[Ptosis as the leading symptom of Kearns-Sayre syndrome. A case report for differential diagnosis of external ophthalmoplegia]. / Ptosis als Leitsymptom eines Kearns-Sayre-Syndroms. Ein kasuistischer Beitrag zur Differentialdiagnosa der äusseren Ophthalmoplegie.

Progressive external ophthalmoplegia has generally been considered a brainstem syndrome, but new investigative techniques have identified a variety of etiologies. We report a 17-year-old girl in whom Kearns-Sayre syndrome presented as unilateral ptosis with progression to bilateral ptosis, and discuss the clinical, biochemical, electrophysiological, histopathological and computerized tomographical findings.

[Clinical and computed tomographic observations on newborn infants with generalized brain edema due to perinatal asphyxia]. / Klinische und computertomographische Verlaufsbeobachtungen bei Neugeborenen mit generalisiertem Hirnödem infolge perinataler Asphyxie.

We observed a sudden respiratory arrest in four term newborn infants after a clinically symptom-free period. There were no cardiac, pulmonary or metabolic changes responsible for these events. Signs of cerebral dysfunction existed (muscular hypotonia, jitterness, seizures). Cranial computerized tomographic scans were performed during the period of respirator treatment within the first week of life. The density of the brain structures was significantly decreased in all cases, three of the patients showed a complete compression of the lateral ventricles. These findings indicated severe brain swelling as a consequence of cerebral hypoxia. There was a history of umbilical cord occlusion in two cases. In the remaining patients we must assume an undetected hypoxic-ischemic episode prior to the onset of labor. We used hyperventilation, corticosteroids, phenobarbital, diuretics and fluid restriction for therapy. Later on the babies received special physiotherapy. Control CT-scans were performed during the fourth or fifth week of life. The findings were normal in one patient. Signs of mild focal brain atrophy developed in two babies. A more severe cortical atrophic lesion of both temporal lobes was found in one patient. He suffered from a slight cerebral palsy. No neurodevelopmental handicaps could be found in all the other patients on long term follow-up. The EEG examination was performed between the fifth and seventh month of life. No pathologic changes were observed. We conclude that severe generalized brain edema in the newborn is not necessarily followed by extensive brain damage. We think it important to develop more sensitive methods for detecting a hypoxic ischemic crisis preceding the birth.