CLINICAL, PATHOGENETIC AND NEUROPATHOLOGICAL CORRELATES IN DYSTONIC CEREBRAL-PALSY

Studies were made of three autopsied cases of dystonic (changing tone) cerebral palsy with the aim of correlating the clinical characteristics and pathogenetic factors with the neuropathological lesions. Clinically all three patients were small-for-dates and born at gestational ages of 31-34 weeks. They sustained either severe birth asphyxia or repeated hypoxic episodes with respiratory arrest, and all three had moderate hyperbilirubinemia during the perinatal period. All of them developed an identical non-progressive extrapyramidal cerebral palsy syndrome. Mental capacity was less affected than motor performance, which remained at a neonatal level. From the pathogenetic point of view, prenatal malnutrition, preterm birth, pre- and perinatal hypoxia, acidosis and icterus were considered to be cumulative brain-damaging factors in each of the three patients. The pattern of combined partial and total asphyxia could be discerned in all the cases. The outstanding neuropathological correlate was selective bilateral sclerosis of the globus pallidus. It is proposed that the time of operation of the above factors in relation to the developmental stage of the globus pallidus determined the site of the cerebral damage. This hypothesis finds support in recent experiments with graded asphyxia in Rhesus monkeys. © 1979 Springer-Verlag.