[Retinovascular findings in Susac's syndrome].
BACKGROUND
Susac syndrome is a vasculopathy affecting the central nervous system, retina and cochlea leading to the triad encephalopathy, branch retinal artery occlusions and hearing loss. To date, about 300 cases have been described in the literature.


METHODS
Three patients with confirmed Susac syndrome were evaluated for disease-specific retinal pathologies. In addition, the overall history of the disease is presented to put the ophthalmological pathology into context.


RESULTS
All three cases showed a retinal occlusive microangiopathy with branch retinal artery occlusions. MRI imaging revealed snowball-like lesions of the corpus callosum in two of three cases. At the initial presentation not all criteria of the disease-specific triad encephalopathy, branch retinal artery occlusions, and hearing loss were fulfilled in the three patients.


CONCLUSIONS
Interdisciplinary collaboration between neurologists, otorhinolaryngologists and ophthalmologists is mandatory to establish the diagnosis of the disease. The occurrence of the characteristic retinal pathology with small artery occlusions and a segmental vasculopathy should always lead to the differential diagnosis of Susac syndrome.
Aachener Centrum für Technologietransfer in der Ophthalmologie (ACTO e. V.), Aachen.
R M
RM
Dutescu
Charité - Universitätsmedizin, NeuroCure Clinical Research Center, Berlin.
J M
JM
Dörr
Charité - Universitätsmedizin, Augenklinik, Berlin.
R
R
Bergholz
D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D034381:Hearing Loss / Q000175:diagnosis*; D006801:Humans; D008297:Male; D011859:Radiography; D012161:Retinal Artery / Q000000981:diagnostic imaging*; D055955:Susac Syndrome / Q000175:diagnosis*; D055815:Young Adult