Reversible Bilateral Vision Loss: An Unusual Presentation of Wernicke Korsakoff Syndrome
Divya Singh1, Tony Lee3, Wilson Rodriguez2, Francesca Pastrana2, Hanan Qaqish2, Pratap Chand4
1Neurology, Saint Louis University, 2Saint Louis University, 3Saint Louis University School of Medicine, 4St Louis University School of Medicine
Objective:
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Background:
Wernicke encephalopathy (WE) encompasses a triad of altered mentation, ataxia, and oculomotor dysfunction due to thiamine deficiency. If worsened with amnesia and confabulation, it constitutes Wernicke Korsakoff syndrome (WKS). The neuro-ophthalmic manifestations of WE are uncommon and vary from nystagmus, oculomotor palsy, optic neuritis, and vision loss. We describe an unusual case of bilateral vision loss due to WKS. 
Design/Methods:

A 53-year-old woman with history of psoriatic arthritis, latent syphilis, and chronic alcoholism presented with subacute bilateral painless vision decline, and lower extremity weakness with impaired ambulation. 

The neurological exam was pertinent for confabulation, bilateral decreased visual acuity limited to light perception, no ocular or fundus abnormalities, and significant allodynia in bilateral lower extremities. Initial workup revealed elevated ESR of 130 mm/hr, positive treponema pallidum antibody, and negative RPR. Lumbar puncture showed protein of 45 mg/dl (normal 15-45 mg/dL), and non-reactive CSF VDRL. Infectious, autoimmune, paraneoplastic, and demyelinating etiologies were ruled out. Brain MRI showed abnormal FLAIR signaling in bilateral mammillary bodies and around periaqueductal gray matter concerning WE. MRI of orbits showed enhancement surrounding optic nerve suggesting retrobulbar optic perineuritis. Electrodiagnostic testing demonstrated diffuse, axonal sensorimotor polyneuropathy. Due to concerns of WKS she was started on parenteral thiamine 500 mg daily for 3 days, followed by daily 100 mg with significant improvement in vision and allodynia. Unfortunately, thiamine level was tested after receiving the first therapeutic dose and was, therefore, normal. The patient was diagnosed with WKS and discharged to acute rehabilitation with daily thiamine supplementation.

Results:
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Conclusions:
We emphasize the prompt consideration of vision loss as a neuro-ophthalmologic manifestation of WKS, especially in patients who have risk factors. We encourage testing serum levels of thiamine in this population; however, initiating empiric treatment is advocated for high clinical suspicion due to its reversible nature and minimal risk for side effects.
10.1212/WNL.0000000000205387