title: Sickle cell disease: ischemia and seizures.
abstract: Although the prevalence of seizures in children with sickle cell disease (SCD) is 10 times that of the general population, there are few prospectively collected data on mechanism. With transcranial Doppler and magnetic resonance imaging (MRI) and angiography, we evaluated 76 patients with sickle cell disease, 29 asymptomatic and 47 with neurological complications (seizures, stroke, transient ischemic attack, learning difficulty, headaches, or abnormal transcranial Doppler), who also underwent bolus-tracking perfusion MRI. The six patients with recent seizures also had electroencephalography. Group comparisons (seizure, nonseizure, and asymptomatic) indicated that abnormal transcranial Doppler was more common in the seizure (4/6; 67%) and nonseizure (26/41; 63%) groups than in the asymptomatic (10/29; 34%) group (chi2; p = 0.045), but abnormal structural MRI (chi2; p = 0.7) or magnetic resonance angiography (chi2; p = 0.2) were not. Relative decreased cerebral perfusion was found in all seizure patients and in 16 of 32 of the remaining patients with successful perfusion MRI (p = 0.03). In the seizure patients, the perfusion abnormalities in five were ipsilateral to electroencephalographic abnormalities; one had normal electroencephalogram results. These findings suggest that vasculopathy and focal hypoperfusion may be factors in the development of sickle cell disease-associated seizures.
authors.affiliation: Neurosciences Unit, Institute of Child Health, University College London, London, United Kingdom. m.prengler@ich.ucl.ac.uk
authors.forename: Mara
authors.initials: M
authors.lastname: Prengler
authors.forename: Steven G
authors.initials: SG
authors.lastname: Pavlakis
authors.forename: Stewart
authors.initials: S
authors.lastname: Boyd
authors.forename: Alan
authors.initials: A
authors.lastname: Connelly
authors.forename: Fernando
authors.initials: F
authors.lastname: Calamante
authors.forename: W K
authors.initials: WK
authors.lastname: Chong
authors.forename: Dawn
authors.initials: D
authors.lastname: Saunders
authors.forename: Timothy
authors.initials: T
authors.lastname: Cox
authors.forename: Michael
authors.initials: M
authors.lastname: Bynevelt
authors.forename: Roderick
authors.initials: R
authors.lastname: Lane
authors.forename: Aidan
authors.initials: A
authors.lastname: Laverty
authors.forename: Fenella J
authors.initials: FJ
authors.lastname: Kirkham
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000453:epidemiology / Q000473:pathology; D002544:Cerebral Infarction / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D004569:Electroencephalography / Q000379:methods; D005260:Female; D007839:Functional Laterality; D049690:History, Ancient; D006801:Humans; D007223:Infant; D007511:Ischemia / Q000150:complications* / Q000453:epidemiology*; D018810:Magnetic Resonance Angiography / Q000379:methods; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D012640:Seizures / Q000145:classification / Q000453:epidemiology / Q000209:etiology* / Q000473:pathology; D017585:Ultrasonography, Doppler, Transcranial / Q000379:methods