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Sturge-Weber syndrome

Case contributed by Farhad Farzam
Diagnosis almost certain

Presentation

Seizure and right sided body weakness.

Patient Data

Age: 10 years
Gender: Male
mri
  • evidence of leptomeningeal hyperintensity in FLAIR images which returns low signal in T2 WI in the left parieto-occipital lobes
  • significant leptomeningeal enhancement and pial angioma in the aforementioned lobes
  • mild atrophic changes in both cerebral hemispheres at the supratentorial level

Case Discussion

Sturge-Weber syndrome is a rare congenital vascular disorder. It is characterized by facial capillary malformation, leptomeningeal angiomatosis and congenital glaucoma. The neurological features include seizures, focal neurological deficits and intellectual disability. CT and MRI demonstrate the characteristics of brain parenchymal changes which help in early diagnosis of the entity. 

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