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Case Report| Volume 16, ISSUE 12, P1677-1681, December 2009

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Late onset Rasmussen’s encephalitis with triple pathology

      Abstract

      Rasmussen’s encephalitis is a devastating illness characterized by intractable focal seizures due to chronic localised encephalitis. We report on a rare variant of delayed onset Rasmussen’s encephalitis with triple pathology. A 27-year-old male, who was initially diagnosed with seizures when he was 16 years old, presented with focal seizures that became refractory to multiple anticonvulsants. Multiple investigations, including subdural electrode monitoring, revealed foci of onset in the right frontotemporal region. The patient underwent right front-temporal lobectomy. Post-operatively, the seizures became more severe and he developed new epilepsia partialis continua. Treatment with intravenous immunoglobulin was unsuccessful. He subsequently underwent a right hemispherectomy that rendered him seizure free. The three pathologies identified were old ischemic changes, type II cortical dysplasia and stage II Rasmussen’s encephalitis.

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      References

        • Bien C.
        • Granata T.
        • Antozzi C.
        • et al.
        Pathogenesis, diagnosis and treatment of Rasmussen’s encephalitis. A European consensus statement.
        Brain. 2005; 128: 454-471
        • Bien C.G.
        • Bauer J.
        • Deckwerth T.L.
        • et al.
        Destruction of neurons by cytotoxic T cells: a new pathogenic mechanism in Rasmussen’s encephalitis.
        Ann Neurol. 2002; 51: 311-318
        • Andrews P.
        • Dichter M.A.
        • Berkovic S.F.
        • et al.
        Plasmapheresis in Rasmussen’s encephalitis.
        Neurology. 1996; 46: 242-246
        • Hart Y.
        • Andermann F.
        • Robitaille M.D.
        • et al.
        Double pathology in Rasmussen’s syndrome.
        Neurology. 1998; 50: 731-735
        • Bien C.
        • Widman G.
        • Urbach H.
        • et al.
        The natural history of Rasmussen’s encephalitis.
        Brain. 2002; 125: 1751-1759
        • Firlik K.
        • Adelson P.D.
        • Hamilton R.L.
        Coexistance of a ganglioma and Rasmussen’s encephalitis.
        Pediatr Neurosurg. 1999; 30: 278-282
        • Palmer C.
        • Geyer J.D.
        • Keating J.M.
        • et al.
        Rasmussen’s encephalitis with concomitant cortical dysplasia: The role of GluR3.
        Epilepsia. 1999; 40: 242-247
        • Thom M.
        • Moran N.F.
        • Plant G.T.
        • et al.
        Cortical dysplasia with angiodysgenesis and chronic inflammation in multifocal partial epilepsy.
        Neurology. 1999; 52: 654-657
        • Yacubian D.
        • Rosemberg S.
        • Marie S.K.N.
        • et al.
        Double pathology in Rasmussen’s Encephalitis: Etiologic considerations.
        Epilepsia. 1996; 37: 495-500
        • Lascelles K.
        • Dean A.F.
        • Robinson R.O.
        Rasmussen’s encephalitis followed by lupus erythematosus.
        Dev Med Child Neurol. 2002; 44: 572-574
        • Hart Y.M.
        • Cortez M.
        • Andermann F.
        • et al.
        Medical treatment of Rasmussen’s syndrome (chronic encephalitis and epilepsy): Effect of high-dose steroids or immunoglobulins in 19 patients.
        Neurology. 1994; 44: 1030-1036
        • Wise M.S.
        • Rutledge S.L.
        • Kuzniecky R.I.
        Rasmussen syndrome and long-term response to gamma globulin.
        Pediatr Neurol. 1996; 14: 149-152
        • Leach J.P.
        • Chadwick D.W.
        • Miles J.B.
        • et al.
        Improvement in adult-onset Rasmussen’s encephalitis with long-term immunomodulatory therapy.
        Neurology. 1999; 52: 738-742
        • Villani F.
        • Spreafico R.
        • Farina L.
        • et al.
        Positive response to immunomodulatory therapy in an adult patient with Rasmussen’s encephalitis.
        Neurology. 2001; 56: 248-250
        • Granata T.
        • Fusco L.
        • Gobbi G.
        • et al.
        Experience with immunomodulatory treatments in Rasmussen’s encephalitis.
        Neurology. 2003; 61: 1807-1810