John Libbey Eurotext

Good outcome in adult-onset Rasmussen's encephalitis syndrome: is recovery possible? Volume 17, issue 2, June 2015


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1 Service of Neurology, Golgi-Cenci Foundation/ASP Golgi-Redaelli, Milan
2 U.O. Neurology, San Carlo Borromeo Hospital, Milan
3 Department of Neuroradiology, IRCCS Istituto Neurologico C. Mondino Foundation, Pavia
4 IRCCS Istituto Neurologico C. Mondino Foundation; Department of Neurological Science, University of Pavia, Pavia, Italy
* Correspondence: Tino Emanuele Poloni Servizio di Neurologia, Service of Neurology, Golgi-Cenci Foundation/ASP Golgi-Redaelli, via San Martino n. 10 - 20081 Abbiategrasso, Milano, Italy

A healthy 29-year-old man suffered from adult-onset epilepsy, characterized by polymorphic progressive seizures resistant to AEDs, leading to unilateral cortical deficits and atrophy of the left hemisphere. The disorder satisfied the clinical, EEG, and imaging criteria for a diagnosis of Rasmussen's encephalitis. During the acute phase of the disease, intrathecal synthesis of specific anti-CMV IgG was identified. This case was characterized by a very mild course and remission of seizures following a treatment with high-dose intravenous polyvalent immunoglobulins containing a high anti-CMV titre. The patient remained symptomless for more than 15 years from clinical onset and more than eight years after the discontinuation of immunological therapy. In agreement with a recent report, this case confirms that adult-onset Rasmussen's encephalitis syndrome may occur with a very mild clinical picture and persistent remission. In this case, the specific index for intrathecal production of anti-CMV antibodies suggested possible CMV involvement, indicating specific immuno-therapy as a treatment choice.