Advanced

AJNR - American Journal of Neuroradiology

This Article Figures Only Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Barkovich, A. J. Articles by Hahn, J. S. Search for Related Content PubMed PubMed Citation Articles by Barkovich, A. J. Articles by Hahn, J. S.

American Journal of Neuroradiology 23:143-150, January 2002
© 2002 American Society of Neuroradiology

PEDIATRICS

Analysis of the Cerebral Cortex in Holoprosencephaly with Attention to the Sylvian Fissures

A. James Barkovich^a,b,d, Erin M. Simon^a,c,d, Nancy J. Clegg^a,d, Steven L. Kinsman^a,d and Jin S. Hahn^a,d

^a Department of Diagnostic Radiology, University of California, San Francisco, San Francisco, CAI
^b Department of Neurology and Pediatrics, University of California, San Francisco, San Francisco, CA
^c Department of Radiology, Children’s Hospital of Philadelphia, Philadelphia, PA
^d The Carter Centers for Brain Research in Holoprosencephaly and Related Malformations

Address reprint requests to A. James Barkovich, Division of Neuroradiology, L 371, University of California, San Francisco, 505 Parnassus Avenue, Box 0628, San Francisco, CA 94143

BACKGROUND AND PURPOSE: Analysis of specific features in the brain of patients with holoprosencephaly (HPE) may clarify normal and abnormal brain development and help predict outcomes for specific children. We assessed sulcal and gyral patterns of cerebral cortex in patients with HPE and developed a method of grading brain development.

METHODS: Neuroimaging studies (75 MR imaging, 21 CT) of 96 patients with HPE were retrospectively reviewed, with specific attention paid to the cerebral cortex. Thickness of cortex, width of gyri, and depth of sulci were assessed subjectively and by measurement. The angle between lines drawn tangential to the sylvian fissures ("sylvian angle") was measured in each patient with HPE and in 20 control patients.

RESULTS: Thickness of cortex was normal in all 96 patients. Gyral shape and width and sulcal depth were normal in 80 patients. Twelve patients, all with very severe HPE and microcephaly, had reduced sulcal depth, diffusely in eight and limited to the anteromedial cortex in four with lobar HPE. Four patients had subcortical heterotopia, located anterior to the interhemispheric fissure, associated with shallow sulci in the overlying cortex. Sylvian fissures were displaced further anteriorly and medially as HPE became more severe, until, in the most severe cases, no sylvian fissures could be identified. Sylvian angle measurements corresponded closely with severity of HPE, being largest in the most severe and smallest in the least severe cases. All patients with HPE had sylvian angles significantly larger than the mean of 15° measured in the control patients.

CONCLUSION: The only true malformations of cortical development were subcortical heterotopia. However, diffuse and focal abnormal sulci were observed. We propose our sylvian angle measurement of extent of frontal lobe development as an objective means of quantifying the severity of HPE.

This article has been cited by other articles:

				F Lacbawan, B D Solomon, E Roessler, K El-Jaick, S Domene, J I Velez, N Zhou, D Hadley, J Z Balog, R Long, et al. Clinical spectrum of SIX3-associated mutations in holoprosencephaly: correlation between genotype, phenotype and function J. Med. Genet., June 1, 2009; 46(6): 389 - 398. [Abstract] [Full Text] [PDF]

				N. Rollins Semilobar Holoprosencephaly Seen with Diffusion Tensor Imaging and Fiber Tracking AJNR Am. J. Neuroradiol., September 1, 2005; 26(8): 2148 - 2152. [Abstract] [Full Text] [PDF]

				J.-i. Takanashi, A. J. Barkovich, N. J. Clegg, and M. R. Delgado Middle Interhemispheric Variant of Holoprosencephaly Associated with Diffuse Polymicrogyria AJNR Am. J. Neuroradiol., March 1, 2003; 24(3): 394 - 397. [Abstract] [Full Text] [PDF]

				A.J. Lewis, E.M. Simon, A.J. Barkovich, N.J. Clegg, M.R. Delgado, E. Levey, and J.S. Hahn Middle interhemispheric variant of holoprosencephaly: A distinct cliniconeuroradiologic subtype Neurology, December 24, 2002; 59(12): 1860 - 1865. [Abstract] [Full Text] [PDF]

				A.J. Barkovich, E.M. Simon, O.A. Glenn, N.J. Clegg, S.L. Kinsman, M. Delgado, and J.S. Hahn MRI shows abnormal white matter maturation in classical holoprosencephaly Neurology, December 24, 2002; 59(12): 1968 - 1971. [Abstract] [Full Text] [PDF]

				L.L. Plawner, M.R. Delgado, V.S. Miller, E.B. Levey, S.L. Kinsman, A.J. Barkovich, E.M. Simon, N.J. Clegg, V.T. Sweet, E.E. Stashinko, et al. Neuroanatomy of holoprosencephaly as predictor of function: Beyond the face predicting the brain Neurology, October 8, 2002; 59(7): 1058 - 1066. [Abstract] [Full Text] [PDF]