Susac syndrome is a microangiopathy of unknown origin, probably autoimmune, affecting capillaries and precapillary arterioles of the brain, retina, and inner ear. It is often misdiagnosed as acute disseminated encephalomyelitis or multiple sclerosis. We report the case of a 25-year-old male with Susac syndrome who developed the clinical triad of encephalopathy, visual and hearing problems over ...

CASE REPORT A 25-year-old woman with encephalopathy and sensorineural hearing loss was referred to us in order to confirm Susac syndrome, due to bilateral visual loss. Funduscopic examination revealed peripheral retinal microangiopathy. DISCUSSION Susac syndrome is typically characterized by the clinical triad of encephalopathy, branch retinal artery occlusions, and hearing loss. In our case,...

Susac syndrome is an autoimmune microangiopathy affecting the brain, retina and inner ear (cochlea and semicircular canals), leading to encephalopathy, branch retinal artery occlusions (BRAOs) and asymmetric neurosensory hearing loss, respectively. The natural history and long-term prognosis are variable as the disease has been shown to be monophasic and self-limiting, polycyclic or chronic con...

Susac syndrome is a rare microangiopathy of cochlea, retina, and brain. We report a case of a 30-year-old man with Susac syndrome. The patient initially suffered from unilateral hearing loss associated with peripheral vestibular syndrome, and followed with recurrent arterial retinal occlusions and encephalopathy. The patient underwent clinical, laboratory, and neuroradiological examination. Lab...

We thank Drs. Yildirim, Kar and Kaya for their interest regarding our paper reporting the uncommon occurrence of central retinal artery occlusion (CRAO) in Susac syndrome (SS) and for re-emphasizing the point that we have made in the article that SS should be kept in mind even when a patient presents with CRAO in one eye only. However we would like to point out that their statement “retinal art...

Susac’s syndrome consists of the clinical triad of encephalopathy, branch retinal artery occlusions, and hearing loss. In 1975, I saw two patients with this syndrome within a matter of 3 weeks while serving in the United States Army at Walter Reed Army Hospital. The first patient was presented to me at a conference in Albany, New York, and the second was referred to me by Dr. John Selhorst. I r...

Dear Editor, I read with interest the article titled “Acute central retinal artery occlusion associated with livedoid vasculopathy: a variant of Sneddon’s syndrome [1] and there may be a different diagnosis. Since a few cases of young patients have been identified with Susac syndrome with livedo, could this case represent a case of this disorder? Did the patient have an magnetic resonance imagi...

We report the case of a 24-year-old man presenting with sudden visual loss in the left eye from a central retinal artery occlusion. An extensive clinical investigation revealed no etiology. Three weeks later, however, the patient developed hearing loss followed by encephalopathy and multiple branch retinal artery occlusions in the right eye. Fluorescein angiography confirmed retinal vascular oc...