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

J Child Neurol. 2003 Jan;18(1):35-8. doi: 10.1177/08830738030180010601.

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 useof 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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Animals
  • Brain Neoplasms / diagnosis*
  • Brain Neoplasms / embryology
  • Brain Neoplasms / genetics
  • Cerebral Arteries / embryology
  • Cerebral Arteries / pathology
  • Chick Embryo
  • Child
  • Diagnosis, Differential
  • Dominance, Cerebral / physiology
  • Electroencephalography
  • Epilepsy, Temporal Lobe / diagnosis*
  • Epilepsy, Temporal Lobe / embryology
  • Epilepsy, Temporal Lobe / genetics
  • Facial Neoplasms / diagnosis*
  • Facial Neoplasms / embryology
  • Facial Neoplasms / genetics
  • Female
  • Frontal Lobe* / embryology
  • Frontal Lobe* / pathology
  • Humans
  • Male
  • Meninges / blood supply
  • Meninges / embryology
  • Meninges / pathology
  • Occipital Lobe* / embryology
  • Occipital Lobe* / pathology
  • Parietal Lobe* / embryology
  • Parietal Lobe* / pathology
  • Sturge-Weber Syndrome / diagnosis*
  • Sturge-Weber Syndrome / embryology
  • Sturge-Weber Syndrome / genetics
  • Temporal Lobe* / embryology
  • Temporal Lobe* / pathology