A Syndrome of Arterial-Occlusive Retinopathy and Encephalopathy

Unilateral central retinal artery occlusion as the sole presenting sign of Susac syndrome in a young man: case report

“…In two sub sequent papers, SS was defined as a microangiopathic syndrome of arterial occlusive disease leading to the classic triad of encephalopathy, hearing loss and MBRAO (2,3) The pathogenesis of SS remains unknown, but presumably an autoimmune process leads to damage and inflammation-related occlusion of the microvessels in the brain, retina, and inner ear (5) . Because the first 20 cases reported were women aged 21-41 years, the syndrome was initially believed to affect only young females.…”

“…Susac syndrome (SS) is an arterial occlusive disease associated with a triad of symptoms: encephalopathy, hearing loss and multiple branch retinal artery occlusions (MBRAO) (1)(2)(3) . Other findings include diffuse neurological signs as headache, psychiatric disturbances, cognitive changes, memory loss, and confusion that may rapidly progress to dementia.…”

Microangiopathy of the brain, retina, and ear: improvement without immunosuppressive therapy.

“…A cerebral arteriogram done 4 days after the delivery was normal. A second CSF examination showed a protein concentration of 144 mg/dl, a glucose concentration of 59 mg/dl, 1 white blood cell/mm 3 , and a normal immunoelectrophoresis pattern.…”

“…A CT scan of the brain without contrast done on Jury 1 was normal. Anticoagulants were stopped, and cerebrospinal fluid (CSF) examination revealed a protein concentration of 207 mg/dl with a glucose concentration of 55 mg/dl (serum glucose concentration 89 mg/dl), 2 white blood cells/mm 3 , a normal myelin-basic protein concentration and IgG index, and absent oligoclonal bands. Another CT scan without contrast done on Jury 5 showed small areas of hypodensity in the genu of the corpus callosum and the periventricular white matter.…”

Reply: progression of retinal artery occlusion in Susac syndrome

“…In fact, one of the two original cases (case 1) described by Susac, Hardman and Selhorst (2) had BRAO in only one eye despite having an advanced disease. However, when we added the four subsequent cases that allowed a full characterization of the syndrome (3,4) BRAO was present in both eyes in five of the six cases. On the other hand, we completely agree with Drs.…”