Leading an Independent Life: Long-Term Cognitive Outcome in Adult and Adolescent Patients with NORSE/FIRES
Abstract number :
1.38
Submission category :
11. Behavior/Neuropsychology/Language / 11A. Adult
Year :
2019
Submission ID :
2421373
Source :
www.aesnet.org
Presentation date :
12/7/2019 6:00:00 PM
Published date :
Nov 25, 2019, 12:14 PM
Authors :
Julia Taube, University Hospital Bonn; Robert D. Nass, University Hospital Bonn; Christian E. Elger, Beta Klinik Bonn; Rainer Surges, University Hospital Bonn; Christoph Helmstaedter, University Hospital Bonn
Rationale: Febrile infection-related epilepsy syndrome (FIRES) is a rare subtype of new onset refractory status epilepticus (NORSE) with significant mortality. Survivors frequently develop a chronic epilepsy with associated cognitive impairments and behavioral problems. The long-term functional outcome has been rarely addressed in previous research even though it is expected to highly influence the patients' quality of life and their ability to live an independent life. Methods: In a retrospective study, patients with FIRES, seen between 2005 and 2019, were reviewed and contacted for follow-up. Long-term outcome parameters include the global assessment of severity of epilepsy (GASE), International Classification of Functioning based scores of independence for neurologic and geriatric rehabilitation (SINGER) and neuropsychological test results obtained 3 to 5 years after onset. Results: We identified 9 patients who fulfilled the diagnostic criteria with age at onset between 15 and 51 years. One patient experienced non convulsive, prolonged refractory status epilepticus (SE), 3 persistent refractory SE and 6 persistent super refractory SE. At follow-up the SINGER scale indicated mild cognitive impairments in 3 and moderate cognitive impairments in 4 cases. Social or behavioral problems were present in 3 patients. Neuropsychological assessment (available in 6 patients) showed impaired to severely impaired attention and executive functions in 3 patients. Verbal memory was impaired in 4 cases. 5 patients showed impairments in visual memory. One patient was globally impaired and lacked orientation regarding location and time. Conclusions: The long-term follow-up of patients with NORSE/ FIRES indicates a partial loss of independence in daily functioning due to persisting seizures, cognitive impairments and behavioral problems. As the condition is very rare, we propose collaborative projects applying the same outcome measures to investigate factors for a favorable/unfavorable outcome in survivors of NORSE/FIRES. Funding: No funding
Behavior/Neuropsychology/Language