A Case Report on Syntelencephaly - Middle Interhemispheric Holoprosencephaly Variant
A Case Report on Syntelencephaly - Middle Interhemispheric Holoprosencephaly Variant
Keywords:
Syntelencephaly, Middle Interhemispheric Holoprosencephaly VariantAbstract
Holoprosencephaly is a rare congenital brain malformation. It has been traditionally classified in to three types: alobar, semi lobar and lobar forms. Syntelencephaly is a lesser known variant of holoprosencephaly, Middle Interhemispheric Fusion (MIH) variant. We present a case of Syntelencephaly along with a review of imaging findings and the structural abnormalities of the brain in Syntelencephaly, compare these features with those of classic holoprosencephaly (HPE) and consider its embryogenetics. [Ankita P NJIRM 2017; 8(6):102-105]
References
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9. Brown LY, Odent S, David V, et al. Holoprosencephaly due to mutations in ZIC2: alanine tract expansion mutations may be caused by parental somatic recombination. Hum Mol Genet . 2001.
2. Barkovich AJ, Quint DJ. Middle interhemispheric fusion: an unusual variant of holoprosencephaly. AJNR Am J Neuroradiol . 1993; 14: 431-440
3. Simon EM, Hevner RF, Pinter JD, et al. The middle interhemispheric variant of holoprosencephaly. AJNR Am J Neuroradiol. 2002; 23 :151 -155
4. Takanashi J, Barkovich AJ, Clegg NJ, Delgado MR. Middle interhemispheric variant of holoprosencephaly associated with diffuse polymicrogyria. AJNR Am J Neuroradiol. 2003; 24:394-397.
5. Plawner LL, Delgado MR, Miller VS, et al. Neuroanatomy of holoprosencephaly as predictor of function: beyond the face predicting the brain. Neurology. 2002; 59: 1058-1066.
6. Lewis AJ et al. middle interhemispheric variant of holoprosencephaly: a distinct cliniconeuroradiologic subtype. Neurology.2002; 59:1860-65.
7. Merrow AC et al: Postnatal sonographic detection of a subtle case. Pediatric Radiology. 40 Suppl 1:S160,2010
8. Simon EM et al: The middle interhemispheric variant of holoprosencephaly. AJNR Am J Neuroradiology. 23(1):151-6,2002.
9. Brown LY, Odent S, David V, et al. Holoprosencephaly due to mutations in ZIC2: alanine tract expansion mutations may be caused by parental somatic recombination. Hum Mol Genet . 2001.
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Published
2018-02-06
How to Cite
Padia, A., Vaghela, D., Salvi, D., & Patel, Y. T. (2018). A Case Report on Syntelencephaly - Middle Interhemispheric Holoprosencephaly Variant: A Case Report on Syntelencephaly - Middle Interhemispheric Holoprosencephaly Variant. National Journal of Integrated Research in Medicine, 8(6), 102–105. Retrieved from http://nicpd.ac.in/ojs-/index.php/njirm/article/view/1345
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Case Report
