Case 20
History:
54 year old male with history of seizures, complains of nose stuffiness
Findings:
Images 1 and 2 show tram track calcification, skull thickening and prominent of the paranasal sinuses. Image 3, T1 post gad shows left sided pial enhancement. Image 4 shows curvilinear posterior contrast enhancement of ocular choroidal angiomas.
Diagnosis:
Sturge-Weber syndrome, (encephalotrigeminal angiomatosis)
Discussion:
A phakomatosis, Sturge-Weber syndrome, is a rare congenital neurological and cutenous disorder. It is often associated with facial port wine stains, glaucoma, seizures, mental retardation and ipsilateral leptomeningeal angioma. It is believed to result from a failure of development of the normal cortical venous drainage resulting in the formation of a pial vascular malformation that develops ipsilateral to the port wine nevus. Normally, only one side of the head is affected.
On CT, curvilinear calcifications in a gyral pattern are often seen, primarily in the occipital and posterior parietal lobes ipsilateral to the facial angioma. Progressive cortical atrophy, ipsilateral calvarial thickening, and enlargement of the paranasal sinuses and mastoid may also be seen. MRI show the extent of the angiomatous malformation. The ipsilateral choroid plexus is often hypertrophied. The involved parenchyma may show white matter increased T2 signal.
References:
- Griffiths PD & al. Choroid plexus size in young children with sturdge weber syndrome, Am J neurorad 17:175-180. 1996.
