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Published on: December 9, 2015
[Susac syndrome: variable onset modes and disease courses: two case reports]
H El Chehab1, A Le Corre, G Ract-Madoux
1Service d'ophtalmologie, hôpital d'instruction des armées Desgenettes, 108, boulevard Pinel, 69003 Lyon, France. elchehab_hussam@hotmail.fr
Susac syndrome, a rare microangiopathy, affects young women with encephalopathy, visual, and hearing loss. Early neurological symptoms guide therapeutic decisions for this condition.
Area of Science:
- Neurology
- Ophthalmology
- Otorhinolaryngology
Background:
- Susac syndrome is a rare microangiopathy of unknown cause.
- It presents with a triad of encephalopathy, visual loss, and hearing loss.
- The syndrome can manifest with various onset and progression patterns.
Observation:
- Two cases are presented: a 43-year-old woman with subacute encephalopathy and hearing loss, and a 27-year-old woman with recurrent neurological episodes and visual loss.
- Ophthalmic findings included retinal artery branch occlusion.
- Both patients experienced sensorineural hearing loss.
Findings:
- Susac syndrome predominantly affects young women.
- Neurological symptoms are common and varied, often preceding ophthalmic and ENT manifestations.
- Characteristic MRI findings include corpus callosum lesions; branch occlusions and vasculitis are frequent.
Implications:
- Diagnosis relies on the neurological, ophthalmic, and ENT triad, though symptom chronology can complicate it.
- Prompt recognition of neurological damage is crucial for treatment decisions.
- Treatment typically involves corticosteroids and immunosuppressants for severe cases.
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