Atypical clinical and novel radiological findings in Susac syndrome: Experience from a large monocentric cohort

Dekeyser, Cathérine; Vanhoorne, Alexander; Hemelsoet, Dimitri; Hijfte, Liesbeth Van; Zaeytijd, Julie De; Driessche, Veroniek Van; Hoecke, Helen Van; Miatton, Marijke; Vrekhem, Tineke Van; Maes, Leen; Lochard, Guy

doi:10.1016/j.jneuroim.2023.578032

Cited by 7 publications

(7 citation statements)

References 24 publications

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“…All patient characteristics with initial MRI findings, cumulative treatment, disease duration at time of baseline testing, individual test results and disease activity can be found in Table 2. A more comprehensive overview of our study population (information about diagnosis, MRI findings and paraclinical data) has been published previously [28]. Specific patient IDs are included in Table 2 for reference.…”

Section: Resultsmentioning

confidence: 99%

“… a The same patient ID is used as in Dekeyser et al. [28]. For a more comprehensive overview we refer to this article. …”

Section: Resultsmentioning

confidence: 99%

“…corticosteroids, azathioprine, rituximab, mycophenolate mofetil, chronic plasma exchange, cyclophosphamide, natalizumab and intravenous immunoglobulin in various combinations. For specific details on this treatment, we refer to Dekeyser et al[28].Machado et al did TA B L E 2 cyclophosphamide; DR, diffusion restrictive; F, female; GM, grey matter; IT, infratentorial; IVIG, intravenous immunoglobulin; LM, leptomeningeal; M, male; MMF, mycophenolate mofetil; MRI, magnetic resonance imaging; NAT, natalizumab; NP, not performed; PLEX, plasma exchange; RTX, rituximab; ST, supratentorial. aThe same patient ID is used as in Dekeyser et al[28].…”

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confidence: 99%

“…For specific details on this treatment, we refer to Dekeyser et al[28].Machado et al did TA B L E 2 cyclophosphamide; DR, diffusion restrictive; F, female; GM, grey matter; IT, infratentorial; IVIG, intravenous immunoglobulin; LM, leptomeningeal; M, male; MMF, mycophenolate mofetil; MRI, magnetic resonance imaging; NAT, natalizumab; NP, not performed; PLEX, plasma exchange; RTX, rituximab; ST, supratentorial. aThe same patient ID is used as in Dekeyser et al[28]. For a more comprehensive overview we refer to this article.…”

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confidence: 99%

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

Van Vrekhem,

Miatton,

Hemelsoet

et al. 2024

Euro J of Neurology

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Background and purposeSusac 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.MethodsThirteen 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).ResultsPatients 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.ConclusionsOur 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.

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Section: Resultsmentioning

confidence: 99%

“… a The same patient ID is used as in Dekeyser et al. [28]. For a more comprehensive overview we refer to this article. …”

Section: Resultsmentioning

confidence: 99%

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confidence: 99%

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confidence: 99%

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

Van Vrekhem,

Miatton,

Hemelsoet

et al. 2024

Euro J of Neurology

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“…Susac syndrome (SuS) is a rare disease affecting mainly young women and characterized by an occlusive microvessel disease limited to the brain, retina, and inner ear [1][2][3], although spinal cord and skin involvement have been reported [4]. The pathophysiology is not fully elucidated [5] but recent advances support the hypothesis that SuS is an autoimmune endotheliopathy affecting the cerebral, retinal, and cochlear small vessels [6].…”

Section: Introductionmentioning

confidence: 99%

Risk factors for severe hearing loss in Susac syndrome: A national cohort study

Peyre,

Mageau,

Henry Feugeas

et al. 2024

Euro J of Neurology

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BackgroundNonreversible hearing loss (HL) is the main sequelae of Susac syndrome (SuS). We aimed to identify risk factors for HL in SuS.MethodsThe CARESS study is a prospective national cohort study that started in December 2011, including all consecutive patients with SuS referred to the French reference center. The CARESS study was designed with a follow‐up including fundoscopy, audiometry, and brain magnetic resonance imaging at 1, 3, 6, and 12 months after diagnosis and then annually for 5 years. The primary outcome was the occurrence at last follow‐up of severe HL defined as the loss of 70 dB in at least one ear on audiometry or the need for hearing aids.ResultsThirty‐six patients (female 66.7%, median age 37.5 [range 24.5–42.5] years) included in the clinical study were analyzed for the primary outcome. Thirty‐three patients (91.7%) had cochleovestibular involvement at SuS diagnosis including HL >20 dB in at least one ear in 25 cases. At diagnosis, 32 (88.9%), 11 (30.6%), and 7 (19.4%) patients had received steroids, intravenous immunoglobulin, and/or immunosuppressive (IS) drugs, respectively. After a median follow‐up of 51.8 [range 29.2–77.6] months, 19 patients (52.8%) experienced severe HL that occurred a median of 13 [range 1.5–29.5] months after diagnosis. Multivariable analysis showed that the odds of severe HL were lower in patients who received IS drugs at diagnosis (OR 0.15, 95% CI 0.01–1.07, p = 0.058).ConclusionsSevere HL in SuS is associated with the absence of IS drugs given at diagnosis. Our findings support the systematic use of IS drugs in SuS.

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