Date: 2008-03-03 21:45 |
Priority: more information 3 |
Submitted By:
David Kennedy (dnkennedy)
|
Assigned To: more information
Nobody (None) |
Pubmed ID: 15173630 | Authors: Takahashi TS, Kinsman S, Makris N, Grant E, Haselgrove C, McInerney S, Kennedy DN, Takahashi TA, Fredrickson K, Mori S, Caviness VS. |
Citation: J Neurocytol. 2004 Jan;33(1):23-35 | URL: http://www.ncbi.nlm.nih.gov/pubmed/15173630 |
Summary: more information Holoprosencephaly--topologic variations in a liveborn series: a
general model based upon MRI analysis. |
Detailed description |
We present an MRI-based
anatomic analysis of a series of 9 human brains, representing
lobar, semilobar and alobar forms of holoprosencephaly. The
analysis of these variable forms of the malformation is based upon
a topologic systematics established in a prior analysis of a
homogeneous set of semilobar malformations. This systematics has
the dual advantage that it serves both as a uniform reference for
qualitative description and as a quantitative descriptive base for
mathematical correlations between parameters of topology and of
growth and development. Within this systematics, the prosencephalic
midline is divided from caudal to rostral into diencephalic
(DD-right and left, subthalamus through suprachiasmatic junction
with telencephalon), telencephalic (TT-right and left,
suprachiasmatic border of telencephalon midline to hippocampal
commissure) and diencephalic-telencephalic (DT-right and
left-hippocampal commissure through temporal limb of choroid
fissure) segments. The topologic abnormality of the initial
semilobar series was expressed in an orderly rostral to caudal
gradient along the TT segment. In each malformation, normal midline
topology began with a small posterior corpus callosum. Although the
topologic anomaly in the present series invariably also involved
the TT segment, this involvement was not continuous and was
variably associated with anomalies of the DD in 6 and unilaterally
of the DT in 1 brain. In the present as well as with the earlier
series of HPE malformations but not in "normative brains," total
telencephalic growth is strongly correlated with the length of the
midline telencephalic segment. We propose that this system of
analysis will be sensitive to the developmental stage and locus of
expression of genetic and non-genetic determinants of the formal
origin of HPE. For all of the present series, karyotype anlyses
were normal. Mutations in the Shh and Zic2 genes were excluded in 2
cases.
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