Article Text

STURGE-WEBER SYNDROME: ONSET PARTICULARITY
  1. C Cazan1,
  2. A M Rosenberg1,
  3. B Solomon1
  1. 1Pediatric Clinic Of Sibiu, Sibiu, Romania

Abstract

Introduction Cerebro-trigeminal angiomatosis, Sturge-Weber syndrome (SWS) is a neurocutanoeous disease which associates leptomeningeal and facial angiomatosis, located in the distribution of the ophthalmic and maxillary division of the trigeminal nerve. Neurological features include refractory focal seizures, transient focal disability, headache and deterioration of the neuro-mental status. The residual embryologic vascular plexus forms leptomeningela, facial and ocular hemangiomas as etiological defects in SWS; there are reports about somatic mutation, somatic mosaic, 4q inversion and trisomy 10 being responsible for the structural and functional regulation of the vascular plexus. Fibronectin, which is known to intervene in angiogenesis, is reduced, and the decreased level of endorphin I leads to vasoconstriction of the cortical vascular plexus.

Case report The authors present a SWS case whose neurological symptoms, contralateral partial seizures with secondary generalization, appear in febrile context in a 2 year old child with normal neurological development. The acute febrile illness evolves like a viral meningitis.

Conclusion In Sturge-Weber syndrome, the neurological monitoring and control therapy of the critical symptoms are major objectives.

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