Situated along the space between the pia mater and the arachnoid membrane often lead to a classical ct appearance of tram track calcifications. Sturge weber syndrome sometimes referred to as encephalotrigeminal angiomatosis is a rare congenital neurological and skin disorder.
Its etiology remains 4 elusive however some authors believe that angiomas arise.
Tram track sturge weber syndrome triad. Sturge weber syndrome which is also known as sturge weber disease leptomeningofacial angiomatosis sturge weber dimitri syndrome is a rare sporadic neurocutaneous disorder and belongs to a group of disorders called phakomatoses. Although classical sws encompasses a triad of clinical manifestations. It should not be confused with other tram track signs elsewhere in the body.
Sturge weber syndrome can be classified into three different types. Sturge weber syndrome or encephalotrigeminal angiomatosis is a phakomatosis characterized by facial port wine stains and pial angiomas. Sturge weber syndrome sws sws also known as encephalotrigeminal angiomatosis or meningiofacial angiomatosis is a rare neurocutaneous syndrome that includes a facial port wine stain and associated leptomeningeal angiomatosis.
Type i dermatoneurological type ii. Tram track sign brain tram track sign in the brain refers to the parallel calcification of the cortex in patients with sturge weber syndrome 1. Sturge weber syndrome sws also called encephalotrigeminal angiomatosis is a neurocutaneous disorder with angiomas that involve the leptomeninges leptomeningeal angiomas las and the skin of.
1 a case of 55 year old man with first ever generalized seizure diagnosed with sturge weber syndrome type iii by characteristic mri findings. The pial angiomatosis may be bilateral in 20 of cases. Tram track lesions angiography aberrant tortuous vessels non filling dural sinuses ct scan tram track appearance.
It is one of the phakomatoses and is often associated with port wine stains of the face glaucoma seizures intellectual disability and ipsilateral leptomeningeal angioma. Sturge weber syndrome is also known as fourth phacomatosis mother spot. It may or may not be associated with ipsilateral glaucoma 1.
Triad and types broadly 3 types. It is part of a wide spectrum of possible phenotypes included in the craniofacial arteriovenous metameric syndrome cams. Sturge weber syndrome sws also known as encephalotrigeminal angiomatosis is a rare neurocutaneous syndrome characterized by a facial capillary vascular malformation a port wine stain pws with ipsilateral leptomeningeal angiomas lma.
Sturge weber syndrome 1. Review of literature with case illustration romanian neurosurgery vol. Type 1 includes facial and leptomeningeal angiomas as well as the possibility of glaucoma or choroidal.
It is generally considered nonhereditary.