Sturge-weber syndrome (sws): Electroclinical and neuroradiological features

C. Di Bonaventura, J. Fattouch, F. Izzi, A. Francia, P. P. Quarato, C. Scoppetta, M. Serrao, S. Ricci, A. T. Giallonardo, M. Manfredl

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Introduction SWS is a cutaneous angiomatosis associated with CNS abnormalities. Etiology and pathogenesis are unknown. It usually is characterized by facial angioma, intracranial angioma and buphthalmos. Intracranial angioma is revealed by neuroimaging as excessive vascularization in the occipital-parietal region that produces necrosis in the subcortex with subsequent calcium deposit. Typical clinical features are epilepsy, controlateral hemiparesis and mental deficiency. The diagnosis of SWS can be difficult because of neuroradiological variability and the polymorphism of clinical features. Material and methods We describe 4 adult patients with diagnosis of SWS affected by partial intractable epilepsy who underwent presurgical evaluation. We studied electroclinical features (inter-ictal and ictal EEC, semeiology of seizures), and neuroradiological findings. Discussion Physical examination showed mental deficiency of a light degree in 3 patients, mild hemiparesis in 2. visual field deficits in 2, glaucoma and facial nevus in only one. The absence of facial nevus explained an accidental and delayed diagnosis of SWS in 2 cases. The neuroradiological study demonstrated a leptomeningeal vascular malformation in temporal, parietal and occipital regions; it was characterized by different and incomplete findings: hemicranial atrophy, calcifications, leptomeningeal enhancement, abnormal white matter, and enlarged choroid plexus. The only case with all these alterations showed a poorer clinical status, with more epileptic seizures and less success in controlling them medically, and a more severe psychomotor delay. During video-EEG monitoring, seizures arising from temporal lobe and early spreading to a large extratemporal region (involved by the anatomical lesion) were recorded in 3 patients; in one the seizures had a multifocal onset. Ictal events were characterized by variable clinical features according to the involvement of different areas. Conclusions In accordance with the literature, we found a wide clinical polymorphism and neuroradiological variability. Clinical evolution depends on severity of neuroradiological findings. In our cases multifocal seizures and/or early spreading to a large region included in the anatomical lesion excluded surgical therapy.

Original languageEnglish
Pages (from-to)344-345
Number of pages2
JournalItalian Journal of Neurological Sciences
Issue number5
Publication statusPublished - 1999

ASJC Scopus subject areas

  • Neuroscience(all)
  • Clinical Neurology

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    Di Bonaventura, C., Fattouch, J., Izzi, F., Francia, A., Quarato, P. P., Scoppetta, C., Serrao, M., Ricci, S., Giallonardo, A. T., & Manfredl, M. (1999). Sturge-weber syndrome (sws): Electroclinical and neuroradiological features. Italian Journal of Neurological Sciences, 20(5), 344-345.