[Aggressive immunotherapy in Susac's syndrome]

Area of Science:

• Neurology
• Immunology

Background:

• Susac's syndrome is a rare condition affecting young women, presenting as aseptic encephalitis with neurological deficits and white matter lesions.
• It is characterized by a triad of encephalopathy, branch retinal artery occlusions, and sensorineural hearing loss.

Observation:

• A 36-year-old woman presented with encephalopathy, leg weakness, and white matter lesions on MRI.
• She subsequently developed inner ear deafness and retinal artery occlusions, leading to a Susac's syndrome diagnosis.

Findings:

• Initial treatment with high-dose corticosteroids was followed by worsening symptoms upon steroid reduction.
• Sequential escalation of immunotherapy, including high-dose cyclophosphamide, achieved disease control.

Implications:

• This case supports early and aggressive immunosuppression for Susac's syndrome, similar to juvenile dermatomyositis treatment protocols.
• The findings emphasize the importance of considering Susac's syndrome in young women with unexplained neurological and visual symptoms.