An infantile-onset, severe, yet sporadic seizure pattern is common in Sturge-Weber syndrome.

TitleAn infantile-onset, severe, yet sporadic seizure pattern is common in Sturge-Weber syndrome.
Publication TypeJournal Article
Year of Publication2009
AuthorsKossoff EH, Ferenc L, Comi AM
JournalEpilepsia
Volume50
Issue9
Pagination2154-7
Date Published2009 Sep
Abstract

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.

DOI10.1002/ddrr.64
Alternate JournalEpilepsia