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April 2001

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Copyright 2001 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2001

Arch Otolaryngol Head Neck Surg. 2001;127(4):468. doi:

We thank Dr Sirikci for his interest in our article. Dr Sirikci comments on the differential diagnosis between microangiopathy of the inner ear, retina, and brain (Susac syndrome) and MS. In addition to deafness and retinal occlusions, the main clinical manifestation of Susac syndrome is an encephalopathy, and all these manifestations are never observed (retinal occlusions) or are quite rare (profound deafness or encephalopathy) in MS. Moreover, magnetic resonance imaging and cerebrospinal fluid findings are different in MS and Susac syndrome. Brain scans suggest MS when several high-signal lesions are observed in the cerebral white matter, ovoid lesions oriented perpendicular to ventricules, or lesions with a diameter greater than 6 mm or located in the corpus callosum.1 In Susac syndrome, the high-signal lesions are punctiform and are observed not only in the white matter but also in the gray matter.2,3 In MS, cerebrospinal fluid analysis reveals a normal or discrete increase in proteins, mainly in γ-globulins, with IgG oligoclonal bands.4 In Susac syndrome, cerebrospinal fluid protein levels are often higher than 1 g/L (which is never the case in MS), and there are no IgG oligoclonal bands.2,3 Finally, treatment of MS and Susac syndrome differs largely: in MS, several specific treatments are available (eg, interferons and glatiramer acetate), but in Susac's syndrome treatment remains empirical. We do not believe that MS and Susac syndrome are confounding diseases because of the differences in diagnostic criteria.2,5

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