Wernicke-Korsakoff syndrome despite no alcohol abuse: A summary of systematic reports

Abstract

Background: Wernicke-Korsakoff syndrome (WKS) is a neurological disorder typically found in alcohol use disorder. The fact that it also occurs in nonalcoholic patients is less well known and often ignored. For the first time, this review offers a systematic investigation of the frequency and associated features of nonalcoholic WKS in the published literature. Method: We included 11 recent systematic reports, with a total of 586 nonalcoholic WKS cases following hyperemesis gravidarum (n = 177), cancer (n = 129), bariatric surgery (n = 118), hunger strike (n = 41), soft drink diet in children (n = 33), depression (n = 21), Crohn's disease (n = 21), schizophrenia (n = 15), anorexia nervosa (n = 12), ulcerative colitis (n = 10), and incidental thiamine-deficient infant formula (n = 9). Findings: Vomiting and extreme weight loss were strong predictors of nonalcoholic WKS in adults. Blurred vision was a common presenting sign in about one-fourth of the patients. The classic triad of WKS is characterized by confusion, ataxia, and eye-movement disorders. All reviewed studies reported high percentages of patients presenting with an altered mental status, while both motor symptoms were variably present. Interpretation: The foregoing observations led to several important conclusions. First, we can see that nutritional impoverishment leads to profound brain damage in the form of WKS. Second, it seems that physicians are either unaware of or underestimate the risks for nonalcoholic WKS. Physicians must be specifically vigilant in detecting and treating WKS in patients with sudden and severe weight loss and vomiting. Third, lower doses of thiamine frequently lead to chronic Wernicke-Korsakoff syndrome. We noticed that when thiamine treatment for WKS was administered, in many cases doses were too low. In line with proven interventions we therefore recommend a parenteral thiamine treatment of 500 mg 3 times per day in adults.

Micronutrient Deficiencies Presenting with Optic Disc Swelling Associated with or without Intracranial Hypertension: A Systematic Review

“…Thiamine deficiency is well described among those who consume excess alcohol and little else, because alcohol is high in calories but nutrient poor. In non-alcoholic cases of WKS, there is evidence that higher body weight protects against eye movement problems [ 85 ]. Moreover, bariatric surgery is an additional risk factor for WKS secondary to malabsorption in obese individuals.…”

“…Thiamine deficiency is often diagnosed clinically, without biochemical confirmation, because thiamine compounds are photosensitive and only stable for a few hours at room temperature, so specimens need to be transported on ice and in darkness, then rapidly frozen for storage [ 86 ]. As awareness of non-alcoholic WKS among clinicians is generally low and thiamine tests are difficult to arrange, many cases remain undetected and untreated until they start to develop one or more of Wernicke’s triad [ 85 ]. Hence, milder cases of thiamine deficiency may present with isolated optic disc swelling that remains undiagnosed until additional neurological disease develops.…”

A Case of Wernicke’s Encephalopathy Due to Idiopathic Gastroparesis: A Rare Cause of Encephalopathy in a Young Woman

“…Our patient presented with several of the most common symptoms of WE: confusion, oculomotor dysfunction, polyneuropathy, and nystagmus. She also presented with the nonspecific findings of vomiting and severe weight loss central to the presentation of nonalcoholic WE [9]. However, the suspected medication overdose confounded the initial diagnosis.…”

Wernicke’s encephalopathy in advanced cancer

“…Wernicke’s encephalopathy (WE) is a neurological syndrome caused by thiamine (vitamin B1) deficiency 1. The syndrome is most commonly associated with patients with a history of alcohol misuse.…”

“…The syndrome is most commonly associated with patients with a history of alcohol misuse. It has been recognised to occur in patients without alcohol misuse including those with hyperemesis gravidarum, bariatric surgery, depression, inflammatory bowel disease, eating disorders and cancer 1 2. Cancer is thought to increase risk of thiamine deficiency through accelerated usage in a hypermetabolic state and decreased availability, which in turn may be associated with anorexia from disease or chemotherapy 3 4…”