Although adult onset type iii sws is very rare it is important to perform swi and post contrast flair for assessing leptomeningeal angioma in patients with seizure with focal cortical calcification even if they. Skull x ray film may show classic tram line or tram track calcifications.
Ct scan may show brain atrophy calcification and ipsilateral choroid plexus enlargement.
Tram track calcification in sturge weber. Unlike other neurocutaneous disorders phakomatoses sturge weber occurs sporadically i e does not have a hereditary cause. In 1912 weber and volland described the intracranial calcification. Sturge weber syndrome an encephalo trigeminal angiomatosis is a rare.
His condition was improved after treatment with oral levetiracetam 1 000 mg daily. It is caused by a mosaic somatic activating mutation occurring in the gnaq gene. Ipsilateral enlargement of the choroid plexus is also seen 1.
Skull radiographs may show the classic double lined gyriform pattern of calcifications paralleling cerebral convolutions referred to as tram track also called tramline trolley track or. Axial t1 image figure 4 demonstrates volume loss in the right frontal area with thickened diploic space in this region. In our case the atrophy was mild however.
Sturge weber syndrome is a congenital condition characterized by an intracranial vascular anomaly. Sturge weber syndrome there is a right hemisphere volume loss associated with subcortical calcification tram track sign calvarial and regional sinus enlargement may be evident. Cortical atrophy underlying the angioma with gyriform tram track calcification is characteristic imaging feature.
Termed tram track calcification on ct and susceptibility on swi on mri with t2 hypointensity and t1 hypointensity. Radiographic identification of cerebral calcification was first described by dimitri in 1922 2 18 20. The gyral or subcortical calcification is better seen in the ct image figure 3.
Radiological findings will show tram track calcifications on ct bilaterally. In fact schirmer described a male patient with a facial nevus and buphthalmos in 1860 however he did not recognize that it was a neurological condition 20. 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 the face typically in the ophthalmic v1 and maxillary v2 distributions of the trigeminal nerve.
Ipsilateral choroid plexus may be enlarged. Sturge weber syndrome sws. These clinical and neuroimaging findings were compatible with type iii sturge weber syndrome sws.
Calcification is unusual before 2 years of age usually involves high parietal or occipital lobes. The hallmark of sws is a facial cutaneous veno. Sturge weber syndrome sws plain films figure 1 and figure 2 show tram track calcification in the right frontal region.