Wernicke encephalopathy: limitations in a laboratory and radiological diagnosis.

Wernicke encephalopathy is an emergent neurological disorder caused by vitamin B1 (thiamine) deficiency. Here, we present a case of Wernicke encephalopathy in a male patient in his 70s with normal serum thiamine levels and MRI findings on admission. He had a history of heavy alcohol consumption and a gradual decrease in food intake. On arrival at the hospital, his consciousness was impaired which persisted even after glucose replacement. Moreover, horizontal nystagmus and cerebellar ataxia were observed. Head CT scan and MRI revealed no abnormal findings. Further, his serum thiamine level was within the normal range. The patient was clinically diagnosed with Wernicke encephalopathy, and high-dose thiamine therapy was started. Then, his symptoms improved immediately. Thus, in case of clinical suspicion, treatment for Wernicke encephalopathy must be initiated promptly even in patients with normal serum thiamine levels.

[Wernicke-Korsakoff syndrome and other diseases associated with thyamine deficiency]. / Síndrome de Wernicke-Korsakoff y otras patologías asociadas al déficit de tiamina.

Wernicke-Korsakoff syndrome is the best known consequence of thiamine deficiency, frequently associated with patients with chronic and excessive alcohol consumption, but it can be produced by any cause that produces thiamine deficiency. The disease is underdiagnosed so it is essential to have a high clinical suspicion, mainly in patients who do not have alcohol consumption as a risk factor. For this, the diagnosis continues to be eminently clinical, with the difficulty of high clinical variability. Complementary tests are used to support the diagnosis and rule out other causes that can produce similar symptoms, with magnetic resonance imaging being the most cost-effective imaging test. Treatment is based on the administration of thiamine, which should be started early, and parenterally at the appropriate doses, in all patients with compatible symptoms, without waiting to confirm the diagnosis.

Nonalcoholic Wernicke-Korsakoff Syndrome Resulting From Psychosis.

ABSTRACT: Wernicke encephalopathy (WE) results from thiamine deficiency. If undiagnosed or inadequately treated, WE evolves into Korsakoff syndrome (KS). We herein report a case of nonalcoholic Wernicke-Korsakoff syndrome (WKS) that resulted from malnutrition due to psychosis in a 42-years-old male patient. Thiamine deficiency was secondary to severe malnourishment due to poisoning delusions and daily life disorganization in a patient with previously unrecognized schizophrenia. Besides the presence of WE's classic triad of signs, brain magnetic resonance imaging showed also typical thalamic lesions. Furthermore, the patient also presented anterograde and retrograde amnesia, executive dysfunction, and confabulations, compatible with KS being already present. Intravenous treatment with thiamine was given for 37 days. Improvement in cognitive functions and brain imaging alterations was evident. Nevertheless, persistent WKS deficits were present. This case highlights the multiplicity of etiologies of WKS, namely, psychiatric, and its debilitating consequences if not promptly recognized and treated.

Alcohol use disorders and the brain.

A diagnosis of alcohol use disorder is associated with a higher risk of dementia, but a dose-response relationship between alcohol intake consumption and cognitive impairment remains unclear. Alcohol is associated with a range of effects on the central nervous system at different doses and acts on a number of receptors. Acute disorders include Wernicke's encephalopathy (WE), traumatic brain injury, blackouts, seizures, stroke and hepatic encephalopathy. The most common manifestations of chronic alcohol consumption are Korsakoff's syndrome (KS) and alcohol-related dementia (ARD). There is limited evidence for benefit from memantine in the treatment of ARD, but stronger evidence for the use of high-dose parenteral thiamine in the progression of neuropsychiatric symptoms for WE. Accumulating evidence exists for pharmacological treatment in the prevention of hepatic encephalopathy. Rehabilitation of people with ARD may take several years, and requires an approach that addresses physical and psychosocial factors.

A Case of Bariatric Surgery-related Wernicke-Korsakoff Syndrome with Persisting Anterograde Amnesia.

OBJECTIVE: To describe the theoretical and clinical implications of the neuropsychological evaluation of a case of bariatric surgery-related Wernicke-Korsakoff syndrome. METHOD: The patient was a 37-year old, female, bilingual, bachelor's degree educated, Mexican American public relations consultant without preexisting psychiatric, neurological, or substance abuse history. Recovery from laparoscopic sleeve gastrectomy surgery for morbid obesity was complicated by intraabdominal abscess, multibacterial infection, and prolonged nausea and vomiting. About 15 weeks post-surgery she was diagnosed with Wernicke's encephalopathy. She had a positive response to thiamine supplement but was left with persisting self-reported memory problems that were confirmed by family members. Multiple neuroimaging studies were all normal. RESULTS: A neuropsychological evaluation at 14 months post-surgery revealed anterograde amnesia for verbal and visual-perceptual material. There was no clear period of temporally graded retrograde amnesia. Scores on tests of visual-perceptual, language, fine motor, and executive functions were unimpaired. She had awareness of her neurocognitive impairment, but did not exhibit emotional distress. Follow-up neuropsychological evaluation at 17 months showed a similar neurocognitive profile with increased emotional distress. CONCLUSIONS: Her preserved executive functioning is theoretically important as it supports arguments that such impairment in alcohol use-related Korsakoff syndrome derives from the toxic effects of the prolonged misuse of alcohol and not vitamin deficiency. From a clinical perspective, neuropsychological evaluation of thiamine treated, bariatric surgery-related, Wernicke's encephalopathy cases is indicated if there is suspicion of residual memory impairment.

Refeeding Syndrome: An Important Complication Following Obesity Surgery.

BACKGROUND: Refeeding syndrome (RFS) is an important and well-known complication in malnourished patients, but the incidence of RFS after obesity surgery is unknown and the awareness of RFS in obese patients as a postsurgical complication must be raised. We present a case of RFS subsequent to biliopancreatic diversion in a morbidly obese patient. CASE REPORT: A 48-year-old female patient with a BMI of 41.5 kg/m2 was transferred to our hospital due to Wernicke's Encephalopathy in a global malabsorptive syndrome after biliopancreatic diversion. Parenteral nutrition, vitamin supplementation and high-dosed intravenous thiamine supplementation were initiated. After 14 days, the patient started to develop acute respiratory failure, and neurological functions were impaired. Blood values showed significant electrolyte disturbances. RFS was diagnosed and managed according to the NICE guidelines. After 14 days, phosphate levels had returned to normal range, and neurological symptoms were improved. CONCLUSION: Extreme weight loss following obesity surgery has been shown to be associated with undernutrition. These patients are at high risk for evolving RFS, even though they may still be obese. Awareness of RFS as a postsurgical complication, the identification of patients at risk as well as prevention and correct management should be routinely performed at every bariatric center.

Focal thalamic degeneration from ethanol and thiamine deficiency is associated with neuroimmune gene induction, microglial activation, and lack of monocarboxylic acid transporters.

BACKGROUND: Wernicke's encephalopathy-Korsakoff syndrome (WE-KS) is common in alcoholics, caused by thiamine deficiency (TD; vitamin B1) and associated with lesions to the thalamus (THAL). Although TD alone can cause WE, the high incidence in alcoholism suggests that TD and ethanol (EtOH) interact. METHODS: Mice in control, TD, or EtOH groups alone or combined were studied after 5 or 10 days of treatment. THAL and entorhinal cortex (ENT) histochemistry and mRNA were assessed. RESULTS: Combined EtOH-TD treatment for 5 days (EtOH-TD5) showed activated microglia, proinflammatory gene induction and THAL neurodegeneration that was greater than that found with TD alone (TD5), whereas 10 days resulted in marked THAL degeneration and microglial-neuroimmune activation in both groups. In contrast, 10 days of TD did not cause ENT degeneration. Interestingly, in ENT, TD10 activated microglia and astrocytes more than EtOH-TD10. In THAL, multiple astrocytic markers were lost consistent with glial cell loss. TD blocks glucose metabolism more than acetate. Acetate derived from hepatic EtOH metabolism is transported by monocarboxylic acid transporters (MCT) into both neurons and astrocytes that use acetyl-CoA synthetase (AcCoAS) to generate cellular energy from acetate. MCT and AcCoAS expression in THAL is lower than ENT prompting the hypothesis that focal THAL degeneration is related to insufficient MCT and AcCoAS in THAL. To test this hypothesis, we administered glycerin triacetate (GTA) to increase blood acetate and found it protected the THAL from TD-induced degeneration. CONCLUSIONS: Our findings suggest that EtOH potentiates TD-induced THAL degeneration through neuroimmune gene induction. The findings support the hypothesis that TD deficiency inhibits global glucose metabolism and that a reduced ability to process acetate for cellular energy results in THAL focal degeneration in alcoholics contributing to the high incidence of Wernicke-Korsakoff syndrome in alcoholism.

[Wernicke encephalopathy and Korsakoff's psychosis: clinical-pathophysiological correlation, diagnostics and treatment].

Wernicke's encephalopathy and Korsakoff's psychosis are severe unfavorable forms of alcoholic brain damage with poor prognosis. Thiamine deficiency represents a common cause of both diseases. In many cases, Korsakoff's psychosis develops in the outcome of Wernicke's encephalopathy, which, along with the general etiology, lets talk about a single disease - Wernicke-Korsakoff syndrome, acute (usually reversible) stage of which is Wernicke's encephalopathy and a chronic one (often irreversible) is Korsakoff psychosis. The dramatic paradox of Wernicke's encephalopathy is that in most cases it is difficult to detect, but early diagnosed cases are quite easy to cure. Unrecognized and therefore go untreated Wernicke's encephalopathy is a serious threat to the health and lives of patients, worsens the processes of brain aging and increases the risk of Alzheimer's disease in later life. The basic approach to the treatment of Wernicke-Korsakoff syndrome is long-term parenteral administration of thiamine, often in high doses. As an adjuvant means of therapy memantine is considered.

[Diagnostics and treatment of Wernicke-Korsakoff syndrome patients with an alcohol abuse]. / Diagnostik og behandling af Wernicke-Korsakovs syndrom hos alkoholmisbrugere kan optimeres.

Wernicke-Korsakoff syndrome is a condition with high morbidity and mortality and occurs as a consequence of thiamine deficiency. Clinical symptoms are often ambiguous and post-mortem examinations show that the syndrome is underdiagnosed and probably undertreated. There is sparse clinical evidence concerning optimal dosage and duration of treatment. This article reviews the current literature and concludes that all patients with a history of alcohol abuse should be treated with high dosage IV thiamine for an extended period of time, albeit further research is needed.

[Beriberi after bariatric surgery]. / Beriberi na bariatrische chirurgie.

UNLABELLED: Bariatric surgery is in general the only effective treatment for morbid obesity. Bariatric surgery is frequently associated with vitamin and mineral deficiencies which may lead to neurological and other symptoms. We describe a case of severe vitamin B1 (thiamine) deficiency. CASE DESCRIPTION: A 49-year-old man visited the emergency department with acute confusion, muscle weakness in arms and legs and visual impairment after a period of dysphagia and recurrent vomiting. Four months earlier, he had had bariatric gastric sleeve surgery for morbid obesity. Laboratory tests demonstrated that he had vitamin B1 deficiency, in view of which the diagnosis of beriberi and Wernicke encephalopathy was made. Despite normalisation of the vitamin B1 concentration following intravenous supplementation, the muscle strength hardly recovered and the patient developed Korsakov syndrome. CONCLUSION: For this deficiency there is no other treatment than vitamin B1 supplementation. Timely recognition of vitamin deficiencies and pro-active supplementation are essential in order to prevent serious complications following bariatric surgery.

[A new treatment: bariatric surgery; a new complication: Wernicke-Korsakoff encephalopathy]. / Un nouveau traitement: la chirurgie bariatrique; une nouvelle complication: l'encéphalopathie de Wernicke-Korsakoff.

INTRODUCTION: Bariatric surgery has peripheral or central neurological complications in 5-10% of patients. CASE: We report the case of a 39-year-old man with metabolic syndrome who underwent gastric by-pass surgery and then lost 40 kg over a period of 2.5 months. He subsequently developed symptoms suggestive of Wernicke-Korsakoff encephalopathy, secondary to stenosis of the gastrojejunal anastomosis, which led to repeated unreported vomiting. DISCUSSION: This neurological syndrome, linked to thiamine deficiency, can be found with insufficient nutrition after surgery or severe vomiting after bariatric surgery. The complications are essentially peripheral neuropathy and metabolic dysfunctions, including iron and vitamin (B12, D, folate) deficiencies. This case reminds us that bariatric surgery requires close metabolic follow-up with periodic assays of vitamin levels, including thiamine, even when supplementation was prescribed.

Acute Korsakoff-like amnestic syndrome resulting from left thalamic infarction following a right hippocampal hemorrhage.

Korsakoff-like amnestic syndromes have been rarely described following structural lesions of the central nervous system. In this report, we describe a case of acute Korsakoff-like syndrome resulting from the combination of a left anteromedian thalamic infarct and a right hippocampal hemorrhage. We also review the literature relevant to the neuropathology and pathophysiology of Korsakoff syndrome and anterograde amnesia.

A cluster of polyneuropathy and Wernicke-Korsakoff syndrome in a bariatric unit.

BACKGROUND: Wernicke-Korsakoff syndrome and peripheral neuropathy are very uncommon in bariatric surgical practice. The literature indicates that these complications tend to strike patients receiving unbalanced diets or undergoing rapid weight-loss. METHODS: In a retrospective analysis of the initial experience of a bariatric team in the city of Belem, Pará, in northern Brazil, 5 cases were diagnosed in the first year, 4 of them following gastric bypass and the last one after therapy with an intragastric balloon. RESULTS: All episodes followed periods of severe vomiting, which certainly interfered with intake of food as well as of routine vitamin supplements, resulting in severe polyneuropathy and other neurologic manifestions, mostly damaging motility of lower limbs. Therapy consisted of pharmacologic doses of vitamin B1 along with restoration of adequate diet and multivitamin prescriptions. Physical therapy was employed to prevent atrophy and accelerate normalization of muscle strength. All patients responded to this program after variable intervals without significant sequelae. CONCLUSIONS: Thiamine-related neurologic derangements were a cause for much concern and prolonged morbidity in this series, but responded to vitamin B1 replenishment. A high degree of clinical suspicion in bariatric patients and urgent therapeutic intervention whenever postoperative vomiting persists for several days, especially during the first 2-3 months after operation, are the safest approach to these uncommon episodes. It is speculated whether peculiarities in the regional diet of this area in Brazil could have influenced the high incidence of the neurologic aberrations.

Frontal dysfunction and frontal cortical synapse loss in alcoholism--the main cause of alcohol dementia?

Alcoholics often develop personality and behavioural changes, social and personal neglect, confabulation, lack of insight, empathy and emotional control. Such symptoms would increase the risk of engagement in and exposure to acts of violence and criminal activities carrying a risk of physical damage including head trauma and violent death. This was the case in at least 4 of the studied cases. A structural basis for such frontal lobe symptoms was looked for in a forensic material of 18 alcoholics, compared with an age-matched control group with regard to liver disease, brain changes of the Wernicke-Korsakoff type and cortical, especially frontal cortical changes. The salient finding was a consistent pattern of synapse loss in the superior laminae of the frontal cortical area 10 of Brodman in heavy drinkers, not related to liver disease or possible previous mental disease. The synapse loss is more likely related to alcohol, possibly mediated through vitamin B deficiency. Brain stem lesions as a source of additional symptoms cannot be dismissed. This pattern of synapse loss in alcoholism has not been described previously. The cortical changes are closely similar to those found in frontotemporal dementia, and seem to be a plausible main cause of the alcoholic frontal symptomatology and alcoholic dementia.

Wernicke-Korsakoff syndrome following small bowel obstruction.

We report a case of a 64-year-old lady who developed clinical features of Wernicke-Korsakoff syndrome following a laparotomy for small bowel obstruction. Following the operation she developed paralytic ileus and required total parenteral nutrition for one month. A suspected history of average 40 units of weekly alcohol consumption prior to the operation could not be confirmed and the patient did not show any sign of alcohol dependence. Within a few months of treatment with a daily oral dose of thiamine 200 mgs supplemented by multivitamins the patient showed subjective evidence of improvement in confusion, confabulation, and anterograde amnesia, although objective tests showed residual deficits in many areas of cognitive functioning, including immediate and delayed recall of verbal and non-verbal materials, planning and switching of attention.

Rats exposed to acute pyrithiamine-induced thiamine deficiency are more sensitive to the amnestic effects of scopolamine and MK-801: examination of working memory, response selection, and reinforcement contingencies.

Pyrithiamine-induced thiamine deficiency (PTD), which has been used as a model of Wernicke-Korsakoff syndrome (WKS), produces a range of neuropathological and behavioral abnormalities in rodents. The extent of the diencephalic damage produced by this treatment varies from moderate to extreme cell loss. The magnitude of working memory impairment tends to correlate with the degree of neuropathology. In this study a PTD protocol that produces moderate thalamic pathology was used to gain further insight into the neurobehavioral consequences of thiamine deficiency. Towards this end, two distinct manipulations were conducted. First, the differential outcomes procedure (DOP), which correlates specific reinforcers with specific to-be-remembered events, was applied to an operant version of matching-to-position (MTP). This behavioral manipulation was conducted to determine if the DOP would improve memory performance in PTD-treated rats, demonstrating some intact cognitive functions. Additionally, to assess the functional integrity of the cholinergic and glutamatergic systems, normal and PTD-treated rats were administered i.p. injections of scopolamine and MK-801. It was found that the DOP enhanced memory, but not acquisition performance, in both normal and PTD-treated rats. Furthermore, when administered scopolamine, but not MK-801, rats trained with the DOP continued to outperform rats trained with a non-differential outcomes procedure (NOP). However, PTD-treated rats, regardless of training procedure (DOP, NOP), were more disrupted by the 'amnestic' effects of both scopolamine and MK-801. The differential sensitivity of treatment groups to the amnestic effects of scopolamine and MK-801 reveals insights into the neurochemical correlates of memory processes and WKS.