link
Bookmarks
Syntelencephaly (Middle Interhemispheric Variant)
Charles Raybaud, MD, FRCPC
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
2
3
0

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. Winter TC et al: Holoprosencephaly: a survey of the entity, with embryology and fetal imaging. Radiographics. 35(1):275-90, 2015
                                            2. 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
                                            3. Lami F et al: Holoprosencephaly: report of four cases and genotype-phenotype correlations. J Genet. 92(1):97-101, 2013
                                            4. Arora A et al: Teaching NeuroImages: Syntelencephaly: Middle interhemispheric fusion. Neurology. 79(10):e86, 2012
                                            5. Solomon BD et al: Genotypic and phenotypic analysis of 396 individuals with mutations in Sonic Hedgehog. J Med Genet. 49(7):473-9, 2012
                                            6. Srivastava K et al: Molecular analysis of the Noggin (NOG) gene in holoprosencephaly patients. Mol Genet Metab. 106(2):241-3, 2012
                                            7. 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
                                            8. Pineda-Alvarez DE et al: A broad range of ophthalmologic anomalies is part of the holoprosencephaly spectrum. Am J Med Genet A. 155A(11):2713-20, 2011
                                            9. Marcorelles P et al: Neuropathology of holoprosencephaly. Am J Med Genet C Semin Med Genet. 154C(1):109-19, 2010
                                            10. Solomon BD et al: Analysis of genotype-phenotype correlations in human holoprosencephaly. Am J Med Genet C Semin Med Genet. 154C(1):133-41, 2010
                                            11. 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
                                            12. Dubourg C et al: Holoprosencephaly. Orphanet J Rare Dis. 2:8, 2007
                                            13. Cheng X et al: Central roles of the roof plate in telencephalic development and holoprosencephaly. J Neurosci. 26(29):7640-9, 2006
                                            14. 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
                                            15. Hahn JS et al: Endocrine disorders associated with holoprosencephaly. J Pediatr Endocrinol Metab. 18(10):935-41, 2005
                                            16. Biancheri R et al: Middle interhemispheric variant of holoprosencephaly: a very mild clinical case. Neurology. 63(11):2194-6, 2004
                                            17. Lewis AJ et al: Middle interhemispheric variant of holoprosencephaly: a distinct cliniconeuroradiologic subtype. Neurology. 59(12): 1860-5, 2002
                                            18. Marcorelles P et al: Unusual variant of holoprosencephaly in monosomy 13q. Pediatr Dev Pathol. 5(2):170-8, 2002
                                            19. Simon EM et al: The middle interhemispheric variant of holoprosencephaly. AJNR Am J Neuroradiol. 23(1): 151-6, 2002
                                            20. Robin NH et al: Syntelencephaly in an infant of a diabetic mother. Am J Med Genet. 66(4):433-7, 1996
                                            21. Barkovich AJ et al: Middle interhemispheric fusion: an unusual variant of holoprosencephaly. AJNR Am J Neuroradiol. 14(2):431-40, 1993
                                            Related Anatomy
                                            Loading...
                                            Related Differential Diagnoses
                                            Loading...
                                            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. Winter TC et al: Holoprosencephaly: a survey of the entity, with embryology and fetal imaging. Radiographics. 35(1):275-90, 2015
                                                                                      2. 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
                                                                                      3. Lami F et al: Holoprosencephaly: report of four cases and genotype-phenotype correlations. J Genet. 92(1):97-101, 2013
                                                                                      4. Arora A et al: Teaching NeuroImages: Syntelencephaly: Middle interhemispheric fusion. Neurology. 79(10):e86, 2012
                                                                                      5. Solomon BD et al: Genotypic and phenotypic analysis of 396 individuals with mutations in Sonic Hedgehog. J Med Genet. 49(7):473-9, 2012
                                                                                      6. Srivastava K et al: Molecular analysis of the Noggin (NOG) gene in holoprosencephaly patients. Mol Genet Metab. 106(2):241-3, 2012
                                                                                      7. 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
                                                                                      8. Pineda-Alvarez DE et al: A broad range of ophthalmologic anomalies is part of the holoprosencephaly spectrum. Am J Med Genet A. 155A(11):2713-20, 2011
                                                                                      9. Marcorelles P et al: Neuropathology of holoprosencephaly. Am J Med Genet C Semin Med Genet. 154C(1):109-19, 2010
                                                                                      10. Solomon BD et al: Analysis of genotype-phenotype correlations in human holoprosencephaly. Am J Med Genet C Semin Med Genet. 154C(1):133-41, 2010
                                                                                      11. 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
                                                                                      12. Dubourg C et al: Holoprosencephaly. Orphanet J Rare Dis. 2:8, 2007
                                                                                      13. Cheng X et al: Central roles of the roof plate in telencephalic development and holoprosencephaly. J Neurosci. 26(29):7640-9, 2006
                                                                                      14. 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
                                                                                      15. Hahn JS et al: Endocrine disorders associated with holoprosencephaly. J Pediatr Endocrinol Metab. 18(10):935-41, 2005
                                                                                      16. Biancheri R et al: Middle interhemispheric variant of holoprosencephaly: a very mild clinical case. Neurology. 63(11):2194-6, 2004
                                                                                      17. Lewis AJ et al: Middle interhemispheric variant of holoprosencephaly: a distinct cliniconeuroradiologic subtype. Neurology. 59(12): 1860-5, 2002
                                                                                      18. Marcorelles P et al: Unusual variant of holoprosencephaly in monosomy 13q. Pediatr Dev Pathol. 5(2):170-8, 2002
                                                                                      19. Simon EM et al: The middle interhemispheric variant of holoprosencephaly. AJNR Am J Neuroradiol. 23(1): 151-6, 2002
                                                                                      20. Robin NH et al: Syntelencephaly in an infant of a diabetic mother. Am J Med Genet. 66(4):433-7, 1996
                                                                                      21. Barkovich AJ et al: Middle interhemispheric fusion: an unusual variant of holoprosencephaly. AJNR Am J Neuroradiol. 14(2):431-40, 1993