Epidural hematomas in a child with Hutchinson-Gilford progeria syndrome.
BACKGROUND
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.


METHODS
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.


CONCLUSIONS
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.
Mandera
Marek
M
Division of Pediatric Neurosurgery, Department of Pediatric Surgery, Silesian University School of Medicine, ul. Medyków 16, 40-752 Katowice, Poland. marekman@mp.pl
Larysz
Dawid
D
Pajak
Jacek
J
Klimczak
Andrzej
A
D002648:Child; D006259:Craniocerebral Trauma / Q000150:complications*; D015600:Glasgow Coma Scale; D006407:Hematoma, Epidural, Cranial / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D006801:Humans; D019586:Intracranial Hypertension / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D008297:Male; D010291:Paresis / Q000209:etiology; D011371:Progeria / Q000150:complications*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome