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.
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http://dx.doi.org/10.1684/epd.2015.0743 | DOI Listing |
Epileptic Disord
December 2024
Center for Pediatric Neurology and Neurorehabilitation, Epilepsy Center for Children and Adolescents, Schoen Clinic Vogtareuth, Vogtareuth, Germany.
Objective: To investigate the frequency of epileptiform discharges associated with self-limited focal epilepsy (EDSelFEC) in children who have undergone a hemispherotomy and to evaluate whether patients with coexistence of EDSelFEC and structural hemispheric epilepsies differ from patients without coexistence of EDSelFEC and whether there are differences between the two groups with regard to preoperative management and postoperative outcome.
Methods: Data on 131 children who underwent a hemispherotomy between January 1999 and January 2015 were retrieved from the Epilepsy center's epilepsy surgery database. Children with EDSelFEC were compared with children without EDSelFEC with respect to epileptogenic hemispheric pathology, family history, age at epilepsy onset, timing of surgery, lesion laterality, preoperative cognitive function, response to sodium channel blocker antiepileptic medication, and surgical outcome.
Medicina (Kaunas)
November 2024
Neurology Department, Cooper University Hospital, Camden, NJ 08103, USA.
Rasmussen encephalitis (RE) is a rare and progressive form of chronic encephalitis that typically affects one hemisphere of the brain and primarily occurs in pediatric individuals. The current study aims to narratively review the literature about RE, including historical information, pathophysiology, and management of this condition. RE often occurs in individuals with normal development, and it is estimated that only a few new cases are identified each year in epilepsy centers.
View Article and Find Full Text PDFSeizure
December 2024
Ankara University, Faculty of Medicine, Department of Neurology, Ibn-i Sina Hospital, 06100, Ankara, Turkey.
BMC Neurol
October 2024
Department of Obstetrics and Prenatal Medicine, University Hospital Bonn, Venusberg-Campus 1, 53127, Bonn, Germany.
Seizure
November 2024
Department of Epileptology, Krankenhaus Mara, Bethel Epilepsy Center, Medical School OWL, Bielefeld University, Maraweg 21, 33617, Bielefeld, Germany. Electronic address:
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