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.

Central nervous system complications and management in sickle cell disease

“…58,60 The majority of patients have focal abnormality on neuroimaging or electroencephalography 61 and cerebrovascular disease (see supplemental Figure 3A-D). 62 Given the high rate of moyamoya in individuals with SCD, we do not recommend hyperventilation during electroencephalography because of the risk of ischemia. 63 …”

How I treat and manage strokes in sickle cell disease

“…Seizures: the prevalence of seizures in children with SCD is 10 times that of the general population. 18,19 Prengler et al found in a cohort of individuals with SCD, that those with seizures had increased perfusion and electroencephalographic abnormalities, suggesting that vasculopathy and focal hypoperfusion may be factors in the development of SCD-associated seizures. 18 In case 1, an electroencephalogram was done, which revealed generalized nonspecific cerebral dysfunction and no ictal or interictal epileptiform discharges were recorded.…”

Central Nervous System Complications of Sickle Cell Disease in Children: An Overview

“…The prevalence of seizures in children with SCD is 10 times that of the general population (Prengler et al, 2005). In a small series, four of six children with SCD and recent seizures were found to have abnormal TCD velocities and all had relatively (Prengler et al, 2005). In a series of 17 patients with sickle cell disease and epilepsy (Liu, Gzesh, & Ballas, 1994), 11 had generalized tonic-clonic seizures, 2 had partial and 2 had poorly characterized seizures.…”

“…The prevalence of seizures in children with SCD is 10 times that of the general population (Prengler et al, 2005). In a small series, four of six children with SCD and recent seizures were found to have abnormal TCD velocities and all had relatively (Prengler et al, 2005).…”