu DN
Med Ultrason 2019 May 2;21(2):163-169. doi: 10.11152/mu-1614. PMID: 31063520
The middle interhemispheric variant of holoprosencephaly: magnetic resonance and diffusion tensor imaging findings.
Bulakbasi N, Cancuri O, Kocaoğlu M
Br J Radiol 2016 Jul;89(1063):20160115. Epub 2016 Apr 19 doi: 10.1259/bjr.20160115. PMID: 27089898Free PMC Article
Holoprosencephaly: a survey of the entity, with embryology and fetal imaging.
Winter TC, Kennedy AM, Woodward PJ
Radiographics 2015 Jan-Feb;35(1):275-90. doi: 10.1148/rg.351140040. PMID: 25590404
Middle interhemispheric variant of holoprosencephaly: a distinct cliniconeuroradiologic subtype.
Lewis AJ, Simon EM, Barkovich AJ, Clegg NJ, Delgado MR, Levey E, Hahn JS
Neurology 2002 Dec 24;59(12):1860-5. doi: 10.1212/01.wnl.0000037483.31989.b9. PMID: 12499474
The middle interhemispheric variant of holoprosencephaly.
Simon EM, Hevner RF, Pinter JD, Clegg NJ, Delgado M, Kinsman SL, Hahn JS, Barkovich AJ
AJNR Am J Neuroradiol 2002 Jan;23(1):151-6. PMID: 11827888Free PMC Article

Diagnosis

Prenatal diagnosis of middle interhemispheric variant of holoprosencephaly: review of literature and prenatal case series.
Tavano I, De Keersmaecker B, Aertsen M, De Catte L
J Matern Fetal Neonatal Med 2022 Dec;35(25):4976-4984. Epub 2021 Jan 17 doi: 10.1080/14767058.2021.1873942. PMID: 33455493
The wide spectrum of ultrasound diagnosis of holoprosencephaly.
Ionescu CA, Vladareanu S, Tudorache S, Ples L, Herghelegiu C, Neacsu A, Navolan D, Dragan I, Oprescu DN
Med Ultrason 2019 May 2;21(2):163-169. doi: 10.11152/mu-1614. PMID: 31063520
Holoprosencephaly: a survey of the entity, with embryology and fetal imaging.
Winter TC, Kennedy AM, Woodward PJ
Radiographics 2015 Jan-Feb;35(1):275-90. doi: 10.1148/rg.351140040. PMID: 25590404
Holoprosencephaly.
Dubourg C, Bendavid C, Pasquier L, Henry C, Odent S, David V
Orphanet J Rare Dis 2007 Feb 2;2:8. doi: 10.1186/1750-1172-2-8. PMID: 17274816Free PMC Article
Evaluation and management of children with holoprosencephaly.
Hahn JS, Plawner LL
Pediatr Neurol 2004 Aug;31(2):79-88. doi: 10.1016/j.pediatrneurol.2004.03.006. PMID: 15301825

Therapy

Middle interhemispheric variant of holoprosencephaly: a distinct cliniconeuroradiologic subtype.
Lewis AJ, Simon EM, Barkovich AJ, Clegg NJ, Delgado MR, Levey E, Hahn JS
Neurology 2002 Dec 24;59(12):1860-5. doi: 10.1212/01.wnl.0000037483.31989.b9. PMID: 12499474

Prognosis

The middle interhemispheric variant of holoprosencephaly: magnetic resonance and diffusion tensor imaging findings.
Bulakbasi N, Cancuri O, Kocaoğlu M
Br J Radiol 2016 Jul;89(1063):20160115. Epub 2016 Apr 19 doi: 10.1259/bjr.20160115. PMID: 27089898Free PMC Article
Holoprosencephaly: a survey of the entity, with embryology and fetal imaging.
Winter TC, Kennedy AM, Woodward PJ
Radiographics 2015 Jan-Feb;35(1):275-90. doi: 10.1148/rg.351140040. PMID: 25590404
Holoprosencephaly.
Dubourg C, Bendavid C, Pasquier L, Henry C, Odent S, David V
Orphanet J Rare Dis 2007 Feb 2;2:8. doi: 10.1186/1750-1172-2-8. PMID: 17274816Free PMC Article
Evaluation and management of children with holoprosencephaly.
Hahn JS, Plawner LL
Pediatr Neurol 2004 Aug;31(2):79-88. doi: 10.1016/j.pediatrneurol.2004.03.006. PMID: 15301825
The middle interhemispheric variant of holoprosencephaly.
Simon EM, Hevner RF, Pinter JD, Clegg NJ, Delgado M, Kinsman SL, Hahn JS, Barkovich AJ
AJNR Am J Neuroradiol 2002 Jan;23(1):151-6. PMID: 11827888Free PMC Article

Clinical prediction guides

The wide spectrum of ultrasound diagnosis of holoprosencephaly.
Ionescu CA, Vladareanu S, Tudorache S, Ples L, Herghelegiu C, Neacsu A, Navolan D, Dragan I, Oprescu DN
Med Ultrason 2019 May 2;21(2):163-169. doi: 10.11152/mu-1614. PMID: 31063520
Holoprosencephaly: a survey of the entity, with embryology and fetal imaging.
Winter TC, Kennedy AM, Woodward PJ
Radiographics 2015 Jan-Feb;35(1):275-90. doi: 10.1148/rg.351140040. PMID: 25590404
Holoprosencephaly.
Dubourg C, Bendavid C, Pasquier L, Henry C, Odent S, David V
Orphanet J Rare Dis 2007 Feb 2;2:8. doi: 10.1186/1750-1172-2-8. PMID: 17274816Free PMC Article

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