Atypical Clinical Presentations and Neuroimaging Findings of Wernicke Encephalopathy
Shubham Biyani1, Khushboo Verma1, Narenraj Arulprakash1, Vishank Shah2, Kelly-Ann Patrice1
1Neurology, University of Arkansas for Medical Sciences, 2Neurology, Johns Hopkins School of Medicine
Objective:
 Wernicke encephalopathy is an acute neurological disorder secondary to thiamine deficiency with a clinical triad of confusion, eye disorders, and ataxia. Typical MRI Findings show symmetric hyperintensities in T2-weighted sequences predominantly affecting medial thalami, periaqueductal area, mammillary body, and the tectal plate. We describe two atypical cases of Wernicke encephalopathy.
Background:
N/A 
Design/Methods:
Retrospective chart review.
Results:

First Case: 50-year-old female with history of endometrial cancer presented with altered mental status (AMS), truncal ataxia, neck titubation, and incomplete external ophthalmoplegia, progressing rapidly to stupor.  She had recently undergone chemoradiation, complicated by chronic radiation enteritis causing poor oral intake, recurrent emesis, and weight loss. After an extensive negative infectious workup, MRI Brain with contrast was performed, which revealed restricted diffusion and T2-FLAIR sequence hyperintensities of bilateral cerebella, bilateral dorsomedial thalami, fornix with contrast enhancement of mammillary bodies concerning for Wernicke Syndrome. Thiamine levels were confirmed to be 70 nmol/l.

Second Case: 22-year-old female with history of Autism Spectrum Disorder presented with AMS and seizure-like activity after experiencing severe nausea and vomiting for one week. Infectious workup including CSF analysis was unremarkable. No seizures or epileptiform changes were captured on vEEG. MRI Brain showed T2 FLAIR hyperintensities with restricted diffusion throughout the posterior brainstem, bilateral thalami, caudate nuclei, left medial lentiform nucleus, bilateral periventricular basal ganglia, bilateral frontal and parietal lobes raising concerns for Wernicke encephalopathy. Serum thiamine level was less than 20, corroborating with the diagnosis.

 Both patients had significant clinical and radiographic improvement with high-dose thiamine supplementation.

Conclusions:

The classic triad of symptoms of Wernicke encephalopathy is usually present in only 16% of patients. Atypical MRI Brain findings include involvement of the cerebral cortex and cerebellum which are more commonly seen in non-alcoholic patients. Mortality rate ranges from 10-20% so prompt recognition of the condition and early treatment is vital especially in atypical cases.

10.1212/WNL.0000000000203635