Abstracts

Rationale: Immune-mediated mechanisms has been postulated for some primary epilepsies, especially in Rasmussen's encephalitis. Inflammatory reactions have been reported (McNamara,1999) in surgical specimens from some patients who had temporal lobectomies for refractory epilepsy.Mantegazzaa et al (2002) reported the presence of anti-GluR3 antibodies against peptides A and B in patients with Rasmussen's(RE)(n=11),an other epilepsies (n=85)and concluded they were specific for epilepsy not RE. We had the opportunity of evaluating two women with adult onset progressive focal seizures and neurlogical deficit where no etiology was found and where a primary immune-mediated focal encephalitis was postulated and treated with steroids and ultimately IVIG. Methods: A 35 years old white woman presented with the first left simple partial sensory-motor seizure that progressed to a generalized tonic-clonic. Extensive medical,infectious, immunological and neurological work up was negative except for an MRI where an incidental, small, temporal encephalocele. 5 FDG PET scan was normal.EEG studies showed severe, persistent focal right temporo-parietal slow wave and spike abnormalities.Her past medical history was unremarkable except for idiopathic primary ovarian failure. Her seizures persited in spite of multiple anti-convulsants and she developed a mild motor deficit in her left foot. A 33 years old healthy woman developed episodes of speech arrest and progressive dysphasia associated with severe progressive EEG changes and MRI evidence of varying cortical abnormalities with T2 non-enhancing hyperintenisities in the left parietal and frontal areas without diffusion changes.Cerebral angiogram was normal.Her extensive work-up was also negative for all infectious, immunological and other possible etiologies. Multiple anti-convulsants with therapeutic levels failed to control her seizures. She failed a steroids course,and her speech deteriorated. Results: Case 1: After failing IV steroid course, IVIG was administered in dose of 0.5 gm/kg/day on 4 consecutive days and repeated in 2 weeks. There was progressive resolution of focal seizures and deficit.The treatment was associated with transient neutropenia and thrombocytopenia thought due to complement activation. Her EEG studies improoved, and her anti-convulsants were reduced. Case 2: IVIG course has been started (0.5gm/kg) after failure of IV steroid therapy and persistent medically refractory seizures and speech deterioration. Conclusions: Two cases of women with insidious, progressive focal encehalitis picture where treated with IVIG. One responded gratifyingly to the administration of IVIG. With the exception of primary ovarian failure, neither woman had any suggestion of prior auto-immunity. The possible role of immune process in some focal epilepsy is reviewed.