Susac syndrome.

Abstract:

:Susac syndrome is an occlusive arteriolar disease that provokes infarcts in the cochlea, retina, and brain of young subjects, mostly women. Its cause is unknown. Some infarcts may be asymptomatic and only revealed by ancillary investigation: 1) audiogram that shows bilateral sensorineural hearing loss predominating on low frequencies, 2) funduscopy and fluorescein retinal angiography demonstrating bilateral distal branch retinal artery occlusions, and 3) brain MRI T2-weighted images disclosing small multifocal hyperintensities in white and gray matter. Treatment options are not codified, ranging from antithrombotic drugs to immunomodulatory therapy. Course is self-limited after an active fluctuating phase. Dementia, blindness, and deafness are rare late sequelae, and half of patients return to normal life.

journal_name

Medicine (Baltimore)

journal_title

Medicine

authors

Papo T,Biousse V,Lehoang P,Fardeau C,N'Guyen N,Huong DL,Aumaitre O,Bousser MG,Godeau P,Piette JC

doi

10.1097/00005792-199801000-00002

subject

Has Abstract

pub_date

1998-01-01 00:00:00

pages

3-11

issue

1

eissn

0025-7974

issn

1536-5964

journal_volume

77

pub_type

杂志文章,评审

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