[Susac syndrome-interdisciplinary tracking of the chameleon: two different case reports]. / Das Susac-Syndrom ­ interdisziplinär dem Chamäleon auf der Spur: 2 unterschiedliche Falldarstellungen.

BACKGROUND: The diagnosis of Susac syndrome, a presumed autoimmune retinocochleocerebral microvasculopathy, is extremely complex. At the onset of this orphan disease patients can present with an incomplete clinical triad consisting of sensorineural hearing loss, visual loss because of retinal ischemia and diverse neurological symptoms. In terms of the pathophysiology, it is assumed that the vascular endothelial cells swell, occlude the lumina of the vessels and consequently cause ischemia in the surrounding tissue. Due to the wide range of symptoms it is extremely challenging to establish a correct diagnosis. Susac syndrome should be considered as an important differential diagnosis from other neurological, ophthalmological, psychological and otorhinolaryngological diseases. CASE REPORT: This report presents two different courses of the disease in patients with Susac's syndrome. The first case of a 46-year-old woman, with previously confirmed Susac's syndrome, describes the treatment adjustment and monitoring. The second case of a 30-year-old woman shows the establishment of the initial diagnosis. DISCUSSION: The two reported cases of Susac's syndrome show the multifaceted range of clinical findings and courses of the disease. Furthermore, the diagnostic and therapeutic options are discussed with respect to the current literature. Diagnostic criteria already published by the European Susac Consortium and a good interdisciplinary collaboration enable a diagnosis as early as possible, which is essential for avoiding delayed treatment and reducing morbidity.

[Early onset of fingolimod-associated macular edema]. / Frühzeitiges Auftreten eines Fingolimod-assoziierten Makulaödems.

BACKGROUND: Fingolimod, a disease-modifying sphingosine 1­phosphate receptor modulator, which was approved in Germany in 2011, decreases the relapse rate and reduces neuroinflammation in patients with relapsing-remitting multiple sclerosis. Macular edema is a well-known ocular side effect of fingolimod therapy. Specific intervals for ophthalmologic check-ups after starting fingolimod and definite treatment schedules for fingolimod-associated macular edema are, however, still lacking. CASE REPORT: We present a case of early fingolimod-associated macular edema in a 45-year-old female patient with relapsing-remitting multiple sclerosis. The patient complained about visual impairment 1 month after the start of fingolimod and visited an eye specialist. Funduscopic examination and imaging diagnostics revealed macular edema in both eyes. The treatment with fingolimod was immediately stopped. For therapy of macular edema topical application of nepafenac and oral acetazolamide were given. During the 6 months of treatment the macular edema completely disappeared and visual function recovered completely. DISCUSSION: At the time of diagnosis, it is fundamentally important to discuss the continuation of fingolimod administration with the attending neurologist and if necessary to discontinue the drug. Regular ophthalmologic check-ups at 4­week intervals over a period of 3 months are meaningful after beginning fingolimod treatment. As before, it is still a key aspect to determine predictive opthalmologic and neurological factors before beginning treatment to evaluate which patients are at risk of fingolimod-associated macular edema.