Semilobar Holoprosencephaly with Midline 'Seam': A Topologic and Morphogenetic Model Based Upon MRI Analysis

Takao Takahashi, S. Kinsman, N. Makris, E. Grant, C. Haselgrove, S. McInerney, D. N. Kennedy, Ta Takahashi, K. Fredrickson, S. Mori, V. S. Caviness

Research output: Contribution to journalArticle

21 Citations (Scopus)

Abstract

We present an MRI-based anatomic analysis of a series of seven human brains with the semilobar form of holoprosencephaly. The analysis defines a set of common descriptors for a pattern of topological anomaly which is uniform for the set of seven brains. The core of the anomaly is a rostro-caudally aligned midline gray matter 'seam' that extends from the telencephalic-suprachiasmatic junctional region to abut the posterior aspect of the callosal commissure. The seam forms the ventricular roof throughout its extent. Rostrally it is formed by the conjoined heads of caudate/accumbens nuclei. It continues caudally as a gray matter bridge in the fundus of the interhemispheric fissure, where it bridges right and left neocortex. Fornix, septal nuclei and septal limb of the choroid plexus are absent, and the telencephalic ventricles communicate with the diencephalic via open septal limbs of the choroid fissures. By contrast, the temporal limb of hippocampal formation and the choroid plexus are normal and the temporal limb of the choroid fissure is closed. This topological anomaly of conjoined left and right cortical and nuclear gray matter into a midline seam and absent septal structures is thus confined to the region of the midline telencephalic hemisphere evagination. Total telencephalic growth is strongly correlated with the length of this topologically abnormal midline telencephalic segment. The set of findings is consistent with graded failure of induction of rostral to caudal specification in the midline rostral telencephalic zone.

Original languageEnglish
Pages (from-to)1299-1312
Number of pages14
JournalCerebral Cortex
Volume13
Issue number12
DOIs
Publication statusPublished - 2003 Dec

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Holoprosencephaly
Telencephalon
Extremities
Choroid Plexus
Choroid
Septal Nuclei
Corpus Callosum
Caudate Nucleus
Neocortex
Brain
Hippocampus
Growth
Gray Matter

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Takahashi, T., Kinsman, S., Makris, N., Grant, E., Haselgrove, C., McInerney, S., ... Caviness, V. S. (2003). Semilobar Holoprosencephaly with Midline 'Seam': A Topologic and Morphogenetic Model Based Upon MRI Analysis. Cerebral Cortex, 13(12), 1299-1312. https://doi.org/10.1093/cercor/bhg077

Semilobar Holoprosencephaly with Midline 'Seam' : A Topologic and Morphogenetic Model Based Upon MRI Analysis. / Takahashi, Takao; Kinsman, S.; Makris, N.; Grant, E.; Haselgrove, C.; McInerney, S.; Kennedy, D. N.; Takahashi, Ta; Fredrickson, K.; Mori, S.; Caviness, V. S.

In: Cerebral Cortex, Vol. 13, No. 12, 12.2003, p. 1299-1312.

Research output: Contribution to journalArticle

Takahashi, T, Kinsman, S, Makris, N, Grant, E, Haselgrove, C, McInerney, S, Kennedy, DN, Takahashi, T, Fredrickson, K, Mori, S & Caviness, VS 2003, 'Semilobar Holoprosencephaly with Midline 'Seam': A Topologic and Morphogenetic Model Based Upon MRI Analysis', Cerebral Cortex, vol. 13, no. 12, pp. 1299-1312. https://doi.org/10.1093/cercor/bhg077
Takahashi, Takao ; Kinsman, S. ; Makris, N. ; Grant, E. ; Haselgrove, C. ; McInerney, S. ; Kennedy, D. N. ; Takahashi, Ta ; Fredrickson, K. ; Mori, S. ; Caviness, V. S. / Semilobar Holoprosencephaly with Midline 'Seam' : A Topologic and Morphogenetic Model Based Upon MRI Analysis. In: Cerebral Cortex. 2003 ; Vol. 13, No. 12. pp. 1299-1312.
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