Cognitive dysfunction and brain atrophy in Susac syndrome

Machado, Stéphanie; Jouvent, Éric; Klein, Isabelle; Guio, François De; Machado, Carla; Cohen‐Aubart, Fleur; Sacré, Karim; Papo, Thomas

doi:10.1007/s00415-019-09664-8

Cited by 18 publications

(14 citation statements)

References 39 publications

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“…Clemence David 1,2 Thomas Papo 1,2 Ibrahima Ba 3,4 Emmanuelle Ollivier 5 Catherine Boileau 3,4,6 Philippe Dieudé 4,7 Boris Keren 8 Caroline Kannengiesser 3,4 Karim Sacre…”

Section: Co N Fli C T O F I Nte R E S T Sunclassified

“…

Because of the possible sequelae affecting these young patients [7] such fundamental studies are needed to decipher the physiopathology of SuS to improve medical care.

ACK N OWLED G M ENTSThe authors thank the UMR 8199 LIGAN-PM Genomics platform (Lille, France) which belongs to the 'Federation de Recherche' 3508 Labex EGID (European Genomics Institute for Diabetes; ANR-10-LABX-46) and was supported by the ANR Equipex 2010 session

…”

mentioning

confidence: 99%

“…

Because of the possible sequelae affecting these young patients [7] such fundamental studies are needed to decipher the physiopathology of SuS to improve medical care.

…”

mentioning

confidence: 99%

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Hunting for the genetic basis of Susac syndrome

David

Papo

et al. 2021

Euro J of Neurology

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Because of the possible sequelae affecting these young patients [7] such fundamental studies are needed to decipher the physiopathology of SuS to improve medical care. ACK N OWLED G M ENTSThe authors thank the UMR 8199 LIGAN-PM Genomics platform (Lille, France) which belongs to the 'Federation de Recherche' 3508 Labex EGID (European Genomics Institute for Diabetes; ANR-10-LABX-46) and was supported by the ANR Equipex 2010 session

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“…Clemence David 1,2 Thomas Papo 1,2 Ibrahima Ba 3,4 Emmanuelle Ollivier 5 Catherine Boileau 3,4,6 Philippe Dieudé 4,7 Boris Keren 8 Caroline Kannengiesser 3,4 Karim Sacre…”

Section: Co N Fli C T O F I Nte R E S T Sunclassified

“…

Because of the possible sequelae affecting these young patients [7] such fundamental studies are needed to decipher the physiopathology of SuS to improve medical care.

…”

mentioning

confidence: 99%

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Hunting for the genetic basis of Susac syndrome

David

Papo

et al. 2021

Euro J of Neurology

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“…Despite a worrisome presentation in most cases, the burden of sequelae is relatively mild, as already reported by our group. [17,18] Notably, the time between the first clinical event and treatment initiation was rather short. Most patients received early intravenous high-dose steroids.…”

Section: Brain Lesion Load At the Acute Phase And Outcomementioning

confidence: 99%

Brain magnetic resonance imaging lesion load at diagnosis, severity at onset and outcomes in Susac syndrome: A prospective cohort study

Scheifer

Feugeas

Roriz

et al. 2021

Euro J of Neurology

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Background Susac syndrome (SuS) is a rare occlusive microvessel disease of the brain, retina and inner ear. We aimed to determine whether brain lesion load at the acute phase predicts poor outcomes in SuS. Methods A prospective national cohort study was conducted from December 2012 to December 2019 in 20 centres in France. Patients included at the principal investigator's center with available brain magnetic resonance imaging (MRI) at diagnosis were analyzed. MRI was reviewed by an experienced neuroradiologist blinded to clinical status. The size, topography and number of hyperintense lesions on diffusion‐weighted imaging (DWI‐HL) were analyzed at diagnosis and during follow‐up. Outcomes involved descriptive characteristics of patients at onset and last follow‐up. Results Twenty‐three patients (38.1 [18.8–56.5] years, 16 females) were prospectively studied. The triad (i.e., brain, eye and ear involvement) was complete at onset in 17 patients. Brain MRI was performed 1.1 (0.1–3.4) months after the first symptom. All patients had DWI‐HL at the acute phase. Patients were separated into two groups according to the number of DWI‐HL on first MRI: a first group of patients (n=15) displaying low brain lesion load (<50 DWI‐HL per patient) and a second group of patients (n=8) displaying high brain lesion load (≥100 DWI‐HL). The median follow‐up was 57.9 (9.7–98) months. Clinical features, treatment, relapse rate, time to disappearance of DWI‐HL, disabilities and professional outcome did not differ according to brain lesion load. Conclusion Brain lesion load assessed by DWI at the acute phase is not associated with risks of disability in SuS.

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“…Nous avons parfois observé des présentations similaires, sans épisode aigu préalable, dans les formes monogéniques en particulier CADASIL. En revanche dans le syndrome de Susac les troubles cognitifs semblent toujours précédés d'épisodes aigus et en dehors des poussées, les troubles cognitifs sont voisins de ceux observés les formes sporadiques et dans CADASIL mais avec un ralentissement encore plus marqué [34]. Dans les formes post-radiques, l'installation est souvent progressive avec au premier plan un syndrome dysexécutif, et l'anamnèse permet le plus souvent d'identifier une plainte cognitive dès la prise en charge de la tumeur [12].…”

Section: Troubles Cognitifs Et Comportementauxunclassified

Spectre clinico-radiologique des maladies des petits vaisseaux cérébraux

Ouin

Jouvent

2020

La Revue de Médecine Interne

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Cognitive dysfunction and brain atrophy in Susac syndrome

Cited by 18 publications

References 39 publications

Hunting for the genetic basis of Susac syndrome

Hunting for the genetic basis of Susac syndrome

Brain magnetic resonance imaging lesion load at diagnosis, severity at onset and outcomes in Susac syndrome: A prospective cohort study

Spectre clinico-radiologique des maladies des petits vaisseaux cérébraux

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