link
Bookmarks
Syntelencephaly
Anne M. Kennedy, MD
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
1
3
2
2

KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Middle interhemispheric variant of holoprosencephaly (HPE)
          • Definitions

            • Variant of HPE characterized by midline fusion of posterior frontal and parietal lobes

          IMAGING

          • General Features

            • Ultrasonographic Findings

              • MR Findings

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Gross Pathologic & Surgical Features

                        • Microscopic Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                DIAGNOSTIC CHECKLIST

                                • 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. Arora A et al: Teaching neuroImages: syntelencephaly: middle interhemispheric fusion. Neurology. 79(10):e86, 2012
                                  4. Winter TC et al: The cavum septi pellucidi: why is it important? J Ultrasound Med. 29(3):427-44, 2010
                                  5. Abe Y et al: EYA4, deleted in a case with middle interhemispheric variant of holoprosencephaly, interacts with SIX3 both physically and functionally. Hum Mutat. 30(10):E946-55, 2009
                                  6. Atalar MH et al: Middle interhemispheric variant of holoprosencephaly associated with bilateral perisylvian polymicrogyria. Pediatr Int. 50(2):241-4, 2008
                                  7. 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
                                  8. Malinger G et al: Differential diagnosis in fetuses with absent septum pellucidum. Ultrasound Obstet Gynecol. 25(1):42-9, 2005
                                  9. Biancheri R et al: Middle interhemispheric variant of holoprosencephaly: a very mild clinical case. Neurology. 63(11):2194-6, 2004
                                  10. Pulitzer SB et al: Prenatal MR findings of the middle interhemispheric variant of holoprosencephaly. AJNR Am J Neuroradiol. 25(6):1034-6, 2004
                                  11. Takanashi J et al: Middle interhemispheric variant of holoprosencephaly associated with diffuse polymicrogyria. AJNR Am J Neuroradiol. 24(3):394-7, 2003
                                  12. Lewis AJ et al: Middle interhemispheric variant of holoprosencephaly: a distinct cliniconeuroradiologic subtype. Neurology. 59(12):1860-5, 2002
                                  13. Marcorelles P et al: Unusual variant of holoprosencephaly in monosomy 13q. Pediatr Dev Pathol. 5(2):170-8, 2002
                                  14. Simon EM et al: The middle interhemispheric variant of holoprosencephaly. AJNR Am J Neuroradiol. 23(1):151-6, 2002
                                  15. Coleman LT et al: Syntelencephaly presenting with spastic diplegia. Neuropediatrics. 31(4):206-10, 2000
                                  16. Fujimoto S et al: Syntelencephaly associated with connected transhemispheric cleft of focal cortical dysplasia. Pediatr Neurol. 20(5):387-9, 1999
                                  17. Robin NH et al: Syntelencephaly in an infant of a diabetic mother. Am J Med Genet. 66(4):433-7, 1996
                                  18. 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

                                        • Clinical Issues

                                          TERMINOLOGY

                                          • Synonyms

                                            • Middle interhemispheric variant of holoprosencephaly (HPE)
                                          • Definitions

                                            • Variant of HPE characterized by midline fusion of posterior frontal and parietal lobes

                                          IMAGING

                                          • General Features

                                            • Ultrasonographic Findings

                                              • MR Findings

                                                • Imaging Recommendations

                                                  DIFFERENTIAL DIAGNOSIS

                                                    PATHOLOGY

                                                    • General Features

                                                      • Gross Pathologic & Surgical Features

                                                        • Microscopic Features

                                                          CLINICAL ISSUES

                                                          • Presentation

                                                            • Demographics

                                                              • Natural History & Prognosis

                                                                DIAGNOSTIC CHECKLIST

                                                                • 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. Arora A et al: Teaching neuroImages: syntelencephaly: middle interhemispheric fusion. Neurology. 79(10):e86, 2012
                                                                  4. Winter TC et al: The cavum septi pellucidi: why is it important? J Ultrasound Med. 29(3):427-44, 2010
                                                                  5. Abe Y et al: EYA4, deleted in a case with middle interhemispheric variant of holoprosencephaly, interacts with SIX3 both physically and functionally. Hum Mutat. 30(10):E946-55, 2009
                                                                  6. Atalar MH et al: Middle interhemispheric variant of holoprosencephaly associated with bilateral perisylvian polymicrogyria. Pediatr Int. 50(2):241-4, 2008
                                                                  7. 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
                                                                  8. Malinger G et al: Differential diagnosis in fetuses with absent septum pellucidum. Ultrasound Obstet Gynecol. 25(1):42-9, 2005
                                                                  9. Biancheri R et al: Middle interhemispheric variant of holoprosencephaly: a very mild clinical case. Neurology. 63(11):2194-6, 2004
                                                                  10. Pulitzer SB et al: Prenatal MR findings of the middle interhemispheric variant of holoprosencephaly. AJNR Am J Neuroradiol. 25(6):1034-6, 2004
                                                                  11. Takanashi J et al: Middle interhemispheric variant of holoprosencephaly associated with diffuse polymicrogyria. AJNR Am J Neuroradiol. 24(3):394-7, 2003
                                                                  12. Lewis AJ et al: Middle interhemispheric variant of holoprosencephaly: a distinct cliniconeuroradiologic subtype. Neurology. 59(12):1860-5, 2002
                                                                  13. Marcorelles P et al: Unusual variant of holoprosencephaly in monosomy 13q. Pediatr Dev Pathol. 5(2):170-8, 2002
                                                                  14. Simon EM et al: The middle interhemispheric variant of holoprosencephaly. AJNR Am J Neuroradiol. 23(1):151-6, 2002
                                                                  15. Coleman LT et al: Syntelencephaly presenting with spastic diplegia. Neuropediatrics. 31(4):206-10, 2000
                                                                  16. Fujimoto S et al: Syntelencephaly associated with connected transhemispheric cleft of focal cortical dysplasia. Pediatr Neurol. 20(5):387-9, 1999
                                                                  17. Robin NH et al: Syntelencephaly in an infant of a diabetic mother. Am J Med Genet. 66(4):433-7, 1996
                                                                  18. Barkovich AJ et al: Middle interhemispheric fusion: an unusual variant of holoprosencephaly. AJNR Am J Neuroradiol. 14(2):431-40, 1993