In the brain with the subependymal nodular dysplasia, an abnormal tuberous expansion of the caudate nucleus was detected on MRI (Fig. 1f) and in histological sections (Fig. 1g, h). Only the ependyma separated the tuber-like expansion of the caudate from the ventricle lumen. The very thick subependymal cell layer that was present close to the caudate was substituted by loosely arranged and poorly differentiated neurons in the affected area.