Case of the Month I August 2019

Case of the Month
August 14, 2019

The Case

The patient was a 51 year old woman who presented with a complaint of a central blind spot in the left eye for two months. Her past medical history was remarkable for diabetes, COPD, hypertension, coronary artery disease, hypothyroidism, and bipolar disorder and anxiety. She had ringing in her ears for about ten years, progressive left ear hearing loss, and vertigo. An MRI showed white matter changes, and demyelinating disease was in the differential diagnosis. There was left hand weakness and numbness. Her most recent hemoglobin A1c was 5.1. 

Her blood pressure was 118/88. The visual acuity was 20/20 J1+ OD and 20/25 J1+ OS. There was no afferent pupillary defect. On brightness testing, light seemed twice as bright in the right eye as the left. There was mild red desaturation in the left eye, but color vision testing was normal in both eyes. Anterior segment examination was remarkable for a mild nuclear sclerotic cataract in each eye. The posterior segment examination was remarkable for focal sheathing of an arteriole inferotemporal to the disc in the left eye. What is the most likely diagnosis? What treatment, if any, would you recommend?

The OCT below the fovea shows thinning of the inner retinal layers and loss of nerve fiber layer. The fluorescein angiogram does not show a clear blockage, but there is mild late disc staining of each eye.

This patient likely had Susac syndrome, a condition with a classic clinical triad of one or more branch retinal artery occlusions, vestibuloauditory symptoms, and personality changes. Patients occasionally have focal neurologic symptoms that typically involve the face or upper extremities. Our patient had many of the typical features of the condition. Susac’s syndrome is generally thought to be an autoimmune endotheliopathy involving arterioles in the eye, cochlea, and brain. The brain involvement often manifests as personality changes, anxiety, and paranoia. There is often asymmetric hearing loss with associated tinnitus and vertigo. There is evidence that corticosteroid and immunomodulating drugs might prevent progression of the condition.

Case Photos

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The OCT below the fovea shows thinning of the inner retinal layers and loss of nerve fiber layer. The fluorescein angiogram does not show a clear blockage, but there is mild late disc staining of each eye.

This patient likely had Susac syndrome, a condition with a classic clinical triad of one or more branch retinal artery occlusions, vestibuloauditory symptoms, and personality changes. Patients occasionally have focal neurologic symptoms that typically involve the face or upper extremities. Our patient had many of the typical features of the condition. Susac’s syndrome is generally thought to be an autoimmune endotheliopathy involving arterioles in the eye, cochlea, and brain. The brain involvement often manifests as personality changes, anxiety, and paranoia. There is often asymmetric hearing loss with associated tinnitus and vertigo. There is evidence that corticosteroid and immunomodulating drugs might prevent progression of the condition.

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