Cognitive outcomes in Susac syndrome: A 2-year neuropsychological follow-up study.

Autor: Van Vrekhem T; Department of Neurology, Ghent University Hospital, Ghent, Belgium., Miatton M; Department of Neurology, Ghent University Hospital, Ghent, Belgium., Hemelsoet D; Department of Neurology, Ghent University Hospital, Ghent, Belgium., Van Hijfte L; Department of Neurology, Ghent University Hospital, Ghent, Belgium., Dekeyser C; Department of Neurology, Ghent University Hospital, Ghent, Belgium., De Zaeytijd J; Department of Ophthalmology, Ghent University Hospital, Ghent, Belgium., Van Driessche V; Department of Radiology, Ghent University Hospital, Ghent, Belgium., Van Hoecke H; Department of Otorhinolaryngology, Ghent University Hospital, Ghent, Belgium., Maes L; Department of Otorhinolaryngology, Ghent University Hospital, Ghent, Belgium.; Department of Rehabilitation Sciences, Ghent University, Ghent, Belgium., Laureys G; Department of Neurology, Ghent University Hospital, Ghent, Belgium.
Jazyk: angličtina
Zdroj: European journal of neurology [Eur J Neurol] 2024 Apr; Vol. 31 (4), pp. e16186. Date of Electronic Publication: 2024 Feb 02.
DOI: 10.1111/ene.16186
Abstrakt: Background and Purpose: Susac syndrome (SuS) is a rare, autoimmune, neurological disease characterized by a clinical triad of branch retinal artery occlusion, sensorineural hearing loss and encephalopathy. Neuropsychological functioning in SuS is little researched and the prevalence, nature, and evolution over time of cognitive deficits in SuS remain unclear. This study aimed to better understand the long-term neuropsychological outcomes of patients with SuS.
Methods: Thirteen patients with SuS (mean [SD] age 39.5 [11.1] years) were enrolled at the Ghent University Hospital by their treating neurologist. The cognitive functioning and emotional well-being of each patient was evaluated by means of a thorough neuropsychological test battery at baseline and after 2 years. Follow-up testing after 2 years was performed in 11 patients (mean [SD] age 42.2 [11.5] years).
Results: Patients showed normal neuropsychological test results at a group level, both at baseline and follow-up testing. Significant improvements over time were found for information processing speed, verbal recognition, and semantic and phonological fluency. Individual test results showed interindividual variability at baseline, with most impairments being in attention, executive functioning and language, which improved after a 2-year period. In addition, patients reported significantly lower mental and physical well-being, both at baseline and follow-up testing.
Conclusions: Our results suggest that neuropsychological dysfunction in SuS is limited at a group level and improves over time. Nonetheless, individual test results reveal interindividual variability, making cognitive screening essential. Furthermore, a high psycho-emotional burden of the disease was reported, for which screening and follow-up are necessary.
(© 2023 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)
Databáze: MEDLINE
Externí odkaz: