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Cognitive dysfunction and brain atrophy in Susac syndrome.
- Source :
-
Journal of neurology [J Neurol] 2020 Apr; Vol. 267 (4), pp. 994-1003. Date of Electronic Publication: 2019 Dec 11. - Publication Year :
- 2020
-
Abstract
- Background: Susac syndrome is a very rare cerebral small vessel disease, which can leave patients with cognitive impairment. We aimed at evaluating processing speed slowing, executive dysfunction and apathy and their relationships with whole brain and callosal atrophy.<br />Methods: Patients with Susac syndrome included in a prospective observational cohort study were evaluated, while clinically steady-state, with standardized brain MRI and a neuropsychological battery specifically designed to capture minimal cognitive alterations in non-disabled young patients. Brain volume and corpus callosum area were measured using 3D-T1 sequences, repeatedly overtime. Relationships between neuropsychological data and brain volumetric measures obtained the same day were tested with linear regression while controlling for sex, age, level of education, scores of depression and of apathy.<br />Results: Nineteen patients aged 37.5 ± 10.5 years were included. Mean follow-up time was 2.6 ± 1.3 years (5.8 ± 2.2 evaluations). While Montreal Cognitive Assessment scores were 25.1 ± 3.6, processing speed slowing was obvious (Trail Making Test version A: 43.1 ± 16.2 s; version B: 95.5 ± 67.9 s; reaction time: 314.6 ± 79.6 ms). Brain and corpus callosum atrophy was striking. No relationship was found between cognitive performances and brain volume or corpus callosum area.<br />Conclusion: Patients with Susac syndrome show largely preserved global cognitive functions but important processing speed alterations. Although brain and corpus callosum area atrophy is prominent and evolving, we did not find any relationship with cognitive alterations, questioning the mechanisms underlying cognitive alterations in these patients.<br />Trial Registration: Clinical Trial Registration-URL: https://www.clinicaltrials.gov Unique Identifier: NCT01481662.
- Subjects :
- Adult
Atrophy pathology
Brain diagnostic imaging
Cognitive Dysfunction etiology
Corpus Callosum diagnostic imaging
Corpus Callosum pathology
Female
Follow-Up Studies
Humans
Magnetic Resonance Imaging
Male
Middle Aged
Neuropsychological Tests
Prospective Studies
Susac Syndrome complications
Susac Syndrome diagnostic imaging
Apathy physiology
Brain pathology
Cognitive Dysfunction physiopathology
Executive Function physiology
Susac Syndrome pathology
Susac Syndrome physiopathology
Subjects
Details
- Language :
- English
- ISSN :
- 1432-1459
- Volume :
- 267
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Journal of neurology
- Publication Type :
- Academic Journal
- Accession number :
- 31828475
- Full Text :
- https://doi.org/10.1007/s00415-019-09664-8