Encephalofacial angiomatosis sparing the occipital lobe and without facial nevus: On the spectrum of Sturge-Weber syndrome variants?

Anne M. Comi, Richard Fischer, Eric H. Kossoff

Research output: Contribution to journalArticlepeer-review

Abstract

We report two cases of leptomeningeal angiomatosis in atypical frontoparietotemporal locations without an associated facial port-wine stain. Evidence of a leptomeningeal angioma was found in each when they were evaluated for headaches and seizures. The diagnosis of a leptomeningeal angioma was suggested by calcifications noted on computed tomographic scan of the head and confirmed with contrast-enhanced magnetic resonance images of the brain. We hypothesize that given the lack of occipital involvement with the angioma, and therefore the noncontiguous nature of this lesion with the developing upper facial ectoderm, the failure to develop a facial angioma would be expected. We found that the use of an anticonvulsant along with a migraine prophylactic medication appeared to have the greatest efficacy in these two cases, whereas anticonvulsants alone were less helpful. This diagnosis should be considered in any child presenting with seizures or complicated migraines and intracranial calcifications.

Original languageEnglish (US)
Pages (from-to)35-38
Number of pages4
JournalJournal of child neurology
Volume18
Issue number1
DOIs
StatePublished - Jan 1 2003

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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