A Case of Wernicke Encephalopathy Secondary to Anorexia Nervosa Complicated by Refeeding Syndrome and Takotsubo Cardiomyopathy.

BACKGROUND Wernicke encephalopathy (WE) is a neurological condition commonly associated with sustained alcohol abuse. However, it should be noted that disorders resulting in severe malnutrition, such as anorexia nervosa (AN), can precipitate nonalcoholic WE. AN is a life threatening psychological and eating disorder defined by inappropriate weight loss from food restriction due to the fear of gaining weight and immoderate desire to be thin. Treatment of those suffering with AN can often be complicated by severe electrolyte derangements after caloric intake termed refeeding syndrome. Although extremely rare, severe cardiomyopathy and ultimately death may occur in patients from AN. CASE REPORT Herein describes the case of a 20-year-old female with AN induced WE complicated by refeeding syndrome and hemodynamic compromise in the setting of findings consistent with takotsubo cardiomyopathy. She required ventilatory and hemodynamic support with aggressive intravenous thiamine and phosphorus repletion. Nutritional supplementation was imperative and carefully administered throughout her hospitalization. Her symptoms improved over the course of a few weeks with an ultimate reversal of her cardiomyopathy. CONCLUSIONS Given the morbidity surrounding AN, practitioners should exhibit caution when caring for those with severe nutritional deficiencies. Clinicians must monitor for severe electrolyte abnormalities and offer aggressive repletion. In addition to electrolyte derangements, severe cardiomyopathy may result as a rare sequela of the aforementioned complications associated with AN. Moreover, it is imperative to understand that patients with AN have the highest mortality of any psychiatric disorder and early intervention is necessary for survival in this vulnerable patient population.

Stability of high-dose thiamine in parenteral nutrition for treatment of patients with Wernicke's encephalopathy.

BACKGROUND & AIMS: Wernicke's encephalopathy is associated mainly with malnourishment in alcohol-dependent patients but can be caused also by cancer, Crohn's disease, gastrointestinal surgery or prolonged parenteral nutrition (PN) without adequate supplementation of vitamins. The disorder, with a significant mortality rate of up to 20%, is often associated with the underlying disease and intensifies after administration of non-supplemented PN. Thus, it seems justified to add thiamine to PN admixtures prepared for parenterally fed patients. Due to the lack of data on the stability of thiamine in PN admixtures at concentrations exceeding 60 mg/L, we decided to determine the possibility of adding a high dose of thiamine (800 mg per bag, 320 mg/L) to PN admixtures in order to treat Wernicke's encephalopathy in malnourished patients. METHODS: The study aimed to assess the stability of the physical properties of PN admixtures (pH, zeta potential, particle size) and to determine thiamine content using an HPLC method. RESULTS: Thiamine was found to degrade regardless of the PN admixture composition and storage conditions. The highest decrease in thiamine content was observed at room temperature without light protection whereas the lowest at a temperature of 4 ± 1 °C with light protection. CONCLUSIONS: The treatment of Wernicke's encephalopathy in parenterally fed patients is possible with the use of high thiamine doses (800 mg) added to PN admixtures without a decrease in the drug content above 10% within the first 24 h. It should be emphasized that thiamine as a photosensitive drug must be stored and administered under conditions ensuring light protection.

Hypothalamic sydrome as an initial presentation of Wernicke encephalopathy: A case report.

RATIONALE: Wernicke encephalopathy (WE) is a syndrome characterized by an acute or subacute onset of ataxia, ophthalmoplegia, and mental status changes. To our knowledge, hypothalamic syndrome is rare in WE. PATIENT CONCERNS: A 73-year-old female patient with acute cerebral infarct, who showed initial symptoms of vomiting, nausea, ataxia, and subsequent anorexia, was treated with parenteral nutritional supplement for 20 days. Nevertheless, the patient still developed refractory hyponatremia despite the appropriate sodium supplement given for a week following parenteral nutritional supplement. In fact, after 14 days of parenteral nutritional supplement, the patient gradually showed hypotension and apathy. Hyponatremia, hypotension, anorexia and apathy were signs of hypothalamic syndrome. DIAGNOSES: Finally, the patient was diagnosed as WE by head magnetic resonance imaging, which showed symmetrical lesions in T2-weighted imaging images and FLAIR high signal intensity in the periaqueduct, hypothalamus, thalamus, mammiliary bodies, medulla oblongata, and vermis cerebelli. INTERVENTIONS: The patient was given thiamine supplementation. OUTCOMES: The patient regained consciousness within 3 days. The sings of hyponatremia, hypotension, and apathy were relieved subsequently. LESSONS: When patients develop unexplained hypothalamic syndrome, we should think of the possibility of WE. The concomitant presence of hyponatremia, hypotension, anorexia, and apathy in WE is rare. Therefore, this case is reported here for discussion.

Wernicke Encephalopathy in Adolescents After Bariatric Surgery: Case Report and Review.

Roughly 1% of all weight loss surgery is performed in adolescents. There is strong evidence demonstrating significant postsurgical weight loss, improvement in quality of life, and reduction in comorbidities such as hypertension and diabetes. Reports of postoperative complications in adolescents are few because of the small sample size in most series. Despite vitamin supplementation, nutritional deficiencies requiring hospitalization occur occasionally after Roux-en-Y gastric bypass. Wernicke encephalopathy, a triad of ophthalmoplegia, ataxia, and altered mental status, is a serious consequence of thiamine (vitamin B1) deficiency. Few cases of Wernicke encephalopathy after weight loss surgery have been reported in the literature and even fewer in the pediatric population. Here we describe a teenage girl who develops vomiting after Roux-en-Y gastric bypass and presented with nystagmus, irritability, and ataxia. The clinical presentation, diagnosis, and treatment of Wernicke encephalopathy in adolescents after bariatric surgery are discussed.

Clinical awareness for health care professionals: Fatal encephalopathy complicating persistent vomiting in pregnancy.

Women with persistent vomiting during pregnancy need early referral to appropriate health facilities. Delayed referral and inappropriate management may lead to metabolic encephalopathy from a variety of causes, including electrolyte derangements or thiamine deficiency (Wernicke's encephalopathy) (WE). We present a case of persistent vomiting in pregnancy in which there was delayed referral, inappropriate treatment and failure to associate neurological signs such as terminal neck stiffness with WE, resulting in poor fetomaternal outcomes. In this report, we discuss the following lessons: (i) the need for early transfer of a patient with persistent vomiting and enigmatic clinical features to a higher healthcare facility; (ii) failure to associate neurological signs with complications of hyperemesis gravidarum/WE; (iii) lack of thiamine supplementation; and (iv) the advantages of magnetic resonance imaging over a computed tomography scan in the diagnosis of WE.

Malnutrition-induced Wernicke's encephalopathy following a water-only fasting diet.

BACKGROUND: Wernicke's encephalopathy is a critical condition of neurological dysfunction resulting from a deficiency in thiamine. Chronic alcoholism is recognized as the most common cause of Wernicke's encephalopathy, but other causes, including fasting/starvation and malnutrition, have been documented within the scientific literature. These causes may not be readily recognized by healthcare professionals and may lead to Wernicke's encephalopathy being overlooked as a diagnosis when a nonalcoholic patient presents with classic signs and symptoms of the disorder. MATERIALS AND METHODS: A narrative review of thiamine and its relationship to the development, diagnosis, and treatment of Wernicke's encephalopathy is presented based on a review of evidence-based guidelines and published research. To heighten awareness of the development of Wernicke's encephalopathy in fasted/starved and malnourished patients and to contribute to the scientific body of knowledge for the identification and management of Wernicke's encephalopathy in these patients, the clinical course and treatment of an adult woman who developed Wernicke's encephalopathy following a 40-day water-only fasting diet is outlined. RESULTS: Clinical suspicion was required to identify the patient's condition and initiate immediate intervention through parenteral thiamine administration. Oral thiamine supplementation of 100 to 800 mg per day for 6 months was required to aid recovery. OUTCOMES: The patient's clinical course and response to treatment illustrate the necessity for clinical awareness and suspicion of Wernicke's encephalopathy among healthcare professionals, timely and adequate parenteral thiamine administration, and oral thiamine supplementation at therapeutic doses to correct the nutrient deficiency, halt the progression of Wernicke's encephalopathy, and promote recovery.

[Wernicke's encephalopathy associated with pellagra encephalopathy: rare and unusual complication in an elderly woman hospitalized for aspiration pneumonia]. / Encéphalopathie de Gayet Wernicke associée à une encéphalopathie pellagrique : complication rare et inhabituelle chez une femme âgée hospitalisée pour pneumopathie d'inhalation.

INTRODUCTION: Wernicke's encephalopathy caused by thiamine deficiency is typically characterised by a mental-status change, oculomotor dysfunction and an ataxia. Pellagra is the clinical presentation of niacin deficiency comprising cutaneous, gastrointestinal and neuropsychiatric manifestations. OBSERVATION: We report a case of encephalopathy due to dual vitamin deficiency of both thiamine (vitamin B1) and niacin (vitamin PP) in an 80-year-old women, hospitalized for severe sepsis caused by aspiration pneumonia. Severe malnutrition and alcohol consumption pointed to a diagnosis of vitamin deficiency. The clinical presentation and magnetic resonance imaging (MRI) were compatible with Wernicke's encephalopathy that remained irreversible despite vitamin B1 supplementation. Niacin supplementation allowed for complete regression of the observed symptoms compatible with niacin deficiency. CONCLUSION: Malnourished and alcoholic patients showing signs of encephalopathy should receive supplemental multivitamins including niacin.

Wernicke's syndrome after sleeve gastrectomy.

We report a case of Wernicke's encephalopathy after sleeve gastrectomy, which had been complicated by stomach wall edema and aggravated by dietary non-compliance. Despite intense parenteral nutrition, thiamine deficiency became clinically evident. It suggests that nutritional preparations used were unable to cover the increased thiamine requirement. After intense thiamine supplementation, gradual improvement occurred during the 6 months after the diagnosis, without permanent cognitive impairment. Clinicians involved in postoperative management of bariatric surgery patients must consider Wernicke's syndrome in hyper-emetic patients, who show unclear neurological deterioration. Early diagnosis and treatment can instantly improve the patient's condition without permanent sequelae.

Hyperemesis gravidarum complicated by Wernicke encephalopathy: background, case report, and review of the literature.

Wernicke encephalopathy (WE) is a rare but known complication of severe hyperemesis gravidarum caused by thiamine deficiency. This article presents an unusual case that occurred at our institution and reviews the 48 previously published cases of WE in pregnancy. Considering all the 49 cases, the mean (+/-standard deviation) patients' age was 26.7 +/- 4.9 years, the mean gestational age when WE manifested was 14.3 +/- 3.4 weeks, and the mean duration of vomiting and feeding difficulties was 7.7 +/- 2.8 weeks. Wernicke's classic triad (confusion, ocular abnormalities, and ataxia) manifested in only 46.9% (23 of 49) of the patients. Confusion affected 63.3% (31 of 49) of the patients, ocular signs 95.9% (47 of 49) and symptoms 57.1% (28 of 49), and ataxia 81.6% (40 of 49). Deterioration of consciousness affected 53.1% (26 of 49) of the subjects and memory impairment 61.2% (30 of 49). Complete remission of the disease occurred in only 14 of 49 cases. Symptom resolution required months and permanent impairments were common. The overall pregnancy loss rate, directly (spontaneous fetal loss) and indirectly (planned abortion) attributable to WE, was 47.9% (23 of 49). The diagnosis of WE is clinical and can be rapidly confirmed by magnetic resonance imaging. We emphasize the importance of thiamine supplementation to women with prolonged vomiting in pregnancy, especially before intravenous or parenteral nutrition. We also underline the necessity to promptly replace vitamin B1 when neurologic symptoms and/or signs develop in a patient with hyperemesis gravidarum.

B Vitamin deficiency and neuropsychiatric syndromes in alcohol misuse.

Alcohol misuse and alcohol withdrawal are associated with a variety of neuropsychiatric syndromes, some of which are associated with significant morbidity and mortality. B vitamin deficiency is known to contribute to the aetiology of a number of these syndromes, and B vitamin supplementation thus plays a significant part in prophylaxis and treatment. In particular, the Wernicke Korsakoff syndrome (WKS). due to thiamine deficiency, is a common condition in association with alcohol misuse, and is associated with high morbidity and mortality. Nicotinamide deficiency may result in a rarer condition, alcoholic pellagra encephalopathy, which often has a similar clinical presentation to WKS. This review considers the role of B vitamins in the aetiology and treatment of neuropsychiatric syndromes associated with alcohol misuse, with particular emphasis on WKS.

Prevention of the Wernicke-Korsakoff syndrome: a cost-benefit analysis.

The Wernicke-Korsakoff syndrome is a thiamine-deficiency disorder occurring primarily among alcoholics. To determine the economic feasibility of preventing this disease by fortification of alcoholic beverages with thiamine, we compared the cost of fortification with the cost of institutionalizing alcoholics with the disorder. The estimated annual incidence of institutionalization is eight per million adult population. The cost of long-term institutionalization, discounted to present value, is $70 million per year. The cost of adequately fortifying alcoholic beverages is estimated to range from $3 million per year if allithiamines are used, to as much as $17 million per year if thiamine hydrochloride proves necessary. Thus, the cost-benefit ratio may range from 1:23 to 1:4. It is economically advantageous to prevent the Wernicke-Korsakoff syndrome by fortification of alcoholic beverages with thiamine. The stability, safety and marketability of thiamine and the allithiamines in alcoholic beverages should be studied further.