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Ultrasonographic Evaluation of Salivary Glands for Sjogren's Syndrome: Diagnostic and Monitoring Insights

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[Susac syndrome: study of five cases].

E Maillart¹, R Deschamps, A Moulignier

¹Service de neurologie, fondation ophtalmologique Adolphe-de-Rothschild, 25-29, rue Manin, 75940 Paris cedex 19, France. elisabethmaillart@yahoo.fr

Revue Neurologique

|January 7, 2009

Summary

Susac syndrome, a rare microangiopathy causing infarcts in the brain, retina, and cochlea, presents a diagnostic challenge. Early immunologic treatment combined with anticoagulants led to significant clinical improvement in five patients.

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Area of Science:

Neurology
Ophthalmology
Otolaryngology

Background:

Susac syndrome is a rare microangiopathy causing small infarcts in the brain, retina, and cochlea.
The classic triad includes neurological signs, retinal artery occlusions, and sensorineural hearing loss.

Observation:

A series of five patients (four women, one man; mean age 35.2 years) with Susac syndrome were studied.
Presenting symptoms varied, including neurological, ophthalmological, auditory, or the full clinical triad.
Neurologic symptoms encompassed encephalopathy, headaches, and transient ischemic attacks.

Findings:

Brain MRI revealed T2 lesions in white/grey matter, corpus callosum, and punctiform gadolinium-enhanced lesions.
Elevated cerebrospinal fluid protein was noted in three patients.
Aggressive immunologic treatments (steroids, cyclophosphamide, IVIg) with anticoagulants resulted in dramatic clinical improvement in all five patients, despite two experiencing early relapses.

Implications:

Susac syndrome's polymorphic nature complicates diagnosis, especially when the classic triad is absent.
Empirical treatment, based on presumed pathogenesis, is currently standard due to a lack of clinical trials.
Early and aggressive treatment appears crucial for favorable outcomes in Susac syndrome.