Received: 25 August 2002 Published online: 19 November 2002 © Springer-Verlag 2002 Abstract Introduction: Hutchinson- Gilford progeria syndrome (HGPS) is a rare genetic disorder. It is char- acterized by severe growth failure, premature aging, and very early ath- erosclerosis with coronary artery dis- ease and cerebrovascular disease. Case report: A 10-year-old boy with HGPS was admitted to our depart- ment because of progressive deterio- ration after a mild head injury. The CT scans revealed epidural hemato- ma 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 hemato- mas were evacuated. In a few days the neurological symptoms disap- peared, 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 atheroscle- rosis of intracranial vessels was responsible for formation of the hematomas. Keywords Hutchinson-Gilford progeria syndrome · Head injury · Epidural hematoma · Atherosclerosis Childs Nerv Syst (2003) 19:63–65 DOI 10.1007/s00381-002-0679-8 CASE REPORT Marek Mandera Dawid Larysz Jacek Pajak Andrzej Klimczak Epidural hematomas in a child with Hutchinson-Gilford progeria syndrome Introduction Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder, with an estimated incidence of 1 per 250,000 births. Although it was first described in the 1880s, so far only approximately 100 cases of progeria have been reported in the literature. The pathogenesis of the disease is unknown. The patients usually appear nor- mal at birth. It is characterized by severe growth failure, premature aging, and very early atherosclerosis with cor- onary artery and cerebrovascular disease. Patients with HGPS progressively develop failure to thrive, alopecia, loss of subcutaneous fat, scleroderma, and stiffening of various joints. The median lifespan is 13 years, and death is most frequently caused by cardiovascular com- plications [1, 3, 5]. Typical manifestations develop grad- ually and are evident by the 1st or 2nd year of life. Those affected are remarkably similar in physical appearance, being characterized by short stature, alopecia, craniofa- cial disproportion, micrognathia, hypoplastic mandible, and beak-like nose. Decreased subcutaneous fat, atrophic skin, sclerodermoid lesions, mottling hyperpigmentation, prominent scalp veins, prominent eyes, protruding ears with no earlobes, faint midfacial cyanosis, delayed clo- sure of fontanels and sutures, delayed dentition, horse- riding stance, thin limbs with prominent stiff joints, coxa valga, skeletal hypoplasia and dysplasia, dystrophic nails, and high-pitched voice are the other characteristic features [1, 5, 8]. Case report A 10-year-old boy in whom HGPS had been diagnosed earlier was admitted to our department because he had symptoms of intracra- nial hypertension after a head injury. The boy had fallen down in his home and suffered the trauma without loss of consciousness. Presented at the XVIII Congress of the European Society for Pediatric Neurosurgery, 14–18 June 2002, Kiruna, Sweden M. Mandera ( ✉ ) · D. Larysz · J. Pajak A. Klimczak Division of Pediatric Neurosurgery, Department of Pediatric Surgery, Silesian University School of Medicine, ul. Medyków 16, 40–752 Katowice, Poland e-mail: marekman@mp.pl Tel.: +48-32-2071826 Fax: +48-32-2071802