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Accidental finding of Sturge-Weber syndrome presented to the emergency department with persistent seizures: an unusual case report from Somalia
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Introduction:
Sturge-Weber syndrome, also known as encephalotrigeminal angiomatosis, is a rare neurocutaneous disorder characterized by the presence of craniofacial capillary malformations (port-wine stain birthmark) in association with leptomeningeal vascular abnormalities. The syndrome is usually sporadic, but some cases may have a genetic basis.
Case presentation:
Here we report a case of an 18-year-old female who was brought to our Emergency department with a history of seizures and a facial birthmark. Based on the clinical presentation, characteristic facial birthmark, and cranian CT scan findings, the patient was diagnosed with Sturge-Weber syndrome.
Discussion:
This case emphasizes the importance of early recognition of Sturge-Weber syndrome in emergency settings. The patient’s port-wine stain, localized to the distribution of the trigeminal nerve, was a critical clue in guiding the diagnosis. Imaging revealed associated leptomeningeal angiomas, corroborating the clinical suspicion. Such findings underline the necessity for healthcare providers to maintain a high index of suspicion for neurocutaneous disorders in patients presenting with unexplained seizures and specific cutaneous manifestations.
Conclusion:
This case underscores the need for healthcare providers to maintain a high index of suspicion for Sturge-Weber syndrome, particularly in patients presenting with unexplained seizures and distinctive facial cutaneous findings. Early diagnosis and intervention are essential to improve patient outcomes and quality of life. Enhanced awareness and education among medical professionals can lead to timely recognition and management of this rare condition.
Ovid Technologies (Wolters Kluwer Health)
Title: Accidental finding of Sturge-Weber syndrome presented to the emergency department with persistent seizures: an unusual case report from Somalia
Description:
Introduction:
Sturge-Weber syndrome, also known as encephalotrigeminal angiomatosis, is a rare neurocutaneous disorder characterized by the presence of craniofacial capillary malformations (port-wine stain birthmark) in association with leptomeningeal vascular abnormalities.
The syndrome is usually sporadic, but some cases may have a genetic basis.
Case presentation:
Here we report a case of an 18-year-old female who was brought to our Emergency department with a history of seizures and a facial birthmark.
Based on the clinical presentation, characteristic facial birthmark, and cranian CT scan findings, the patient was diagnosed with Sturge-Weber syndrome.
Discussion:
This case emphasizes the importance of early recognition of Sturge-Weber syndrome in emergency settings.
The patient’s port-wine stain, localized to the distribution of the trigeminal nerve, was a critical clue in guiding the diagnosis.
Imaging revealed associated leptomeningeal angiomas, corroborating the clinical suspicion.
Such findings underline the necessity for healthcare providers to maintain a high index of suspicion for neurocutaneous disorders in patients presenting with unexplained seizures and specific cutaneous manifestations.
Conclusion:
This case underscores the need for healthcare providers to maintain a high index of suspicion for Sturge-Weber syndrome, particularly in patients presenting with unexplained seizures and distinctive facial cutaneous findings.
Early diagnosis and intervention are essential to improve patient outcomes and quality of life.
Enhanced awareness and education among medical professionals can lead to timely recognition and management of this rare condition.
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