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The old and the new: supratentorial MR findings in Chiari II malformation

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Abstract

Objective

The objective of this study was to use magnetic resonance imaging to develop an improved morphological understanding of the abnormalities of the forebrain in Chiari II malformation.

Materials and methods

Seventy-four patients with Chiari II malformations investigated between 1999 and 2007 were enrolled. Imaging was retrospectively reviewed by two pediatric neuroradiologists, with special attention given to diencephalon, midline commissures, hemispheric white matter, and cortex.

Results

An abnormal gray matter structure that we called hypothalamic adhesion across the anterior-inferior portion of the third ventricle was noted in 48.6%. The anterior commissure was in a low position in the lamina terminalis in 38%. Gross abnormalities of the corpus callosum/hippocampal commissure were found in 57%; they were, however, different from the abnormalities seen in classical commissural agenesis. An abnormal bundle of white matter forming a callosal ridge was noted on the dorsal callosal surface in 60%; it is believed to represent the aberrant cingular bundle recently identified using diffusion tensor imaging. Hemispheric white matter could be considered as normal in 48%, deficient posteriorly in 55%, and globally in 10%. Cortical posterior medial stenogyria was observed in 72%. Gray matter heterotopias were found in 19%. The posterior limbic cortex was thin and dysplastic in 46%. Hippocampi were commonly abnormal (85%), with atypical sulcation of the adjacent temporo-mesial cortex (93%).

Conclusion

Major structural abnormalities were common in Chiari II malformation and were probably not related to hydrocephalus. Important anatomical structures involved in neurocognitive function should be considered as factors in the prognostic assessment of Chiari II patients.

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References

  1. Cameron AH, Hill WC (1955) The Arnold–Chiari malformation in a sacred baboon (Papio hamadryas). J Pathol Bacteriol 70:552–554

    Article  PubMed  CAS  Google Scholar 

  2. Chumas P, Tyagi A, Livingston J (2001) Hydrocephalus—what’s new? Arch Dis Child Fetal Neonatal Ed 85:F149–F154

    Article  PubMed  CAS  Google Scholar 

  3. Dennis M, Jewell D, Edelstein K, Brandt ME, Hetherington R, Blaser SE, Fletcher JM (2006) Motor learning in children with spina bifida: intact learning and performance on a ballistic task. J Int Neuropsychol Soc 12:598–608

    Article  PubMed  Google Scholar 

  4. Detrait ER, George TM, Etchevers HC, Gilbert JR, Vekemans M, Speer MC (2005) Human neural tube defects: developmental biology, epidemiology, and genetics. Neurotoxicol Teratol 27:515–524

    Article  PubMed  CAS  Google Scholar 

  5. Di Virgilio G, Clarke S, Pizzolato G, Schaffner T (1999) Cortical regions contributing to the anterior commissure in man. Exp Brain Res 124:1–7

    Article  PubMed  Google Scholar 

  6. Duvernoy HM (2005) The human hippocampus. Springer, Berlin

    Google Scholar 

  7. el Gammal T, Mark EK, Brooks BS (1988) MR imaging of Chiari II malformation. AJR Am J Roentgenol 150:163–170

    PubMed  Google Scholar 

  8. Ettinger U, Picchioni M, Landau S, Matsumoto K, van Haren NE, Marshall N, Hall MH, Schulze K, Toulopoulou T, Davies N, Ribchester T, McGuire PK, Murray RM (2007) Magnetic resonance imaging of the thalamus and adhesio interthalamica in twins with schizophrenia. Arch Gen Psychiatry 64:401–409

    Article  PubMed  Google Scholar 

  9. Gilbert JN, Jones KL, Rorke LB, Chernoff GF, James HE (1986) Central nervous system anomalies associated with meningomyelocele, hydrocephalus, and the Arnold–Chiari malformation: reappraisal of theories regarding the pathogenesis of posterior neural tube closure defects. Neurosurgery 18:559–564

    Article  PubMed  CAS  Google Scholar 

  10. Cai W, Zhao H, Guo J, Li Y, Yuan Z, Wang W (2007) Retinoic acid-induced lumbosacral neural tube defects: myeloschisis and hamartoma. Childs Nerv Syst 23:549–554

    Article  PubMed  Google Scholar 

  11. Hori A, Stan AC (2004) Supracallosal longitudinal fiber bundle: heterotopic cingulum, dorsal fornix or Probst bundle? Neuropathology 24:56–59

    Article  PubMed  Google Scholar 

  12. Huber-Okrainec J, Blaser SE, Dennis M (2005) Idiom comprehension deficits in relation to corpus callosum agenesis and hypoplasia in children with spina bifida meningomyelocele. Brain Lang 93:349–368

    Article  PubMed  Google Scholar 

  13. Kriebel RM, McAllister JP 2nd (2000) Pathology of the hippocampus in experimental feline infantile hydrocephalus. Neurol Res 22:29–36

    PubMed  CAS  Google Scholar 

  14. Lammens M, Hiel JA, Gabreels FJ, van Engelen BG, van den Heuvel LP, Weemaes CM (2003) Nijmegen breakage syndrome: a neuropathological study. Neuropediatrics 34:189–193

    Article  PubMed  CAS  Google Scholar 

  15. Martin JA, Hamilton BE, Sutton PD, Ventura SJ, Menacker F, Kirmeyer S (2006) Births: final data for 2004. National Vital Statistics Reports 55:1–101

    Google Scholar 

  16. McLone DG, Knepper PA (1989) The cause of Chiari II malformation: a unified theory. Pediatr Neurosci 15:1–12

    Article  PubMed  CAS  Google Scholar 

  17. McLone DG, Dias MS (2003) The Chiari II malformation: cause and impact. Childs Nerv Syst 19:540–550

    Article  PubMed  Google Scholar 

  18. Naidich TP, Pudlowski RM, Naidich JB (1980) Computed tomographic signs of Chiari II malformation. II: midbrain and cerebellum. Radiology 134:391–398

    PubMed  CAS  Google Scholar 

  19. Naidich TP, Pudlowski RM, Naidich JB (1980) Computed tomographic signs of the Chiari II malformation. III: ventricles and cisterns. Radiology 134:657–663

    PubMed  CAS  Google Scholar 

  20. Naidich TP (1981) Cranial CT signs of the Chiari II malformation. J Neuroradiol 8:207–227

    PubMed  CAS  Google Scholar 

  21. Naidich TP, McLone DG, Fulling KH (1983) The Chiari II malformation: Part IV. The hindbrain deformity. Neuroradiology 25:179–197

    Article  PubMed  CAS  Google Scholar 

  22. Peach B (1965) Arnold–Chiari malformation: anatomic features of 20 cases. Arch Neurol 12:613–621

    PubMed  CAS  Google Scholar 

  23. Rakic P, Yakovlev PI (1968) Development of the corpus callosum and cavum septi in man. J Comp Neurol 132:45–72

    Article  PubMed  CAS  Google Scholar 

  24. Raybaud C, Girard N (2005) Malformations of the telencephalic commissures. Callosal agenesis and related disorders. Pediatr Neuroradiol 41–69

  25. Shim I, Ha Y, Chung JY, Lee HJ, Yang KH, Chang JW (2003) Association of learning and memory impairments with changes in the septohippocampal cholinergic system in rats with kaolin-induced hydrocephalus. Neurosurgery 53:416–425; discussion 425

    Article  PubMed  Google Scholar 

  26. Shu T, Shen WB, Richards LJ (2001) Development of the perforating pathway: an ipsilaterally projecting pathway between the medial septum/diagonal band of Broca and the cingulate cortex that intersects the corpus callosum. J Comp Neurol 436:411–422

    Article  PubMed  CAS  Google Scholar 

  27. Shuman RM (1995) The Chiari malformations: a constellation of anomalies. Semin Pediatr Neurol 2:220–226

    Article  PubMed  CAS  Google Scholar 

  28. Tew B, Laurence KM (1975) The effects of hydrocephalus on intelligence, visual perception and school attainment. Dev Med Child Neurol Suppl 129–134

  29. Tortori-Donati P, Rossi A, Biancheri R (2005) Brain malformations. Pediatric neuroradiology. Springer, Berlin Heidelberg New York

    Google Scholar 

  30. Tulipan N, Bruner JP, Hernanz-Schulman M, Lowe LH, Walsh WF, Nickolaus D, Oakes WJ (1999) Effect of intrauterine myelomeningocele repair on central nervous system structure and function. Pediatr Neurosurg 31:183–188

    Article  PubMed  CAS  Google Scholar 

  31. Tulipan N, Hernanz-Schulman M, Lowe LH, Bruner JP (1999) Intrauterine myelomeningocele repair reverses preexisting hindbrain herniation. Pediatr Neurosurg 31:137–142

    Article  PubMed  CAS  Google Scholar 

  32. Utsunomiya H, Takano K, Okazaki M, Mitsudome A (1999) Development of the temporal lobe in infants and children: analysis by MR-based volumetry. AJNR Am J Neuroradiol 20:717–723

    PubMed  CAS  Google Scholar 

  33. Vachha B, Adams R (2005) Influence of family environment on language outcomes in children with myelomeningocele. Child Care Health Dev 31:589–596

    Google Scholar 

  34. Vachha B, Adams R (2005) Myelomeningocele, temperament patterns, and parental perceptions. Pediatrics 115:e58–e63

    PubMed  Google Scholar 

  35. Vachha B, Adams RC, Rollins NK (2006) Limbic tract anomalies in pediatric myelomeningocele and Chiari II malformation: anatomic correlations with memory and learning—initial investigation. Radiology 240:194–202

    Article  PubMed  Google Scholar 

  36. Vonderahe AR (1937) Anomalous commissure of the third ventricle (aberrant dorsal supra-optic decussation). Arch Neurol Psychiatry 37:1283–1288

    Google Scholar 

  37. Wolpert SM, Anderson M, Scott RM, Kwan ES, Runge VM (1987) Chiari II malformation: MR imaging evaluation. AJR Am J Roentgenol 149:1033–1042

    PubMed  CAS  Google Scholar 

  38. Yeates KO, Enrile BG, Loss N, Blumenstein E, Delis DC (1995) Verbal learning and memory in children with myelomeningocele. J Pediatr Psychol 20:801–815

    Article  PubMed  CAS  Google Scholar 

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Acknowledgment

We acknowledge the assistance of Jack Fletcher, Ph.D. and support from National Institute of Child Health and Human Development Grant P01 HD35946 “Spina Bifida: Cognitive and Neurobiological Variability.”

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Authors and Affiliations

  1. Division of Neuroradiology, Diagnostic Imaging, The Hospital for Sick Children, Toronto, Canada

    Elka Miller, Elysa Widjaja, Susan Blaser, Maureen Dennis & Charles Raybaud

Authors
  1. Elka Miller
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  2. Elysa Widjaja
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  3. Susan Blaser
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  4. Maureen Dennis
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  5. Charles Raybaud
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Correspondence to Elka Miller.

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Miller, E., Widjaja, E., Blaser, S. et al. The old and the new: supratentorial MR findings in Chiari II malformation. Childs Nerv Syst 24, 563–575 (2008). https://doi.org/10.1007/s00381-007-0528-x

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  • DOI: https://doi.org/10.1007/s00381-007-0528-x

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