Abstracts

Rationale: We present a case of Rasmussen's encehephalitis that illustrates the developmental, medical, cognitive and psychosocial aspects of this devastating progressive condition over time. The patient is a 25 year-old right-handed woman who developed right hemisphere complex partial seizures at age 8. Methods: Prior to surgical intervention, the patient underwent EEG telemetry, neuropsychological evaluation and Wada test. She underwent a palliative right frontal lobectomy at age 9, sparing the temporal lobe and motor cortex as left upper extremity functions at the time were normal. Since then her seizures have never been completely controlled despite multiple medications. Followup neuropsychological evaluations were conducted at age 10, 13, 18 and 25, and she has had periodic imaging and EEG telemetries. Further neurosurgery (functional hemispherectomy) has been offered on several occasions, but the patient and family have not to date elected this intervention. Results: Although the patient has grown cognitively and has acquired some new skills, her intellectual scores relative to peers have declined from Verbal IQ at the 94th percentile and Performance IQ at the 70th percentile at age 8, to VIQ at the 10th percentile and PIQ at the 4th percentile at age 25. Visual memory scores deteriorated before verbal memory scores, now both in the borderline range. Parents complained of her loss of musical ability immediately after surgery. Left-hand motor and sensory skills deteriorated significantly by age 18 and she now has a contracted left hand; the current MRI scan shows diffuse volume less throughout the remaining right hemisphere including the hippocampal formation. Problems with social inappropriateness, nonexistant prior to seizure onset, became apparent as the patient entered her teenage years. She graduated from high school but is not working and still lives with her parents. Issues with psychogenic nonepileptic spells in addition to her epileptic seizures have been documented.Conclusions: Medical maintenance and counseling efforts with the patient and her family continue. The importance of patient and family acceptance of offered treatment is underscored, and the difficult decision-making process in neurosurgical treatment of Rasmussen's encephalitis is discussed.