Congenital diencephalic and brain stem damage: neuropathologic study of three cases.

Neuropathologic findings and clinical features of three patients with congenital brain stem damage are reported. All of the infants were premature (32 - 36 weeks' gestation) and experienced respiratory difficulty in the immediate postnatal period. One infant was moribund at deliver, dying 3 h after birth. In two infants who survived for 12 and 16 days, detailed neurologic examinations demonstrated multiple cranial nerve palsies with absence of facial expression. Autopsies revealed similar changes in the brains of all three infants: bilateral symmetrical lesions were found predominantly in the thalamus and brain stem. Histological features common to all three cases included prominent basophilic mineralized neurons, neuronal loss astrocytosis and axonal spheroids. Necrosis was observed in two cases. These changes are most compatible with one or more episodes of total asphyxia during fetal life. This study supports the hypothesis that, in some cases, Möbius' syndrome is the result of intrauterine asphyxia.