Classic Struge Weber case

A 47-year-old male patient presented to the OPD with complaints of epilepsy since childhood. The seizure is of generalized tonic and clonic type. On physical examination, there is presence of the portwine stain in the right side of the face which has been there since childhood. Other examinations are within normal limits. He then got the CT scan of the head for the same.

Calcified gyrus overlying the right temporal and occipital lobe is seen in the palin CT head. Calcification is also seen in the contalateral occipital lobe. There is presence of atrophy of the right temporal lobe suggested by relative thinning of the Grey mater underlying the calcifications and prominence of the extraxial CSF space and widened sulci between the gyrus compared to the contrallateral temporal lobe. Contrast-enhanced images demonstate the abnormal increment in the vascularity within the affected right temporal lobe towards the leptomeningeal lined surface suggestive of pial angiomas. Also a dilated and tortuous cortical vein draining the right hemisphere is seen. Close inspection of the temporalis fossa on the right shows the atrophied right temporalis muscle and fatty infiltration of the masticator space.

Struge Weber syndrome belongs to one of the many neurocutaneous syndromes also know as phakomatosis. There can be occlusion or persistence of the fetal circulation which results in cortical hypoxia progressing to anoxia[1]. Classical findings in these patients involve Port wine stain, ipsilateral side pial and subpial angiomas and glaucoma. These may present with seizure disorder, developmental delay and hemiparesis of the body ipsilateral to the Port wine stain[2]. Classical findings in the skull X-ray is tram track-like calcification of the opposing gyri[3]. CT images shows dense gyriform pattern of calcification adjacent to the atrophied cortex. The calcification are in the cerebral cortex and not in the subpial angioma. The frequency and duration of the  epilepsy is related to the degree of calcification of the cerebral hemispheres. Bone window of the CT scan may depict calvarial thickening and over-pneumatization of the ipsilateral frontal sinus as a consequence of long-standing atrophic changes. Volume loss due to cortical atrophy of the involved lobe may be seen. MRI is better in subtle changes depiction compared to the CT scan. On post-contrast images, enhancement of the pial angiomas may be seen but may be obscured by heavy amount of calcification. In these cases however enlarged choroid plexus may hyperenhance. MRI imaging may demonstrate cortical or subcortical T2 hypointensity, blooming of calcification in T2* GRE or SWI imaging,  FLAIR image may demonstrate cortical serpentine hyper signal intensity called “Ivy sign”. Angiomas may enhance following the contrast administration and enlargement of the choroid plexus of the affected site is also seen. CT is better in terms of calcification detection and depiction whilst MRI is better for detection of the subtle changes or angiomas. DSA if done will demonstrate absent superficial cortical veins with dilated deep medullary and subependymal veins. Apart from brain findings the patient may even have hypertrophied limbs, extensive capillary cutaneous, vascular and lymphatic malformations  [4].