A 6-day-old neonate with tuberous sclerosis having brain and cardiac tumors is reported. The patient presented with a seizure 20 minutes after birth. Cranial ultrasonography and computed tomography on admission showed a large and round high-density tumor lesion in the left frontal lobe. Echocardiography also showed a large tumor lesion beside the left ventricle wall, and a diagnosis of tuberous sclerosis was promptly made. Two months later, a periventricular calcified lesion was found on the demonstrated portion, and the tumor lesion initially observed in the brain had disappeared. These findings suggest that the initial tumor lesion found in the brain developed into a typical calcified lesion.
The development of the human putamen was studied using complete serial sections from the brains of 6 fetuses at 21 23 27 30 33 and 34 weeks of gestation (WG). Morphometric analysis revealed the following data. (1) The total number of putaminal neurons increased gradually from 21 to 33 WG, suggesting that the migration of putaminal neurons continues at least up to 33 WG, and the neuronal number increased markedly after 33 WG. (2) No neuronal death was observed from 21 to 34 WG. (3) Large-sized neurons 4–5 times the area of small-sized neurons appeared from 30 WG onward. The ratio of large neurons to the total putaminal neurons increased by up to 1.4% at 33 and 34 WG.
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