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Published on: September 13, 2020
Long-term outcome in Susac syndrome
Fleur Aubart-Cohen1, Isabelle Klein, Jean-François Alexandra
1From Internal Medicine (FA-C, J-FA, TP), Radiology (IK), Ophthalmology (SD), and Neurology (PL) Departments, Bichat Hospital, Paris; and Ophthalmology (BB, CF, PLH) and Internal Medicine (J-CP) Departments, Pitié-Salpétrière Hospital, Paris, France.
Medicine
|April 17, 2007
Summary
Susac syndrome, a rare condition affecting young women, typically improves over time with most patients returning to work. However, pregnancy can trigger relapses, and isolated retinal issues may appear late.
Area of Science:
- Neurology
- Ophthalmology
- Oto-rhino-laryngology
Background:
- Susac syndrome is a rare neurological disorder characterized by encephalopathy, hearing loss, and retinal artery occlusions.
- Long-term outcomes and the impact of pregnancy on Susac syndrome are not well-documented.
Purpose of the Study:
- To evaluate the long-term clinical outcome of Susac syndrome.
- To investigate the influence of pregnancy on the disease course.
- To analyze the natural history and sequelae of Susac syndrome.
Main Methods:
- A retrospective analysis of 9 patients with Susac syndrome, with a mean follow-up of 6.4 years.
- Review of 92 previously reported cases.
- Clinical assessment, brain MRI, funduscopy, retinal angiography, and audiometry were performed regularly.
Main Results:
- Most patients recovered well, with no severe impairment and return to work, despite persistent MRI lesions and hearing loss.
- Pregnancy was associated with disease flares, particularly postpartum.
- Isolated retinal artery involvement can manifest late, indicating the condition may not be self-limiting.
Conclusions:
- Susac syndrome generally has a favorable long-term prognosis regarding functional impairment.
- Pregnancy poses a risk for disease relapse in Susac syndrome patients.
- The non-self-limiting nature of Susac syndrome requires ongoing monitoring for late-onset retinal manifestations.
