1/3. Epidural hematomas in a child with Hutchinson-Gilford progeria syndrome.INTRODUCTION. Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder. It is characterized by severe growth failure, premature aging, and very early atherosclerosis with coronary artery disease and cerebrovascular disease. CASE REPORT. A 10-year-old boy with HGPS was admitted to our department because of progressive deterioration after a mild head injury. The CT scans revealed epidural hematoma in posterior fossa and another one in the temporal region on the left side. On admission the child was given an estimated score of 10 on the GCS. Neurological examination revealed right hemiparesis. The boy was operated on, and both hematomas were evacuated. In a few days the neurological symptoms disappeared, and he was discharged from the hospital with only residual, minimal right hemiparesis. CONCLUSION. Intracranial pathology was certainly caused by the head trauma, but was more severe than would have been expected had the trauma been the sole cause. We suggest that progressive atherosclerosis of intracranial vessels was responsible for formation of the hematomas.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/3. The pseudopapilledema of neonatal-onset multisystem inflammatory disease.PURPOSE: To report a rare case of neonatal-onset multisystem inflammatory disease with serial photographs to characterize the optic nerve findings. DESIGN: Observational case report. methods: A 6-year-old girl with neonatal-onset multisystem inflammatory disease, who had received systemic corticosteroid therapy for 5 years, had bilateral fibrillar opacities that surrounded the optic disks and extended into the peripapillary nerve fiber layer and vessels. A magnetic resonance imaging examination and lumbar puncture revealed elevated intracranial pressure. RESULTS: The elevated intracranial pressure returned to normal following a corticosteroid taper. optic disk photographs, taken 4 years earlier, were subsequently obtained. The optic disk appearance had remained unchanged over the 4-year period, consistent with a pseudopapilledema. CONCLUSION: The optic disk appearance is not consistent with papilledema from increased intracranial pressure. The optic disk findings, in conjunction with the underlying inflammatory syndrome, suggest an infiltrative etiology for the atypical optic nerve findings in neonatal-onset multisystem inflammatory disease.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/3. Management of haemorrhagic type moyamoya disease with intraventricular haemorrhage during pregnancy.Moyamoya ("hazy puff-of-smoke") disease represents a rare condition with progressive narrowing and occlusion of basal cranial vessels with secondary specific neoangiogenesis; we report on a 25-year-old primigravida with known moyamoya disease who suffered from acute bilateral intraventricular haemorrhage at 24 weeks gestation. She underwent bilateral external ventricular drainage and intraventricular recombinant tissue plasminogen activator (r-TPA) lysis was performed. At 34 weeks' gestation, a healthy girl was delivered via Caesarean section. Encephalomyosynangiosis (EMS) and extra-intracranial (EC/IC) bypass surgery were performed six and eight months after delivery, respectively. The patient recovered almost completely and showed only mild residual deficits. Prompt diagnosis and immediate interdisciplinary treatment might have been the key for optimal maternal and neonatal outcome in our patient.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |