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  • i. One of the phakomatoses which include Sturge-Weber, Tuberous sclerosis, Lhermitte-Duclos and neurofibromatosis
  • ii. Consist of cutaneous vascular nevus in the territory supplied by the sensory branches of CN 5; associated with choroidal ocular angioma, meningeal venous angiomatosis; does not typically present with subarachnoid hemorrhage; abnormality on chromosome 3; Remember: VHL and Sturge-Weber are both on chromosome 3
  • iii. Patients often have a hemisensory deficit contralateral to the facial nevus
  • iv. Intracranial calcification is present and can be detected on CT scan even in neonates
  • v. MRI shows subarachnoid enhancement (from meningeal angiomatosis) and cortical atrophy; brain and facial lesions are usually ipsilateral but can be bilateral
    • 1. plain films show tramline calcification which outlines the convolutions of the parieto-occipital cortex (calcification is NOT in the vessels)
  • vi. Seizures are very common opposite of the facial nevus
  • vii. 1/3 of patients develop glaucoma
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