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From the Divisions of Child Neurology (Drs. Granata, Ragona, and Freri), Experimental Neurophysiology and Neuroanatomy (Dr. Spreafico), Neuroradiology (Dr. Chiapparini), Neuromuscular Diseases (Dr. Bernasconi), and Clinical Neurophysiology (Dr. Casazza), Istituto Nazionale Neurologico "C. Besta," Milan; Division of Neuropediatrics (Dr. Gobbi), Ospedale Maggiore Cà Pizzardi, Bologna; Division of Neurology (Drs. Vigevano and Fusco), Ospedale Pediatrico "Bambino Gesù," Rome; Division of Neuropediatrics (Dr. Capovilla), Ospedale "C. Poma," Mantova; Division of Neuropediatrics (Dr. Giordano), Ospedali Civili, Brescia; Division of Neuropediatrics (Dr. Bertani), Arcispedale S. Maria Nuova, Reggio Emilia; and Division of Neuropediatrics (Dr. Dalla Bernardina), Policlinico "Borgo Roma," Verona, Italy. All authors form part of the study group on RF of the Italian League Against Epilepsy.
Address correspondence and reprint requests to Dr. Tiziana Granata, Divisione di Neuropsichiatria Infantile, Istituto Nazionale Neurologico "C. Besta," Via Celoria 11, 20133 Milan, Italy; e-mail: granata{at}istituto-besta.it
Objective: To identify early manifestations of Rasmussen encephalitis (RE) that can prompt early and reasonably secure diagnosis, allowing medical or surgical therapies at an early stage when they may be more effective in slowing the disease.
Methods: The authors studied 12 patients with clinical and neuropathologic diagnosis of RE, followed from disease onset, assessing clinical history, imaging, and EEG and focusing on early characteristics. Anti-GluR3 antibody assays were also considered in 11 patients.
Results: By 4 months from first symptoms, all cases had 1) refractory focal seizures with a predominant motor component, 2) slow focal activity on EEG contralateral to the motor manifestations, and 3) focal contralateral white matter hyperintensity with insular cortical atrophy on neuroimaging. Less constant or later findings were epilepsia partialis continua, oligoclonal bands, and serum anti-GluR3 antibodies.
Conclusions: The association of partial seizures with focal EEG and neuroimaging changes allows a tentative diagnosis of RE 4 to 6 months after first symptoms.
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