Abstract

IntroductionSturge-Weber syndrome sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and skin disorder.Case presentationThis is case report of a 18-year-old mentally disabled boy, with long-standing seizures, with a port-wine nevi on the left side of the face along the distribution of trigeminal nerve. Interictal encephalogram showed bilateral slow activity, pronounced in the left hemisphere, with epileptogenic activity in the left temporo-parietal region. Skull radiograph, computerized tomography and magnetic resonance imaging showed intracranial calcifications and atrophy of the left brain hemisphere.ConclusionProfessional counseling and support in addition to drug treatment can provide help to patients and their family to overcome their problems and improve the treatment outcome.

Highlights

  • Sturge-Weber syndrome sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and skin disorder.Case presentation: This is case report of a 18-year-old mentally disabled boy, with long-standing seizures, with a port-wine nevi on the left side of the face along the distribution of trigeminal nerve

  • The Sturge-Weber syndrome is a neurocutaneus congenital but not an inherited disease and it occurs sporadically. It is a disorder of vasculature which belongs to the group of phacomatoses characterized by nevus flammeus and angiomas of the meninges [1]

  • It is a rare disease characterized by a birth mark called port wine nevi, associated with abnormalities of the brain caused by abnormal blood vessels that occur on the cerebral cortex [2,3]

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Summary

Introduction

The Sturge-Weber syndrome is a neurocutaneus congenital but not an inherited disease and it occurs sporadically. It is a disorder of vasculature which belongs to the group of phacomatoses characterized by nevus flammeus and angiomas of the meninges [1] It is a rare disease characterized by a birth mark called port wine nevi, associated with abnormalities of the brain caused by abnormal blood vessels (angiomas) that occur on the cerebral cortex [2,3]. Cases Journal 2009, 2:9394 http://www.casesjournal.com/content/2/1/9394 zures (right-sided tonic clonic seizures with facial twitch) that lasted 2-3 minutes with a frequency of 2-3 times per day While he was experiencing these frequent seizures (for years) the only treatment he received was some cold wet towels placed on his forehead.

Discussion
Conclusion
Castilo M
Findings
Comi AM
15. Kugler RM

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