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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.
 
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

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
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

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
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
Winter TC, Kennedy AM, Woodward PJ
Radiographics 2015 Jan-Feb;35(1):275-90. doi: 10.1148/rg.351140040. PMID: 25590404
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
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

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
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
Winter TC, Kennedy AM, Woodward PJ
Radiographics 2015 Jan-Feb;35(1):275-90. doi: 10.1148/rg.351140040. PMID: 25590404
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
Hahn JS, Plawner LL
Pediatr Neurol 2004 Aug;31(2):79-88. doi: 10.1016/j.pediatrneurol.2004.03.006. PMID: 15301825

Therapy

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

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
Winter TC, Kennedy AM, Woodward PJ
Radiographics 2015 Jan-Feb;35(1):275-90. doi: 10.1148/rg.351140040. PMID: 25590404
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
Hahn JS, Plawner LL
Pediatr Neurol 2004 Aug;31(2):79-88. doi: 10.1016/j.pediatrneurol.2004.03.006. PMID: 15301825
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

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
Winter TC, Kennedy AM, Woodward PJ
Radiographics 2015 Jan-Feb;35(1):275-90. doi: 10.1148/rg.351140040. PMID: 25590404
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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