Abstract
Objective Describe a rare and complicated case.
A 46 y/o female with CRS presented, post head-strike, with imaging showing remote right parietal infarct, colpocephaly and thinning of the corpus callosum.
Colpocephaly is a rare condition, reported in only a dozen adults. It occurs in 1:700,000 live births and is usually diagnosed before birth. It is an imaging diagnosis of the lateral ventricles: when the widest portion of posterior horn is greater than three times that of the anterior horn. It may be unilateral, bilateral, or asymmetric.
Pathophysiologically, it appears to reflect the retention of the early fetal configuration of enlarged ventricles that, usually, are slowly filled by the radial out-migration of cells from the subventricular zone (SVZ), to the Subplate (Sp) with the formation of white matter being the remnant of the subplate.
First reported in 1941, it has been associated with: dysgenesis of the corpus callosum, pachygyria, lissencephaly, schizencephaly, microcephaly, meningomyelocoele. Aetiologically it has been associated with intra-uterine infection, Intra-Uterine Growth Retardation, perinatal anoxic-hypoxic injury, maternal drugs, genetics, migration disorder. It may affect: cognition, cerebral palsy, gait, speech, epilepsy, vision.
Conclusion Remarkably, neurologically normal (or at least asymptomatic) children and adults have been reported although few have had extensive, appropriate, neuropsychological assessments. Neuropsychological assessment is marred by the lack of normalized data for such a unique group. Cerebral connectivity in this condition remains unexplored.
