Abstracts

Rationale: Case description of a 3 year-old female with intractable complex partial seizures, progressive hemiparesis and pathology suggestive of a Rasmussen's syndrome variant to illustrate the possible autoimmune basis of intractable focal seizure disordersMethods: Case reportResults: A 3 year-old white female with normal birth history had first seizure at 3 week-old with acute onset of recurrent “right” hand twitching every 30 min for 2 days. Initial MRI showed high signal T2 lesion in right central head region involving high convexity of motor area. Initial EEG showed sharp transient in right central head region. A repeat EEG one month later was normal. Patient was seizure-free for 2 years. Patient was seen again at 18month-old for mild left hemiparesis. MRI showed “Old cortical infarct” involving primary motor cortex on the right, with associated atrophy and wallerian degeneration. Patient had recurrent complex partial seizures since 27 month-old. Seizures were characterized as left hand twitching and sometimes involved left upper and lower extremities. Patient had video-EEG monitor at 3 year-old due to Increasing seizures and progressive left hemiparesis. Video-EEG showed frequent ictal and interictal high amplitude spike and wave discharges localized in right central head region. Repeated MRI at 3 year-old demonstrated evidence of high signal lesion in right motor cortex with atrophy and Wallerian degeneration and delayed myelination in the subcortical U fibers and periventricular white matter. Epilepsy surgery with right cortical resection was performed 3 month ago and the pathology showed findings consistent with chronic meningoencephalitis, characterized by diffuse leptomeningeal (not just superficial), and variable but widespread intraparenchymal, CD3-immunoreactive (and CD20-immunonegative) T-lymphocytes, as well as Kp1-immunoreactive monocyte/macrophages. The intraparenchymal lymphocytes are perivascular and/or vasocentric and often associated with microglial activation, which is most striking in subpial cortex, but also forms frank microglial nodules (CD45-immunoperoxidase). Cortex is also irregularly astrogliotic. In some more discrete gliotic microfoci, in both gray and white matter, there is more overt, albeit incomplete, asociated organized (old) parenchymal loss (necrosis). The most prominent such foci showed signs of ongoing inflammatory process. These finding is suggestive of Rasmussen’s encephalitis. Toxoplasma and CMV are negative. Patient was seizure-free following epilepsy surgery. In addition, her left hemiparesis and speech delay were much improved after the surgery. Conclusions: This is an unusual case with focal pathological changes suggestive of Rasmussen’ syndrome presenting with progressive complex partial seizures and progressive hemiparesis. Focal resection surgery in involved motor area has not only controlled seizures, but also improved her motor function.