Sturge Weber Syndrome Tram Track Calcification

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On ct imaging gyral subcortical calcification is seen with a tram track appearance unilateral cerebral atrophy can also be appreciated. Type 1 includes facial and leptomeningeal angiomas as well as the possibility of glaucoma or choroidal.

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Sturge weber syndrome tram track calcification. However different variants have been described. To determine the relationship between calcified cortex and perfusion status of white matter and seizure severity in patients with sturge weber syndrome sws a sporadic neurocutaneous disorder characterized by a leptomeningeal angioma progressive brain ischemia and a high incidence of seizures using susceptibility weighted imaging swi and dynamic susceptibility contrast enhanced perfusion weighted imaging dsc pwi. Four children affected by sturge weber syndrome and demonstrating bilateral intracranial calcification are described bringing up to 21 the number of similar reported cases.

1 a case of 55 year old man with first ever generalized seizure diagnosed with sturge weber syndrome type iii by characteristic mri findings. 5 this sign can be observed on plain radiograph noncontrast ct ncct scans fig 5a. On mr imaging t1 weighted imaging with contrast shows leptomeningeal enhancement this may not be seen in older patients.

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. It should not be confused with other tram track signs elsewhere in the body. We report one such variant in a 14 months old child with bilateral facial nevus and bilateral curvilinear calcification.

Sturge weber syndrome sometimes referred to as encephalotrigeminal angiomatosis is a rare congenital neurological and skin disorder. Sturge weber syndrome is characterized by unilateral facial nevus with a tram track appearance on ct scan. The tram track sign is caused by cortical calcifications in sturge weber syndrome.

Sturge weber syndrome can be classified into three different types. Imaging characteristics of sturge weber syndrome. The frequency of bilateral hemisphere involvement in this syndrome is not known but it might be as high as 15.

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.

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