Telencephalic leucoencephalopathy in premature infants dying after prolonged artificial respiration. Report on 6 cases.

This study reports clinical and neuropathological findings in six premature infants dying after prolonged assisted ventilation (IPPB and CPPB) due to pulmonary insufficiency (gestational age: 26.--32. week; birth weight 820--1400 Gm; respirator therapy 41--143 days; survival 104--263 days). During the rspirator therapy the children developed marked extensor rigidity of the trunc and spasticity of the extremities. Postmortem examination revealed cor pulmonale and right ventricular failure. Signs of marked perinatal brain damage were missed. The slightly atrophic brains showed predominant damage to the telencephalic white matter of varying intensity ranging from focal necroses to gliosis and retarded myelination. Constant findings were increased vascularisation and transformation of the premyelinating glia into astrocytes in the deep and subcortical white matter. This form of telencephalic leucoencephalopathy indicates the particular vulnerability of the developing white matter in conditions with chronic hypoxia, hypercapnia, acidosis and vascular congestion. The varying intensity of the lesions suggests that, in principle, minor lesions are either reversible or may be compensated in surviving children.