Purpose: To report the first Japanese case of the Susac syndrome characterized by microangiopathy of the brain, retina, and cochlea.
Methods: Case report. A 36-year-old Japanese man underwent extensive clinical and laboratory examination for encephalopathy, bilateral recurrent branch retinal arterial occlusion, and hearing loss.
Results: This case was initially diagnosed as acute disseminated encephalomyelitis. Despite exhaustive clinical and laboratory examination of the patient, no evidence of a known systemic disease was found. The bilateral recurrent branch retinal arterial occlusions were widely disseminated. Remission occurred 1 year after symptom onset. This patient demonstrated all of the clinical features associated with the Susac syndrome.
Conclusion: Susac syndrome has been considered rare, but it is probably more common than previously thought and seems to occur in any race. This disorder usually occurs in young women, but it can affect men.