Potential benefits of early aggressive immunotherapy in Susac syndrome

Acta neurologica Belgica
F LondonChristian Sindic

Abstract

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.

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Citations

Jun 23, 2018·Journal of Neuro-ophthalmology : the Official Journal of the North American Neuro-Ophthalmology Society·Robert A Egan
Dec 13, 2019·Journal of Neurology·Stéphanie MachadoThomas Papo
Jan 16, 2020·The Journal of Maternal-fetal & Neonatal Medicine : the Official Journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians·Luis Ramos-RupertoÁngel Robles-Marhuenda
Feb 2, 2019·Retina·Tom BuelensFrançois Willermain
Apr 3, 2021·Therapeutic Advances in Neurological Disorders·Barbara Willekens, Ilka Kleffner

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