Abstract
ABSTRACTRasmussen encephalitis is a rare disease consisting of a chronic, slowly progressive inflammatory reaction of brain tissues. The objective of this study was to describe the case of an 18-year-old female patient, studying on the fourth grade of elementary school, and right-handed, who underwent left hemispherectomy in a neurological rehabilitation program. Her seizures began at 10 years of age and were unresponsive to drug treatment, with functional repercussions. She underwent hemispherectomy in March 2019, with 7 years of ongoing disease, and was admitted for the rehabilitation program with a multidisciplinary team in June 2020. The quality-of-life questionnaire (WHOQOL-BREF) was applied as a comparison measure before and after the rehabilitation program, along with measures of participation on the program activities. Despite the patient’s short time in a rehabilitation program, data show the importance of an interdisciplinary intervention through the establishment of specific and contextualized objectives in chronic patients.
Highlights
Rasmussen encephalitis (RE) is a rare disease consisting of a chronic, slowly progressive hemispheric unilateral inflammation of the cerebral cortex, probably driven mostly by a T-cell response to one or more antigenic epitopes.[1,2,3] It is characterized by difficult-to-treat focal epileptic seizures, progressive hemiparesis, and intellectual disability.[4]
As there are no studies in the literature addressing the therapeutic effects of interdisciplinary rehabilitation in this chronic neurological condition, the objective of this study was to describe the case of a patient in an interdisciplinary neurological rehabilitation program, who underwent left hemispherectomy, focusing on engaging and stimulating individuals/increasing personal satisfaction and social participation
The rehabilitation program was started on June 30, 2020, and the patient was accompanied by her mother (3 months)
Summary
Rasmussen encephalitis (RE) is a rare disease consisting of a chronic, slowly progressive hemispheric unilateral inflammation of the cerebral cortex, probably driven mostly by a T-cell response to one or more antigenic epitopes.[1,2,3] It is characterized by difficult-to-treat focal epileptic seizures, progressive hemiparesis, and intellectual disability.[4] RE patients, despite their normal development, occasionally deteriorate to a condition of mild to severe mental retardation, with persistent encephalitis and seizures.[5]. Polypharmacy with antiseizure medication is frequently used once seizures may be intractable at onset or become so over time. Since epilepsy is driven by ongoing inflammation, either immunosuppressive or immunomodulatory treatment has been used.[6] Hemispherectomy, the only established method to cure seizures, with success rates of 70–80%, is usually considered for patients whose brain injury produced hemiplegia or severe hemiparesis, drug-resistant seizures, and homonymous hemianopsia. Timing of surgery is often guided by the epilepsy severity, especially if the neurological decline is associated with the appearance of contralateral, independent interictal epileptic discharges.[2]
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