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Query: UMLS:C0004134 (ataxia)
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Beriberi is a disease caused by thiamine (vitamin B1) deficiency. Peripheral and central nerve involvement causes psychosis and memory loss as well as cardiocirculatory effects. We report the case of a 35-year-old woman 8 weeks pregnant who came to the emergency department after bouts of nausea and vomiting over a period of 6 days, with intolerance of both solids and liquids. The initial diagnosis of gastroenteritis was later changed to hyperemesis gravidarum. Episodes of vomiting and nausea continued 48 hours after admission, accompanied by vertical nystagmus, ataxia, and diminished osteotendinous reflexes. Evaluation of the clinical picture confirmed vitamin B1 deficiency, leading to a diagnosis of Wernicke-Korsakoff syndrome. Symptoms improved with thiamine therapy but did not entirely disappear. The patient was admitted for elective cesarean section at 37 weeks' gestation. Examination revealed neurological involvement (horizontal and vertical nystagmus) and general anesthesia was therefore chosen to assure adequate hemodynamic control given the possibility of cardiocirculatory alteration.
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PMID:[Anesthetic management for elective cesarean section for a woman with beriberi]. 1739 Jun 94

Achalasia is an incurable neuromuscular disorder of the esophagus, resulting from destruction of the esophageal myenteric plexus. This leads to aperistalsis and failure of the lower esophageal sphincter to relax after swallowing. Symptoms of achalasia are gradual in onset and include dysphagia, regurgitation, and weight loss. Severe malnutrition can ensue. Wernicke's encephalopathy (WE) is a serious, potentially fatal, neurologic disorder caused by thiamine deficiency (vitamin B(1)), classically described as presenting with a triad of ocular abnormalities, ataxia, and confusion. The incidence is uncertain, and many cases likely go unrecognized. It is usually diagnosed in the alcoholic population. We describe its onset after the successful surgical treatment of achalasia.
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PMID:Wernicke's encephalopathy after laparoscopic cardiomyotomy for achalasia. 1759 43

First described in 1881, Wernicke's encephalopathy continues to be an unrecognized and often misunderstood disease. The cause of Wernicke's encephalopathy is thiamine deficiency as a result of any nutritionally deficient state, though many physicians erroneously consider this disease to be confined only to alcoholics. Unfortunately, the syndrome is most often recognized only on autopsy, especially among nonalcoholics. Despite advances in magnetic resonance imaging, Wernicke's encephalopathy remains primarily a clinical diagnosis. The common clinical findings include mental status changes, ocular dysfunction, and gait ataxia. Additional signs may be present, or 1 or more of the common findings may be absent. Treatment mandates timely intravenous thiamine therapy, for which the optimum dosage remains controversial. This review traces the history of Wernicke's encephalopathy from the first description to our current understanding of the disease and includes many of the misconceptions, myths, and controversies that surround this disease. Emergency physicians need to be well versed in the varied presentation of Wernicke's encephalopathy because most of these patients will present to the emergency department and are oftentimes unrecognized. Further, physician knowledge of this disease is vital because the failure to diagnose results in severe neurologic morbidity and possible mortality, but the treatment is safe and effective.
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PMID:Myths and misconceptions of Wernicke's encephalopathy: what every emergency physician should know. 1768 41

A 42-year-old woman presented with lowered consciousness, ataxia and ophthalmoplegia; MRI of the brain revealed abnormalities compatible with Wernicke encephalopathy (thiamine deficiency).
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PMID:[Diagnostic image (360). A woman with lowered consciousness]. 1832 12

Wernicke's encephalopathy (WE) is one of the potential complications of obesity surgery. It is an acute neuropsychiatric syndrome resulting from thiamine deficiency often associated with repeated vomiting. The classic triad is frequently reported in these patients (optic neuropathy, ataxia and confusion), associated with uncommon features. Cerebral impairment affects the dorsal medial nucleus of the thalamus and the periaqueductal grey area, appearing on MRI, as hyperintense signals on T2, Flair and Diffusion weighted imaging. Early diagnosis and parenteral thiamine are required to decrease morbidity and mortality. We report a case of WE and Korsakoff's syndrome in a young obese patient after subtotal gastrectomy, who still has substantial sequelae. The contribution of MRI with diffusion-weighted imaging is illustrated. The interest of nutritional supervision in the first weeks and preventive thiamine supplementation in case of repeated vomiting are of particular importance in these risky situations.
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PMID:[Wernicke encephalopathy after subtotal gastrectomy for morbid obesity]. 1855 79

Wernicke's encephalopathy (W.E.), a potentially reversible condition caused by thiamine deficiency, is usually suspected in the setting of chronic alcoholism and might not be recognized when associated with other conditions. We describe a young pregnant woman who presented with rapidly evolving ataxia, diplopia and irrelevant speech following repeated vomiting. Characteristic brain MRI and rapid response to thiamine suggested that she had W.E. possibly due to hyperemesis gravidarum. A high index of suspicion is required, since delayed or lack of treatment may lead to high morbidity and mortality.
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PMID:Hyperemesis-gravidarum-induced Wernicke's encephalopathy: serial clinical, electrophysiological and MR imaging observations. 1947 64

Wernicke encephalopathy is caused by thiamine deficiency in the central nervous system, and is defined by the triad of confusional symptoms, ocular alterations and ataxia. Some other factors may also predispose alcoholic patients to this deficiency. We report two patients with hyperglicaemia and ketoacidosis due to diabetes mellitus decompensation and chronic alcoholism who developed Wernicke encephalopathy before their hospital admission. The outcome was successful after intravenous thiamine administration and insulinotherapy. The presence of Wernicke encephalopathy in alcoholics with diabetic ketoacidosis, suggests that metabolic decompensation is essential in the onset of the disease.
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PMID:Wernicke encephalopathy in alcoholics with diabetic ketoacidosis. 1957 57

A 52-year-old woman was admitted to the hospital because of appetite loss, unsteadiness, psychogenic symptoms, ataxia, and consciousness disturbance as a result of the ingestion of a diet restricted to only carbohydrates for a long term. Laboratory examination indicated the presence of pancytopenia with macrocytic anemia; further, decreased vitamin B1 and B12 levels were detected in her serum. Magnetic resonance imaging fluid attenuated inversion recovery (FLAIR), revealed high-signal intensity in the bilateral corpus striatum, third ventricle circumference, and cerebellar cortex. Thereafter, she received drip infusion that did not include vitamin B1 or B12 and subsequently suffered a cardiac arrest due to the aggravation of cardiac insufficiency; consequently, she was transferred to our hospital. Upon admission the patient was diagnosed to have obvious cardiomegaly with pleural effusion; further, a negative T-wave was obtained on the electrocardiogram. A diagnosis of beriberi heart disease was made because of thiamine deficiency. She was treated by thiamine administration, following which the cardiac symptoms improved immediately. Various neurological symptoms caused by encephalopathy, peripheral neuropathy and subacute combined spinal cord degeneration improved by treatment with thiamine and cyanocobalamine administration; however, some of these symptoms still remained. General awareness of the fact that neurological symptoms can be caused by vitamin deficiency is essential.
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PMID:[Case of Wernicke's encephalopathy and subacute combined degeneration of the spinal cord due to vitamin deficiency showing changes in the bilateral corpus striatum and cardiac arrest due to beriberi heart disease]. 1980 6

A 75-year-old man with diabetic nephropathy treated with hemodialysis visited to a medical office because of slight fever, and received intravenous glucose infusion without any vitamins. Thereafter, he noticed gait disturbance and began to tell inconsistent stories. He was admitted to our hospital due to aggravation of these symptoms. On admission, he was disoriented and not able to sit by himself because of severe truncal ataxia without weakness. He had also gaze direction nystagmus. Based on clinical features, we considered him as having Wernicke's encephalopathy (WE) and treated him with 100 mg thiamine per day. The thiamine supply diminished these symptoms soon. Plasma thiamine level prior to the administration was 7 ng/ml, which confirmed the diagnosis. MRI did not disclose any abnormalities frequently seen in WE. WE is a life-threatening disease, and 'early detection, early cure' is important for recovering without sequelae. The thiamine deficiency is often seen in dialysis patients because of dietary restrictions as well as its loss during dialysis. This case gives us the caution; when hemodialysis patients present acute/subacute gait disturbance and/ or abnormal mental state, we should consider WE. Furthermore, high-risk patients, such as elderly patients under hemodialysis may need some supplement including thiamine even at preclinical stage.
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PMID:[Wernicke encephalopathy in a non-alcoholic patient with diabetic nephropathy under hemodialysis]. 2059 67

Thiamine (vitamin B(1)) is an essential component of a number of metabolic pathways and thiamine deficiency results in a progressive encephalopathy in both humans and animals. Confirming thiamine deficiency is problematic and relies on demonstrating reduced red blood cells transketolase activity, or indirect methods including urinary organic acid analysis and dietary analysis. The characteristic and selective vulnerability of different brain regions in carnivores has been demonstrated by magnetic resonance (MR) imaging in the dog and cat as an aid to diagnosis. A 2-year-old, female, domestic shorthair cat was presented with an acute onset of seizures and ataxia. MR imaging was consistent with thiamine deficiency and supplementation resulted in a progressive clinical improvement. Repeated MR imaging 4 days after starting thiamine supplementation revealed near complete resolution of the MR abnormalities. Repeated MR imaging following appropriate therapy may be useful to further confirm thiamine deficiency.
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PMID:Thiamine deficiency in a cat: resolution of MRI abnormalities following thiamine supplementation. 2067 25


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