Stem Cell Therapy and Thiamine Deficiency-Induced Brain Damage.

Wernicke's encephalopathy (WE) is a major central nervous system disorder resulting from thiamine deficiency (TD) in which a number of brain regions can develop serious damage including the thalamus and inferior colliculus. Despite decades of research into the pathophysiology of TD and potential therapeutic interventions, little progress has been made regarding effective treatment following the development of brain lesions and its associated cognitive issues. Recent developments in our understanding of stem cells suggest they are capable of repairing damage and improving function in different maladys. This article puts forward the case for the potential use of stem cell treatment as a therapeutic strategy in WE by first examining the effects of TD on brain functional integrity and its consequences. The second half of the paper will address the future benefits of treating TD with these cells by focusing on their nature and their potential to effectively treat neurodegenerative diseases that share some overlapping pathophysiological features with TD. At the same time, some of the obstacles these cells will have to overcome in order to become a viable therapeutic strategy for treating this potentially life-threatening illness in humans will be highlighted.

A case report of Wernicke Korsakoff syndrome in a patient with cholangiocellular carcinoma: An underestimated cause of encephalopathy in cancer patients.

INTRODUCTION: Wernicke's encephalopathy, a disorder caused by thiamin deficiency, is characterized by a classical triad of encephalopathy, ataxia, and ophthalmoplegia. Although alcoholism is the most common predisposing factor, it can also be associated with nonalcoholic states (hyperemesis gravidarum, intestinal obstruction, bariatric surgery, and others). This work presents a case of nonalcoholic Wernicke-Korsakoff syndrome diagnosed in a cholangiocellular carcinoma patient and literature review. CASE REPORT: A 65-year-old male patient with a history of cholangiocellular carcinoma (Klatskin tumor) was treated with radiotherapy at the operation site after Roux-en-Y hepaticojejunostomy. During follow-up, the patient developed gastric outlet obstruction and was diagnosed with peritoneal carcinomatosis after a palliative gastrojejunostomy. As the patient could not tolerate oral nutrition during hospitalization, total parenteral nutrition was administered. After 10 days of admission, the patient showed decreased response to verbal stimuli as well as bilateral horizontal nystagmus, lethargy, and disorientation. Furthermore, the patient displayed confabulation. Clinical and imaging findings were consistent with Wernicke's encephalopathy. Therefore, treatment with intravenous thiamin replacement was initiated. The patient's encephalopathy regressed on the second day after treatment, and he recovered the place-person-time orientation. In the following month, the abnormal imaging findings were almost entirely resolved. CONCLUSION: In order to prevent irreversible brain damage induced by chronic thiamin deficiency, thiamin replacement therapy with parenteral nutrition solutions should be included as a treatment for hospitalized cancer patients unable to receive enteral nutrition for a long time.

Non-Alcohol-Related Wernicke's Encephalopathy: Diagnosis and Treatment.

The Psychiatric Consultation Service at Massachusetts General Hospital sees medical and surgical inpatients with comorbid psychiatric symptoms and conditions. During their twice-weekly rounds, Dr Stern and other members of the Consultation Service discuss diagnosis and management of hospitalized patients with complex medical or surgical problems who also demonstrate psychiatric symptoms or conditions. These discussions have given rise to rounds reports that will prove useful for clinicians practicing at the interface of medicine and psychiatry.

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

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.

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.

Don't seek, don't find: The diagnostic challenge of Wernicke's encephalopathy.

Wernicke's encephalopathy is caused by thiamine deficiency and has a range of presenting features, including gait disturbance, altered cognitive state, nystagmus and other eye movement disorders. In the past, Wernicke's encephalopathy was described almost exclusively in the alcohol-dependent population. However, in current times, Wernicke's encephalopathy is also well recognized in many other patient groups, including patients following bariatric surgery, gastrointestinal surgery, cancer and pancreatitis. Early recognition of Wernicke's encephalopathy is vital, as prompt treatment can restore cognitive or ocular function and can prevent permanent disability. Unfortunately, Wernicke's encephalopathy is often undiagnosed - presumably because it is relatively uncommon and has a variable clinical presentation. Clinical biochemists have a unique role in advising clinicians about potential nutritional or metabolic causes of unexplained neurological symptoms and to prompt consideration of thiamine deficiency as a potential cause in high-risk patient groups. The aim of this review is to summarize the clinical features, diagnosis and treatment of Wernicke's encephalopathy and to highlight some non-traditional causes, such as after bariatric surgery.

[The knowledge of resident doctors on diagnostics, etiology and treatment of Wernicke encephalopathy]. / De kennis van a(n)iossen over diagnostiek, etiologie en behandeling van wernicke-encefalopathie.

BACKGROUND: Wernicke encephalopathy (we) is a severe, acute neuropsychiatric disorder caused by a deficiency in thiamine. There have been indications that we is undertreated, which can lead to the Korsakoff syndrome, delirium or death. Treatment according to protocol is simple and effective. The knowledge of physicians about we has not been researched before.
AIM: To test the knowledge of resident doctors on diagnosis, etiology and treatment of we.
METHOD: The knowledge of 70 resident doctors in different medical specialties was examined through two clinical cases: the first with we due to hyperemesis gravidarum and the second due to alcohol abuse. Both open and multiple-choice questions were asked. Cues of the classical triad of we (cognitive disorder, eye movement disorder and gait disorder) were given accumulatively.
RESULTS: The classical triad of we was not recognized by 73% of the resident doctors in the case of hyperemesis gravidarum and they missed we in the case of alcohol abuse. Many of the resident doctors were not able to name the thiamin deficiency, the triad of we, more than three causes of we or the correct treatment with thiamine sufficiently. 67% of resident doctors indicated that their knowledge of we was insufficient and 76% expressed a need for more information about we.
CONCLUSION: The knowledge of resident doctors about the diagnostics, etiology and management of we is insufficient. Moreover, the resident doctors evaluate their knowledge about we to be insufficient. Medical school and postgraduate specialization have to focus more on this common and severe syndrome, which can appear in different medical areas.

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.

Desnutrición en el anciano y encefalopatía de Wernicke / Malnutrition in the elderly and Wernicke encephalopathy

No disponible

A view from the acute hospital: managing patients with alcohol problems.

Regular heavy consumption of alcohol is associated with a wide range of physical, psychological and social problems. All health-care clinicians should be able to screen for and detect problematic levels of alcohol consumption in their patients, and deliver an effective brief intervention. When patients with alcohol dependence are admitted to hospital there must be an assessment of whether medication is required to prevent withdrawal symptoms and potential delirium tremens and withdrawal seizures. Medically assisted alcohol withdrawal using a long-acting benzodiazepine such as chlordiazepoxide should be carefully monitored and titrated to effect, and the clinician should be aware of the risk of Wernicke-Korsakoff syndrome and other complications. Abstinence from alcohol is usually only the first step in treatment, and effective linkage to community alcohol services is an important step.

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 concurrent with polyradiculoneuropathy in a young man after bariatric surgery: A case report.

RATIONALE: Bariatric surgery is the recommended treatment for morbid obesity because of its rapid and sustained body weight loss effect. Nutrient deficiency-related neurological complications after bariatric surgery are often disabling. Thus, early recognition of these complications is important. Neurological complications involving the central and peripheral nerve system after bariatric surgery were reported. However, the report on the clinical course of the concurrent involvement of central and peripheral nervous system is limited. We present a rare case of a patient who developed Wernicke encephalopathy concurrent with polyradiculoneuropathy after receiving bariatric surgery. PATIENT CONCERNS: A 22-year-old man with a history of morbid obesity presented progressive bilateral lower limbs weakness, blurred vision, and gait disturbance 2 months after receiving laparoscopic sleeve gastrectomy. Bilateral lower limb numbness and cognition impairment were also noted. DIAGNOSIS: Brain magnetic resonance imaging and electrophysiologic studies confirmed the diagnosis of Wernicke encephalopathy concurrent with acute polyradiculoneuropathy. INTERVENTIONS: Vitamin B and folic acid were given since admission. He also received regular intensive rehabilitation program. OUTCOMES: The subject's cognitive impairment and diplopia improved 1 week after admission under medical treatments, yet lower limb weakness and gait disturbance were still noted. After a month of intensive inpatient rehabilitation, he was able to ambulate with a walker for 30 munder supervision. LESSONS: Nutrient deficiency-related neurological complications after bariatric surgery are often disabling and even fatal. Prevention of neurological complications can be improved through close postsurgical follow-up of the nutritional status. Recognizing the signs and symptoms and evaluating the medical history are critical to the early diagnosis and treatment of this potentially serious yet treatable condition.

[The prevention of Korsakoff's syndrome by offering involuntary care?] / Voorkómen van het syndroom van Korsakov door aanbieden onvrijwillige zorg?

BACKGROUND: The new draft Law on care and coercion focuses on the care of people with a psychogeriatric disorder or intellectual disability. Perhaps this law can be applied to people suffering from chronic alcoholism, associated with an increased risk of Korsakoff's syndrome. AIM: To explore whether the new draft law on care and coercion can be applied to people suffering from chronic alcoholism who still live at home. METHOD: A description of possibilities of involuntary homecare to people suffering from chronic alcoholism based on literature, current legislation and two new draft laws. RESULTS: It seems possible to apply the law care and coercion to people suffering from chronic alcoholism. CONCLUSION: The application of the draft law care and coercion offers the possibility of providing involuntary homecare to people suffering from chronic alcoholism, enabling the administration of thiamine. This reduces the chances of developing Korsakoff's syndrome. Further research is necessary to explore the practical possibilities in offering involuntary care.

Early detection and successful treatment of Wernicke's encephalopathy in outpatients without the complete classic triad of symptoms who attended a psycho-oncology clinic.

OBJECTIVE: Wernicke's encephalopathy (WE) is a neuropsychiatric disorder caused by a thiamine deficiency. Although WE has been recognized in cancer patients, it can be overlooked because many patients do not exhibit symptoms that are typical of WE, such as delirium, ataxia, or ocular palsy. Furthermore, outpatients with WE who intermittently present at psycho-oncology clinics have not been described as far as we can ascertain. METHOD: This report describes two patients who did not exhibit the complete classic triad of symptoms among a series with cancer and WE, and who attended a psycho-oncology outpatient clinic.ResultCase 1, a 76-year-old woman with pancreatic cancer and liver metastasis, periodically attended a psycho-oncology outpatient clinic. She presented with delirium and ataxia as well as appetite loss that had persisted for 8 weeks. We suspected WE, which was confirmed by low serum thiamine levels and the disappearance of delirium after thiamine administration. Case 2, a 79-year-old man with advanced stomach cancer, was referred to a psycho-oncology outpatient clinic with depression that had persisted for about 1 month. He also had appetite loss that had persisted for several weeks. He became delirious during the first visit to the outpatient clinic. Our initial suspicion of WE was confirmed by low serum thiamine levels and the disappearance of delirium after thiamine administration. The key indicator of a diagnosis of WE in both patients was appetite loss.Significance of resultsThis report emphasizes awareness of WE in the outpatient setting, even when patients do not exhibit the classical triad of WE. Appetite loss might be the key to a diagnosis of WE in the absence of other causes of delirium.

Are Psychiatric Inpatients at Risk of Developing Wernicke's Encephalopathy Being Identified and Adequately Treated?

OBJECTIVE: Wernicke's encephalopathy is caused by thiamine deficiency and occurs predominantly in alcohol-dependent individuals but also develops in those who are malnourished due to other reasons including medical and psychiatric disorders. This study examined the frequency rate and management of Wernicke's encephalopathy in alcohol-dependent and non-alcohol-dependent patients admitted to a psychiatric hospital. METHODS: Data were retrospectively collected from electronic medical records of psychiatric inpatients admitted to a teaching hospital located in Texas between September 2013 and March 2014. The diagnostic criteria of Caine and colleagues and thiamine dosing strategies were used to identify cases of suboptimal management. RESULTS: A total of 486 charts were reviewed. Nine patients (1.85%) had clinical signs of Wernicke's encephalopathy, and 36 (7%, n = 486) were at a high risk for developing the disorder. None of these patients received adequate doses of parenteral thiamine, and of those who were prescribed thiamine, the majority, including high-risk patients, were prescribed oral thiamine at the traditional dose of 100 mg/d. CONCLUSIONS: The findings suggest that Wernicke's encephalopathy is underdiagnosed and undertreated. Our study also highlights the need for clarifying diagnostic criteria, identifying the risk factors for thiamine deficiency, and improving awareness among physicians about diagnosis, prevention, and adequate treatment of Wernicke's encephalopathy in alcohol-dependent and non-alcohol-dependent patients.

[From thiamine deficiency to Wernicke encephalopathy, unknown pathology]. / Du déficit en thiamine à l'encéphalopathie de Gayet-Wernicke, pathologie méconnue.

The thiamine deficiency and its clinical consequences, especially the Wernicke encephalopathy, are still often not diagnosed. The etiologies are numerous, but the most frequent is the alcohol dependence. The diagnostic is mainly clinical and the treatment must be started as soon as possible by substituting the thiamine with intravenous injection.

Le déficit en thiamine et ses conséquences cliniques, en particulier l'encéphalopathie de Gayet-Wernicke, sont encore très souvent non diagnostiqués. Les étiologies sont multiples, la plus fréquente étant la dépendance à l'alcool. Le diagnostic est avant tout clinique et le traitement doit être débuté le plus rapidement possible en substituant la thiamine par voie intraveineuse.