title: Severe unilateral headache caused by skull bone infarction with epidural haematoma in a patient with sickle cell disease.
abstract: BACKGROUND
  The clinical manifestations of sickle cell disease (SCD) vary, but may be attributed to vaso-occlusion, chronic hemolytic anemia, and infections as a result of functional asplenia. We report a case of a man who presented with severe headache caused by an uncommon complication of SCD.
  
  
  METHODS
  A 19-year-old Surinamer man presented to the emergency department with severe headache. The progressive headache started one day previously. The headache was located frontotemporally on the right side. It was pulsating with paroxysms of fierce pain. There was no nausea or vomiting. The medical history reported sickle cell disease of the HbSC type. The physical and neurological examination was normal. He was afebrile with a blood pressure of 118/72 mmHg. Blood tests and CSF investigation showed no abnormalities. CT-scan of the head was normal. The headache disappeared after two days. Eight days later he presented again, with a relapsing severe headache. Physical, neurological examination and blood investigations were normal. MRI now showed infarction located in the parietal skull bone, with a small adjacent epidural hematoma. The headache disappeared gradually over 8 days. Repeat MRI one month later showed complete disappearance of the epidural hematoma. The first headache episode was thought to be due to the initial skull bone infarction as no epidural hematoma had been present initially. The second headache episode was thought to be due to the development of the epidural hematoma.
  
  
  CONCLUSIONS
  A skull bone infarction is an uncommon complication of SCD, as typically these are located in the long bones. Even more uncommon is a epidural hematoma which was probably the result of the altered bone and vessel-wall structure following the skull bone infarction. To our knowledge this is the first case reporting a skull-bone infarction with adjacent spontaneous epidural hematoma in an adult with sickle cell disease of the HbSC type. Our case emphasizes the need to recognize skull infarction and a concomitant spontaneous epidural hematoma as a possible complication of SCD.
authors.affiliation: Haga Hospital, Pelikaanhof 66 g, Leiden, The Netherlands. s.arends@hagaziekenhuis.nl
authors.forename: Samuel
authors.initials: S
authors.lastname: Arends
authors.forename: Jan Adriaan
authors.initials: JA
authors.lastname: Coebergh
authors.forename: Jean Louis
authors.initials: JL
authors.lastname: Kerkhoffs
authors.forename: Adrianus
authors.initials: A
authors.lastname: van Gils
authors.forename: Hille
authors.initials: H
authors.lastname: Koppen
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications*; D006261:Headache / Q000209:etiology*; D006407:Hematoma, Epidural, Cranial / Q000209:etiology* / Q000473:pathology; D006801:Humans; D007238:Infarction / Q000209:etiology* / Q000473:pathology; D008297:Male; D012886:Skull / Q000098:blood supply*; D055815:Young Adult