PNEUMOGRAPHIC DEMONSTRATION OF SUB-EPENDYMAL HETEROTOPIC CORTICAL GRAY MATTER IN CHILDREN

¹ From the Department of Radiology, Columbia University, College of Physicians and Surgeons, and the Radiological Service of the Neurological Institute, Columbia-Presbyterian Medical Center, New York, New York

Heterotopic cortical gray matter occurs as a consequence of migrational arrest of primitive nerve cells (neuroblasts) in the developing fetal cerebrum. In the mature brain, these hetenotopias may be subependymal; they deform the lateral ventricular surfaces and are demonstrable at cerebral pneumography.

Ten cases with autopsy findings are reviewed. An additional 4 patients are presented in whom the diagnosis is based on pneumographic and clinical criteria. The pneumographic features of subependymal cortical heterotopias include the following:

1. Nodular lateral vertricular irregularities; these are solid masses and may be single or multiple; their attachment is usually broad based. Commonly, a large area is involved, giving the ventricular wall a mammilated appearance. The third ventricle, aqueduct of Sylvius and fourth ventricle are never involved.

2. The nodules do not calcify.

3. The nodules do not change in number or size on serial studies.

4. The nodules commonly occur in the presence of other brain abnormalities, such as agenesis of the corpus calbosum, microcephaly, congenital aqueduct stenosis and cerebellar dysgenesis.

The differential diagnosis includes tuberous sclerosis, choroid plexus papilloma, ependymal seeding from malignant tumors, as well as anatomic variants.

Pneumographically demonstrable subependymal cortical heterotopias may be regarded as an index of significant insult sustained by the developing brain. Their presence correlates uniformly with retarded psychomotor development. The diagnosis in infancy or childhood is a warning that difficulties may be anticipated in motor and intellectual achievement.

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