Differential long-term evolution after surgery or pharmacotherapy of Rasmussen encephalopathy in adult patients
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Rasmussen encephalitis (RE) is a severe encephalopathy marked by a chronic brain inflammation including microglial nodules, perivascular infiltrates, neuronal loss and gliosis , which typically affects one hemisphere. Although clinical onset is usually in the childhood, some cases begin in the adolescence or young adulthood. Clinically, it is characterized by drug-resistant seizures, often epilepsia partialis continua (EPC) and epileptic status, motor and sensorial impairments, cognitive impairments secondary to progressive brain dysfunction. Magnetic resonance shows progressive unilateral atrophy of the cortex, caudate nucleus and signal alteration of the white matter. Treatment is aimed at controlling the seizures and stopping clinical worsening. To date, the surgical exclusion of the affected hemisphere has been the only therapeutic option to achieve both objectives [2, 3]. Functional hemispherectomy of the affected hemisphere is unanimously recognized as the treatment...
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The authors declare that they have no conflict of interest.
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The authors state that no approval by ethics committee is required for the issue of this letter, and that all applicable Italian laws have been observed.
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