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Syntelencephaly (Middle Interhemispheric Variant)
Luke L. Linscott, MD; Charles Raybaud, MD, FRCPC
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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

                                            1. Dubourg C et al: Recent advances in understanding inheritance of holoprosencephaly. Am J Med Genet C Semin Med Genet. 178(2):258-69, 2018
                                            2. Fallet-Bianco C: Neuropathology of holoprosencephaly. Am J Med Genet C Semin Med Genet. 178(2):214-28, 2018
                                            3. Kousa YA et al: Prenatal diagnosis of holoprosencephaly. Am J Med Genet C Semin Med Genet. 178(2):206-13, 2018
                                            4. Yang E et al: A practical approach to supratentorial brain malformations: what radiologists should know. Radiol Clin North Am. 55(4):609-27, 2017
                                            5. Akyol Y et al: Syntelencephaly associated with cystic cochleovestibular malformations. Neurology. 86(2):198-9, 2016
                                            6. Bulakbasi N et al: The middle interhemispheric variant of holoprosencephaly: magnetic resonance and diffusion tensor imaging findings. Br J Radiol. 89(1063):20160115, 2016
                                            7. 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
                                            8. Winter TC et al: Holoprosencephaly: a survey of the entity, with embryology and fetal imaging. Radiographics. 35(1):275-90, 2015
                                            9. 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
                                            10. Arora A et al: Teaching neuroimages: syntelencephaly: middle interhemispheric fusion. Neurology. 79(10):e86, 2012
                                            11. Solomon BD et al: Genotypic and phenotypic analysis of 396 individuals with mutations in Sonic Hedgehog. J Med Genet. 49(7):473-9, 2012
                                            12. 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
                                            13. 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
                                            14. Cheng X et al: Central roles of the roof plate in telencephalic development and holoprosencephaly. J Neurosci. 26(29):7640-9, 2006
                                            15. 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
                                            16. Hahn JS et al: Endocrine disorders associated with holoprosencephaly. J Pediatr Endocrinol Metab. 18(10):935-41, 2005
                                            17. Biancheri R et al: Middle interhemispheric variant of holoprosencephaly: a very mild clinical case. Neurology. 63(11):2194-6, 2004
                                            18. Lewis AJ et al: Middle interhemispheric variant of holoprosencephaly: a distinct cliniconeuroradiologic subtype. Neurology. 59(12): 1860-5, 2002
                                            19. Simon EM et al: The middle interhemispheric variant of holoprosencephaly. AJNR Am J Neuroradiol. 23(1): 151-6, 2002
                                            20. 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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                                            Related Differential Diagnoses
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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

                                                                                      1. Dubourg C et al: Recent advances in understanding inheritance of holoprosencephaly. Am J Med Genet C Semin Med Genet. 178(2):258-69, 2018
                                                                                      2. Fallet-Bianco C: Neuropathology of holoprosencephaly. Am J Med Genet C Semin Med Genet. 178(2):214-28, 2018
                                                                                      3. Kousa YA et al: Prenatal diagnosis of holoprosencephaly. Am J Med Genet C Semin Med Genet. 178(2):206-13, 2018
                                                                                      4. Yang E et al: A practical approach to supratentorial brain malformations: what radiologists should know. Radiol Clin North Am. 55(4):609-27, 2017
                                                                                      5. Akyol Y et al: Syntelencephaly associated with cystic cochleovestibular malformations. Neurology. 86(2):198-9, 2016
                                                                                      6. Bulakbasi N et al: The middle interhemispheric variant of holoprosencephaly: magnetic resonance and diffusion tensor imaging findings. Br J Radiol. 89(1063):20160115, 2016
                                                                                      7. 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
                                                                                      8. Winter TC et al: Holoprosencephaly: a survey of the entity, with embryology and fetal imaging. Radiographics. 35(1):275-90, 2015
                                                                                      9. 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
                                                                                      10. Arora A et al: Teaching neuroimages: syntelencephaly: middle interhemispheric fusion. Neurology. 79(10):e86, 2012
                                                                                      11. Solomon BD et al: Genotypic and phenotypic analysis of 396 individuals with mutations in Sonic Hedgehog. J Med Genet. 49(7):473-9, 2012
                                                                                      12. 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
                                                                                      13. 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
                                                                                      14. Cheng X et al: Central roles of the roof plate in telencephalic development and holoprosencephaly. J Neurosci. 26(29):7640-9, 2006
                                                                                      15. 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
                                                                                      16. Hahn JS et al: Endocrine disorders associated with holoprosencephaly. J Pediatr Endocrinol Metab. 18(10):935-41, 2005
                                                                                      17. Biancheri R et al: Middle interhemispheric variant of holoprosencephaly: a very mild clinical case. Neurology. 63(11):2194-6, 2004
                                                                                      18. Lewis AJ et al: Middle interhemispheric variant of holoprosencephaly: a distinct cliniconeuroradiologic subtype. Neurology. 59(12): 1860-5, 2002
                                                                                      19. Simon EM et al: The middle interhemispheric variant of holoprosencephaly. AJNR Am J Neuroradiol. 23(1): 151-6, 2002
                                                                                      20. Barkovich AJ et al: Middle interhemispheric fusion: an unusual variant of holoprosencephaly. AJNR Am J Neuroradiol. 14(2):431-40, 1993