Wernicke's Encephalopathy in Type 2 Achalasia: Case Report and Literature Review.

Achalasia is primarily a smooth muscle motility disorder of the esophagus driven by aberrant peristalsis and failure of sphincter relaxation. Notably, achalasia is a heterogeneous disease with primarily 3 possible pattern subtypes. According to the review of current cases and literature regarding achalasia, patients primarily present with dysphagia, usually to solids and, if progressed, to solids and liquids. Rarely, untreated achalasia may result in thiamine deficiency and present as Wernicke-Korsakoff syndrome (WKS). This acute neurologic condition primarily affects the central and peripheral nervous system and is known by the triad of ataxia, ophthalmoplegia, and confusion. Individuals who present with WKS typically have a notable history of chronic alcohol abuse with decreased thiamine intake and metabolism. Although less common, individuals with WKS may have a pertinent history of starvation, anorexia nervosa, and malnutrition. This case highlights a unique presentation of Wernicke's encephalopathy (WE) in a 30-year-old woman with severe type II achalasia complicated by a 60-pound weight loss in a span of 2 months. According to our literature review, there have only been 2 previously reported cases of severe achalasia leading to the development of WE. Considering the limited number of case reports available, WE must be in the differentials in patients with underlying achalasia, and our case report highlights this unusual presentation with corresponding brain imaging and manometry testing.

Diagnosis of Wernicke's Encephalopathy in Patients With a Psychiatric History: A Case Series and Literature Review.

Objective: To review the literature on diagnostic criteria and management of Wernicke's encephalopathy (WE) and its application in psychiatric populations.Evidence Review: A PubMed MEDLINE search was conducted in February 2022 and updated in April 2022 for articles published in English between 2012 and 2022 describing clinical findings and treatment of WE. Reference lists of included articles and treatment guidelines were reviewed. Search terms included Wernicke's encephalopathy, thiamine, thiamine administration dosage, and prescribing. Additionally, 2 cases with co-occurring psychiatric and WE manifestations were selected from the consult-psychiatry service between July and December 2021. Pertinent clinical findings and management were extracted from the literature and compared with that of the cases.Findings: 113 titles were retrieved; 39 studies were excluded. Exclusion criteria included studies done in patients < 18 years old, animal studies, studies with no abstract, and studies with no clinical discussion. Twelve articles were added from the grey literature. Eighty-six articles were included in the review. Only 7 studies discussed WE in psychiatry. The results reaffirm the lack of information regarding diagnosis and treatment of WE in the general population. Clinical cases suggest further increased risk in the psychiatric population.Conclusion and Relevance: Evidence suggests underdiagnosis and undertreatment of WE in general populations and psychiatric patients at increased risk for malnutrition. An interdisciplinary approach improves time to diagnosis and management of comorbidities. We recommend dosing guided by clinical response; however, evidence suggests lengthier and higher doses of thiamine may be needed. Addition of neuroleptics may be necessary for management of psychiatric symptoms and relapse prevention.Prim Care Companion CNS Disord 2023;25(4)22nr03447. Author affiliations are listed at the end of this article.

The importance of thiamine (vitamin B1) in humans.

Thiamine (thiamin, B1) is a vitamin necessary for proper cell function. It exists in a free form as a thiamine, or as a mono-, di- or triphosphate. Thiamine plays a special role in the body as a coenzyme necessary for the metabolism of carbohydrates, fats and proteins. In addition, it participates in the cellular respiration and oxidation of fatty acids: in malnourished people, high doses of glucose result in acute thiamine deficiency. It also participates in energy production in the mitochondria and protein synthesis. In addition, it is also needed to ensure the proper functioning of the central and peripheral nervous system, where it is involved in neurotransmitter synthesis. Its deficiency leads to mitochondrial dysfunction, lactate and pyruvate accumulation, and consequently to focal thalamic degeneration, manifested as Wernicke's encephalopathy or Wernicke-Korsakoff syndrome. It can also lead to severe or even fatal neurologic and cardiovascular complications, including heart failure, neuropathy leading to ataxia and paralysis, confusion, or delirium. The most common risk factor for thiamine deficiency is alcohol abuse. This paper presents current knowledge of the biological functions of thiamine, its antioxidant properties, and the effects of its deficiency in the body.

Concomitant occurrence of Wernicke's encephalopathy and sarcopenia due to vitamin D depletion in patients with alcohol use disorder: a case report.

Individuals with alcohol use disorder frequently suffer from vitamin D deficiency, in addition to deficiencies in vitamins B12, folic acid and B1. This is due to inadequate dietary intake and behavioural changes. Each of these deficiencies results in different clinical symptoms. Subacute spinal cord degeneration, together with radicular and sensorimotor peripheral neuropathy, arises from B12 vitamin and folic acid deficiencies. B1 vitamin deficiency leads to Wernicke's encephalopathy, which can include the classical triad of symptoms (i.e. cognitive changes, ataxia and ophthalmoplegia). Sarcopenia is a consequence of a long-term deficiency of vitamin D. This current case report describes a 43-year-old female patient with alcohol use disorder who complained of dizziness, postural disturbance and episodes of intermittent paraesthesia. She was subsequently shown to have concomitant Wernicke's encephalopathy and sarcopenia due to vitamin D deficiency. This case report presents the diagnostic process undertaken to exclude conditions related to ataxia and paraparesis other than vitamins D and B1 deficiencies. It also emphasizes the importance of concomitant replacement of the depleted vitamins because the vitamin deficiency may occur simultaneously, which causes the accompanying manifestations of several clinical syndromes.

A Case of Sensorineural Hearing Loss in Wernicke Encephalopathy.

Extremely rare cases of Wernicke encephalopathy (WE) can involve sensorineural hearing loss (SNHL). Here, we present a 46-year-old female with SNHL and clinical and radiologic suspicion for WE. After initiating thiamine therapy, the patient experienced robust improvement. Laryngoscope, 133:1492-1494, 2023.

[Neurological Symptoms Associated With Vitamin Deficiency and Excess: Past and Present Findings].

Vitamin deficiencies and excesses are known to cause various types of brain or peripheral nerve injuries. Improved nutritional status and dietary patterns have led to reduced prevalence, and at least some of these conditions are rare in recent times. However, delayed diagnosis (For example, in patients with Wernicke's encephalopathy) may worsen patient prognosis even in current times. Recent studies have reported an association between cognitive function and some vitamins. In this article, we summarize past and present research focused on neurological symptoms that occur secondary to vitamin deficiencies and excesses.

Wernicke's encephalopathy in advanced cancer.

This 54-year-old woman with metastatic breast cancer presented with severe confusion, incontinence and self-neglect. MRI suggested Wernicke's encephalopathy (WE) without any history of alcohol misuse. The patient's symptoms seemed to not respond to acute treatment. Cancer progression was assumed to be causing her condition and she was discharged to a nursing home with community palliative care team follow-up. This case demonstrates the natural course of cancer-associated WE and eventual improvement with thiamine therapy. It also illustrates a role for specialist palliative care follow up for patients discharged to a nursing home for expected end-of-life care.

Wernicke Encephalopathy Mimicking MELAS.

OBJECTIVES: a stroke-like lesion, the morphological equivalent of a stroke-like episode and the hallmark of mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome, have not been reported as manifestations of thiamine deficiency. CASE REPORT: a 62-year-old man with a history of chronic alcoholism was admitted after a series of epileptic seizures. Upon waking up from the coma, he presented with disorientation, confusion, confabulation, psychomotor agitation, aggressiveness, right hemianopsia, aphasia, and right hemineglect over weeks. Electroencephalography showed a questionable focal status epilepticus over the left hemisphere, responsive to lorazepam and oxcarbazepine. Follow-up electroencephalographies no longer recorded epileptiform discharges. Cerebral magnetic resonance imaging (MRI) revealed T2-/diffusion weighted imaging (DWI) hyperintensity in the left occipito-temporal region that was not congruent to a vascular territory which persisted for at least nine weeks. Since a lactate-peak could be seen in this lesion by magnetic resonance-spectroscopy, this was interpreted as a stroke-like lesion. Since thiamine was reduced, the stroke-like lesion was attributed to thiamine deficiency after the exclusion of differential diseases, including MELAS and status epilepticus. The patient's behavioural and cognitive dysfunctions largely resolved upon vitamin-B1 substitution. CONCLUSIONS: the case suggests that thiamine deficiency presumably causes mitochondrial dysfunction with cerebrospinal fluid lactic acidosis and a stroke-like lesion mimicking MELAS syndrome. It should be further studied whether nutritional deficits, such as thiamine deficiency, could give rise to secondary stroke-like lesions.

Clinical analysis of alcoholic pellagra: A single-center retrospective study.

BACKGROUND: Pellagra caused by niacin deficiency in alcoholics can be easily misdiagnosed because of similar symptoms to other alcohol-related diseases and the lack of the classical triad of signs. AIM: This study aimed to define the clinical presentation of alcoholic pellagra for early diagnosis and timely treatment. METHODS: The clinical data of 16 alcohol-dependent patients who had pellagra and treated in our hospital from January 2002 to December 2019 were retrospectively analyzed. The local medical ethics committee approval (Medical Ethics Committee of Affiliated Hospital of XuZhou Medical University, XYFY2020-KL247-02) for this study has been obtained. RESULTS: The main complaints of the 16 patients were skin lesions (six cases), diarrhea (six cases), and mental disorders (four cases). Then, 13 cases had typical skin lesions, and 3 patients had a full spectrum of diarrhea, dementia, and dermatitis (3D). In terms of the main diagnosis, 2 patients had pellagra and Wernicke's encephalopathy, 3 patients had pellagra and alcohol-withdrawal syndrome, and the other patients had pellagra. After sufficient amounts of niacin and multivitamin B were given, clinical symptoms improved rapidly, and no sequelae were observed during follow-up. CONCLUSIONS: Pellagra is rarely manifested as a full 3D spectrum, with only one or two characteristics, which lack diagnostic specificity, especially in individuals with alcoholism. Physicians should maintain a high degree of suspicion of niacin deficiency in alcoholics. Alcohol-dependent patients with pellagra may be accompanied by complications of Wernicke's encephalopathy and alcohol-withdrawal syndrome. Prompt identification and timely treatment with a sufficient amount of niacin in combination with other vitamins and a certain amount of Zn can achieve a good prognosis of pellagra.

Wernicke encephalopathy with epileptic seizures during pregnancy.

A 22-year-old woman was admitted to the emergency department with headache, dizziness, and numbness on the left side of the body. Neurologic examination revealed gaze-evoked nystagmus. Cranial magnetic resonance imaging, venous and arterial magnetic resonance angiography were normal. Generalized epileptogenic activity was observed in the electroencephalography. The erythrocyte thiamine transketolase levels were found to be 13 u/L. She received intravenous thiamine. On discharge, she had no neurologic sequelae. The diagnosis of Wernicke encephalopathy in our patient is based on increased thiamine requirement due to pregnancy, nystagmus, low transketolase level, and reversal of symptoms after thiamine replacement. .

What is the optimum thiamine dose to treat or prevent Wernicke's encephalopathy or Wernicke-Korsakoff syndrome? Results of a randomized controlled trial.

BACKGROUND: The primary cause of Wernicke-Korsakoff syndrome (WKS) is thiamine deficiency, and more than 90% of cases are reported in alcohol-dependent patients. While observational studies show parenteral thiamine administration drastically reduced WKS-related mortality, relevant treatment trials have never been conducted to determine the optimum thiamine dose. METHODS: Two double-blind, parallel groups, randomized controlled trials (RCTs) were conducted to determine the optimal thiamine dose required for (1) the prevention of Wernicke's encephalopathy (WE), the acute phase of WKS, in asymptomatic but "at-risk" alcohol misuse patients (Study 1) and (2) the treatment of WE in symptomatic alcohol misuse patients (Study 2). Each study had a dosage regimen comprising three parenteral thiamine doses that were allocated at a ratio of 1:1:1. Study 1: Asymptomatic At-Risk patients (N = 393) received either 100 mg daily, 100 mg thrice daily, or 300 mg thrice daily, for 3 days. Study 2: Symptomatic patients (N = 127) received either 100 mg thrice daily, 300 mg thrice daily, or 500 mg thrice daily, for 5 days. Cognitive function was the primary outcome, assessed using the Rowland Universal Dementia Assessment Scale, two Cogstate subtests, and an adapted Story Memory Recall test. Secondary analyses examined differences in neurological function (ataxia, oculomotor abnormalities, and confusion) at follow-up. RESULTS: No significant differences were observed between any of the dosage conditions for either Study 1 or Study 2 on cognition or neurological functioning. This real-world study found that having a clinically unwell target population with high comorbidity and multiple presentations, coupled with challenges in cross-cultural assessment is likely to complicate RCT findings. CONCLUSIONS: The results of this study showed no clear benefit of high dose thiamine over intermediate or lower doses of thiamine, over the time intervals examined, for the treatment and prevention of cognitive and neurological abnormalities related to WKS. Several study limitations temper the interpretation of these findings. Nevertheless, the absence of conclusive evidence for the superiority of high-dose thiamine supports a recommendation for patient-specific treatment, while ensuring that the potential impact of other biochemical factors (e.g., magnesium and other B vitamin deficiencies) are considered and corrected if necessary.

Identification of Wernicke Encephalopathy in a Patient Presenting With Altered Mental Status and Dehydration.

INTRODUCTION: Providing glucose before thiamine can cause or exacerbate Wernicke encephalopathy, a potentially life-threatening condition associated with a variety of neurological impairments. CASE PRESENTATION: An emaciated, middle-aged woman with a longstanding history of alcohol abuse and an undifferentiated seizure disorder presented to a local emergency department with altered mental status of unknown duration. Initial labs showed signs of acute kidney injury and she could not tolerate oral intake. Overnight, dextrose-containing maintenance fluids were started. The next day, she had an acute deterioration of mental status. Empiric therapy for Wernicke encephalopathy was begun, resulting in resolution of most of her symptoms over a matter of days. DISCUSSION: It is generally recommended to administer thiamine treatment prior to glucose in patients with suspected thiamine deficiency. The Caine criteria can assist in the decision to start empiric treatment to prevent delays in thiamine therapy. CONCLUSION: Wernicke encephalopathy is a disease with high morbidity that is usually treated with the generally benign therapy of thiamine. Given the risk of harm of untreated Wernicke encephalopathy and the benign nature of treatment, clinicians should have a low threshold to provide thiamine therapy.

What is the Korsakoff syndrome? - a paper in tribute to Prof Alwyn Lishman.

INTRODUCTION: Alwyn Lishman was interested in how memory research could be applied to clinical psychiatry. After a brief review of his major contributions, this paper will focus on his research on the alcoholic Korsakoff syndrome. It will consider how his findings relate to contemporary debates, particularly on how the syndrome should be defined, and its relationship to broader alcohol-induced cognitive impairments. METHODS: A review of the contribution of Alwyn Lishman, Robin Jacobson and colleagues to our knowledge of Korsakoff's syndrome, together with a review of the pertinent recent literature. RESULTS: Lishman and colleagues followed earlier authors in defining the Korsakoff syndrome in terms of disproportionate memory impairment, but they also noted a variable degree of IQ, frontal-executive, and timed visuo-spatial impairment in their cases. More recent authors have included such features in their definitions of the syndrome. Lishman also argued for a specific "alcoholic dementia". The present paper argues that recent definitions of the Korsakoff syndrome confound its core and associated features, and also fail to recognise the multifactorial basis of alcohol-related brain damage. CONCLUSIONS: Korsakoff's syndrome is best defined in terms of disproportionate memory impairment, and more widespread cognitive impairment is best encompassed within "alcohol-related brain damage".

Can the Ketogenic diet lead to Wernicke's encephalopathy?

BACKGROUND: Wernicke's encephalopathy (WE), a neurological disorder due to the deficiency of thiamine, is often underdiagnosed in the pediatric population. The classic triad of mental status changes, oculomotor abnormality, and ataxia is observed in only 16-21% of all pediatric presentations. Wernicke's is most often associated with alcohol dependence, but also malignancy, parenteral nutrition, and gastrointestinal (GI) malformations. The correlation between following a Ketogenic diet and acquiring Wernicke's, however, has not previously been reported. CASE REPORT: A 16-year-old previously healthy male presented with an eleven-day history of neurological deficits and GI upset. The patient had recently lost one hundred pounds while following a "ketogenic" diet. He was subsequently diagnosed with Wernicke's, received intravenous thiamine, and was transferred to the inpatient rehabilitation, where he received extensive diet education. After making significant functional improvement, he was discharged home. RELEVANCE: This case illustrates the importance of including Wernicke's in the differential diagnosis when a pediatric patient presents with neurological deficits after rapid weight loss. Wernicke's encephalopathy may be fatal in the pediatric population, therefore, it must be treated immediately if clinically suspected. Children presenting with Wernicke's would benefit from early intervention, intensive inpatient rehabilitation, and comprehensive education regarding the role of food and exercise on weight loss and health.

A rare case of Wernicke encephalopathy in stage IV gastric cancer.

A 38-year-old male with stage IV gastric adenocarcinoma presented with abdominal pain, nausea, bilious non-bloody vomiting, and lethargy. He was found to have an ileus and was treated appropriately with bowel rest and nasogastric tube decompression. However, the patient was also noted to have confusion and nystagmus. While he was abstinent from alcohol for 20 years, he was found to have Wernicke Encephalopathy (WE) as a result of malnutrition from the underlying malignancy.