The prevalence of cerebral vasculopathy in 76 patients with sickle cell disease (SCD), with and without seizures, and of magnetic resonance (MR) perfusion abnormality in those with recent seizures, was determined in a study at Great Ormond Street Hospital for Children, London, UK. Neurologic complications of SCD in 47 patients (23 male; median age 12 years; range 1.7-27 years) included recurrent seizures in 6, stroke in 9, transient ischemic attack (8), headaches (9), behavior and/or learning difficulties (9), or abnormal transcranial Doppler, more frequent in the seizure (4/6) and nonseizure (26/41; 63%) groups than in the asymptomatic (10/29; 34%) group. All seizure patients had relative decreased cerebral perfusion, and perfusion abnormalities were ipsilateral to electroencephalographic abnormalities. The development of seizures in patients with SCD is related to vasculopathy and focal hypoperfusion. [1]

COMMENT. Seizures are reported in 12 to 14% of patients with sickle cell disease (SCD), and the prevalence in children with SCD is 10 times the general population (Liu JE et al, 1994; cited by authors). Vasculopathy and ischemia are factors in the occurrence of seizures in SCD. MR perfusion studies showing relative hypoperfusion in gray and white matter may be indicative of the cause of seizures when other neuroimaging methods are negative.