Cerebral inception: a “brain” within a brain
The present case has been reported in the journal Neurology.
An 8-week-old term infant with congenital nephrotic syndrome developed stiffening following general anesthetic. His neurologic examination and EEG were normal.

MRI brain demonstrated a large mass of dysplastic brain isointense to cortex with intervening “sulcal” spaces (figure). The infant continued to have infrequent seizures.

Glioneuronal hamartomas are well-circumscribed, non-neoplastic, masses of disorganized mature neuronal and glial cells arising from abnormal neural development. Accompanying gray and white matter heterotopia are common.

Glioneuronal hamartomas make up a small but significant proportion of patients operated on for drug-resistant epilepsy. Very large hamartomas, as described, are rare.

Source: http://n.neurology.org/content/92/4/e392
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