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Potential benefits of early aggressive immunotherapy in Susac syndrome.

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London, Frédéric [UCL] Pothalil, Deepa Duprez, Thierry [UCL] Sindic, Christian [UCL]

Susac syndrome is a rare vasculopathy of unknown aetiology, affecting prominently young women and electively targeting brain (encephalopathy), retina (visual field defects), and cochlea (hearing loss), of which optimal treatment has yet to be established. We report clinical, CSF and MRI features together with the long-term outcome in a monocentric series of eight consecutive patients with unusual sex ratio (5 male; 3 female), to define the best diagnostic/therapeutic strategy. Six patients presented with the classical clinical triad within less than 6 months after symptoms onset; two did not suffer from sensorineural hearing loss. All but one received a treatment combining high doses of methylprednisolone and cyclophosphamide intravenously. The first two patients had very delayed diagnosis (6-4 months) resulting in severe cognitive sequelae. The third one had only mildly delayed diagnosis (2 months) with subsequent behaviour impairment and severe right hypoacousia. All three were unable to return to work. The last five patients who had early diagnosis and undelayed aggressive treatment were able to resume their professional activities.

Document type Article de périodique (Journal article) – Article de recherche
Access type Accès restreint
Publication date 2016
Language Anglais
Journal information "Acta Neurologica Belgica" - Vol. 116, no. 4, p. 451-460 (2016)
Peer reviewed yes
Publisher Springer ((Italy) Milano)
issn 0300-9009
e-issn 2240-2993
Publication status Publié
Affiliations UCL - (MGD) Service de neurologie
UCL - SSS/IONS - Institute of NeuroScience
UCL - SSS/IREC/MONT-Pôle Mont Godinne
UCL - SSS/IONS/CEMO - Pôle Cellulaire et moléculaire
UCL - SSS/IONS/NEUR - Clinical Neuroscience
UCL - (SLuc) Service de radiologie
UCL - (SLuc) Service de neurologie
Keywords Encephalopathy ; Hearing loss ; Immunotherapy ; Retina ; Vasculopathy
Links
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Bibliographic reference London, Frédéric ; Pothalil, Deepa ; Duprez, Thierry ; Sindic, Christian. Potential benefits of early aggressive immunotherapy in Susac syndrome.. In: Acta Neurologica Belgica, Vol. 116, no. 4, p. 451-460 (2016)
Permanent URL http://hdl.handle.net/2078.1/179803