Investigators from Tubingen and Munster, Germany, report the case of a 32-year-old woman who at 32 weeks of pregnancy developed a change in personality, disorientation, ataxia, dysarthria, and hemispasticity. MRI showed multiple diffuse T2-intense lesions, many involving the corpus callosum. CSF showed mild lymphocytic pleocytosis (13 cells/mcl) and elevated protein (1,800 mg/l) and no oligoclonal bands. A bluish, net-like exanthema on trunks and legs was diagnosed as livedo racemosa. Weeks later, she was readmitted with visual field loss and ischemic damage to both retinae, and bilateral hearing loss. With a diagnosis of Susac syndrome, IV cyclophosphamide, the standard treatment, was not instituted because of risk of permanent infertility. A combination of prednisolone, IV immunoglobulins, mycophenolate mofetil, and methotrexate provided a sustained control of symptoms. [1]
COMMENTARY. Susac syndrome (SS)[2] consists of a triad of encephalopathy, branch retinal artery occlusions and hearing loss. Associated abnormalities include multifocal corpus callosal lesions on MRI [3], resembling a vasculitis, and autoimmune disorder such as juvenile dermatomyositis. Headache is usually constant but was absent in the above case. Women are affected more often than men (3:1); the age of onset ranges from 7 to 72 years, but ages 20-40 are most vulnerable [4]. SS is an autoimmune endotheliopathy that responds to treatment with immunosuppressants, steroids, cyclophosphamide, and IV immunoglobulin, with aspirin as an adjunct [3, 5].