Susac Syndrome: an uncommon cause of impaired vision.

Adult Brain / pathology Female Humans Magnetic Resonance Imaging / adverse effects Retinal Artery Occlusion / diagnosis Susac Syndrome / diagnosis Vertigo / etiology
Encephalopathy Hearing loss Retinal occlusions Susac's syndrome

Journal

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
ISSN: 1590-3478
Titre abrégé: Neurol Sci
Pays: Italy
ID NLM: 100959175

Informations de publication

Date de publication:
Jun 2022
Historique:
received: 12 10 2021
accepted: 30 12 2021
pubmed: 11 1 2022
medline: 24 5 2022
entrez: 10 1 2022
Statut: ppublish

Résumé

A 35-year-old Caucasian woman presented an abrupt onset of bilateral impaired vision, and arrived to our attention two weeks later. She had a previous episode of mild dizziness. She underwent a fluorescein angiography showing branch retinal artery occlusions and a brain magnetic resonance imaging (MRI) revealing several supraand infratentorial FLAIR-hyperintense white matter lesions, two with contrast enhancement. Thrombophilic, autoimmune and infective (including Human Immunodeficiency Virus, Borrelia burgdorferi, Hepatitis B Virus, Hepatitis C Virus, Herpes Simplex Virus 1-2, Varicella Zoster Virus) screening was negative. Cerebrospinal fluid analysis showed intrathecal IgG synthesis. We suspected a Primary Central Nervous System Vasculitis, and intravenous steroids were started. Three months later a second brain MRI showed seven new lesions without contrast enhancement, and she revealed a cognitive impairment and bilateral hearing loss. Reviewing the clinical history and MRI, she fulfilled diagnostic criteria for Susac syndrome. She had two cycles of cyclophosphamide, and recovered in 6 months and then remained stable with metotrexate.

Identifiants

DOI: 10.1007/s10072-022-05865-8 PMID: 35006444 PMC: PMC9119872
pubmed: 35006444
doi: 10.1007/s10072-022-05865-8
pii: 10.1007/s10072-022-05865-8
pmc: PMC9119872
doi:

Types de publication

Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

3919-3922

Informations de copyright

© 2022. The Author(s).

Références

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Auteurs

Paolo Barbero (P)

Neurology Unit, Maggiore Della Carità Hospital, Department of Translational Medicine, University of Piemonte Orientale, Ospedale Maggiore Della Carità, via Mazzini 18, Novara, Italy. paolo91b@gmail.com.
ORCID: 0000-0002-2641-2383

Domizia Vecchio (D)

Neurology Unit, Maggiore Della Carità Hospital, Department of Translational Medicine, University of Piemonte Orientale, Ospedale Maggiore Della Carità, via Mazzini 18, Novara, Italy.
Interdisciplinary Research Center of Autoimmune Diseases (IRCAD), Department of Health Sciences, University of Piemonte Orientale, Novara, Italy.

Eleonora Virgilio (E)

Neurology Unit, Maggiore Della Carità Hospital, Department of Translational Medicine, University of Piemonte Orientale, Ospedale Maggiore Della Carità, via Mazzini 18, Novara, Italy.
Neurology Unit, S. Andrea Hospital, Department of Translational Medicine, University of Piemonte Orientale, Vercelli, Italy.

Paola Naldi (P)

Neurology Unit, Maggiore Della Carità Hospital, Department of Translational Medicine, University of Piemonte Orientale, Ospedale Maggiore Della Carità, via Mazzini 18, Novara, Italy.

Cristoforo Comi (C)

Neurology Unit, Maggiore Della Carità Hospital, Department of Translational Medicine, University of Piemonte Orientale, Ospedale Maggiore Della Carità, via Mazzini 18, Novara, Italy.
Neurology Unit, S. Andrea Hospital, Department of Translational Medicine, University of Piemonte Orientale, Vercelli, Italy.

Roberto Cantello (R)

Neurology Unit, Maggiore Della Carità Hospital, Department of Translational Medicine, University of Piemonte Orientale, Ospedale Maggiore Della Carità, via Mazzini 18, Novara, Italy.

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Classifications MeSH

questionsmedicales.fr › Personnes › Tranches d'âge › Adulte › Susac Syndrome: an uncommon cause of impaired vision.
questionsmedicales.fr › Système nerveux › Système nerveux central › Encéphale › Susac Syndrome: an uncommon cause of impaired vision.
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Susac Syndrome: an uncommon cause of impaired vision.
questionsmedicales.fr › Diagnostic › Techniques et procédures diagnostiques › Imagerie diagnostique › Tomographie › Imagerie par résonance magnétique › Susac Syndrome: an uncommon cause of impaired vision.
questionsmedicales.fr › Maladies cardiovasculaires › Maladies vasculaires › Artériopathies oblitérantes › Occlusion artérielle rétinienne › Susac Syndrome: an uncommon cause of impaired vision.
questionsmedicales.fr › États, signes et symptômes pathologiques › Signes et symptômes › Manifestations neurologiques › Syndrome de Susac › Susac Syndrome: an uncommon cause of impaired vision.
questionsmedicales.fr › États, signes et symptômes pathologiques › Signes et symptômes › Manifestations neurologiques › Vertige › Susac Syndrome: an uncommon cause of impaired vision.