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Abstract Details

Deaf, Confused and Blind: A Rare Presentation of Wernicke Encephalopathy
General Neurology
P1 - Poster Session 1 (5:30 PM-6:30 PM)
4-046
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Wernicke’s encephalopathy is an acute, potentially fatal neuropsychiatric syndrome resulting from thiamine deficiency. It has traditionally been associated with alcoholism, but also been observed in other clinical settings.  Its hallmark presentation is confusion, ataxia and ophthalmoplegia but rarely manifests with hearing loss.


We report case of a 35-year-old-Caucasian female, who arrived at our ER with severe confusion/altered sensorium. She was found to have vertical nystagmus, bilateral 6th nerve palsy, bilateral hearing difficulties,  and gait instability.  Few months before, she underwent gastric-bypass surgery.  After surgery, she did not supplement her diet. One month later, she experienced abdominal pain, severe vomiting and appetite loss. She was diagnosed with acute cholecystitis, underwent cholecystectomy.  Subsequently, she started to develop confusion, hearing difficulty,  and gait instability.  ENT evaluation with audiogram revealed bilateral sensorineural-hearing deficits.  Symptoms gradually got worse, prompting multiple ER visits. Upon presentation to our ER, Wernicke’s encephalopathy was suspected. Serum vitamin levels were sent and treatment with high dose IV thiamine was started. MRI brain revealed increased FLAIR-signal within the medial thalami, hypothalami, mammillary bodies, and periaqueductal gray matter with corresponding diffusion-signal abnormality.  Later, low serum vitamin B1 was confirmed. Soon after initiation of IV thiamine, global confusion and 6th nerve palsy improved but memory deficits, nystagmus, gait dysfunction and hearing deficits persisted. At three  months follow up, there was significant improvement in gait, up-beating nystagmus, memory deficits and hearing loss.
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In this case of Wernicke's Encephalopathy, inadequate vitamin supplementation after multiple abdominal surgeries is thought to be the main cause of nutritional insufficiency and subsequent encephalopathy with ocular dysfunction, gait instability in addition to bilateral hearing loss. Thiamine deficiency was most likely the leading cause for her multiple neurological deficits. Rising number of elective procedures such as gastric sleeve are usually not associated with vitamin deficiencies such as this case.
Authors/Disclosures
Waqas I. Gilani, MD (Neurology Consultants of North Mississippi)
PRESENTER
Dr. Gilani has nothing to disclose.
Shahab Khazanehdari, MD No disclosure on file
Emad R. Noor, MD (JFK University Medical Center) Dr. Noor has nothing to disclose.