An infantile-onset, severe, yet sporadic seizure pattern is common in Sturge-Weber syndrome.
The young age of onset and frequently intractable nature of seizures associated with Sturge-Weber syndrome (SWS) have been well-reported in large studies. However, many clinicians also anecdotally describe prolonged but sporadic seizure clusters. Over a 5-year period, 77 children and adults with SWS and at least one reported seizure were referred to and evaluated at the Hunter Nelson Sturge-Weber Center at the Kennedy Krieger Institute. The median age of seizure onset was 6 months with 43 (56%) presenting <1 year of age. Age at seizure onset < or = 6 months was associated with increased hemiparesis, but not seizures. A characteristic pattern of clustering, intense seizures followed by prolonged seizure-free periods was reported in 30 (39%), but was not associated with worse prognosis. This seizure pattern appears to be common with SWS and leads to difficult treatment decisions, especially in regard to the timing of potential surgical resection.
Kossoff
Eric H
EH
The Johns Hopkins Hospital, Baltimore, MD 21287-1000, USA. ekossoff@jhmi.edu
Ferenc
Lisa
L
Comi
Anne M
AM
D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D016000:Cluster Analysis; D015897:Comorbidity; D005260:Female; D007839:Functional Laterality / Q000502:physiology; D006801:Humans; D007223:Infant; D008297:Male; D010291:Paresis / Q000175:diagnosis / Q000453:epidemiology; D011379:Prognosis; D012640:Seizures / Q000175:diagnosis* / Q000453:epidemiology; D012720:Severity of Illness Index; D013341:Sturge-Weber Syndrome / Q000175:diagnosis* / Q000453:epidemiology