Presentation
Rasmussen encephalitis (>10 year history) with severe epilepsy. Interval disease?
Patient Data
Focal encephalomalacia within the left temporal lobe involves the superior and middle temporal gyri. There is surrounding FLAIR hyperintensity at this site, and these appearances are stable. A new region of FLAIR hyperintensity involving both the grey and subcortical white matter within the left inferior parietal lobule, involving both the supramarginal gyrus and angular gyrus is noted. This has progressed compared with the prior study, with only subtle abnormality in this area evident in retrospect. There is no mass effect at this location. No restricted diffusion or abnormal enhancement at this site or elsewhere. Progressive mild dilatation involves the left trigone. Subtle atrophy of the left hippocampus and equivocal T2 hyperintensity is unchanged. Nodular heterotopia adjacent the right frontal horn is again noted.
Patient age 25 years old. Mild left hippocampal atrophy otherwise stable intracranial appearance. No other interval parenchymal volume loss, caudate/basal ganglia change.
Patient age 14 years old. Left superior temporal gyrus resection, minimal surrounding gliosis. Left mesial temporal lobe was relatively spared with symmetrical hippocampi, ventricles and caudate nuclei.
Case Discussion
Rasmussen encephalitis is a chronic idiopathic CNS inflammatory disease predominantly diagnosed in children, usually affecting a single cerebral hemisphere and causing severe/refractory epilepsy. On most recent follow-up of this young man with a recorded history of RE diagnosed as a child at pediatric institution, imaging demonstrated progressive left hemispheric abnormality in the inferior parietal lobule likely representing a site of new chronic encephalitis. Over the 10+ years of scanning, it is easier to appreciate progressive disease course with mild dilatation of the left trigone and stable mild left hippocampal atrophy.
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