Wernicke's encephalopathy in a patient with non-Hodgkin's lymphoma post-Autologous HSCT

SUMMARY Wernick's Encephalopathy (WE) is an acute neuropsychiatric syndrome caused by thiamine deficiency post hematopoietic stem cell transplant (HSCT). WE is associated with high mortality and morbidity rates, but due to its rare occurrence, it is rarely considered in patients submitted to this procedure. Considering that, the manuscript reports the clinical characteristics and the possible factors that predisposed the occurrence of WE in a patient with non-Hodgkin's lymphoma post-Autologous HSCT. We conclude that WE should be considered in patients submitted to autologous HSCT associated with prolonged use of TPN and malnutrition.

Thiamine deficiency contributes to synapse and neural circuit defects

BACKGROUND: The previous studies have demonstrated the reduction of thiamine diphosphate is specific to Alzheimer's disease (AD) and causal factor of brain glucose hypometabolism, which is considered as a neurodegenerative index of AD and closely correlates with the degree of cognitive impairment. The reduction of thiamine diphosphate may contribute to the dysfunction of synapses and neural circuits, finally leading to cognitive decline. RESULTS: To demonstrate this hypothesis, we established abnormalities in the glucose metabolism utilizing thiamine deficiency in vitro and in vivo, and we found dramatically reduced dendrite spine density. We further detected lowered excitatory neurotransmission and impaired hippocampal long-term potentiation, which are induced by TPK RNAi in vitro. Importantly, via treatment with benfotiamine, Aß induced spines density decrease was considerably ameliorated. CONCLUSIONS: These results revealed that thiamine deficiency contributed to synaptic dysfunction which strongly related to AD pathogenesis. Our results provide new insights into pathogenesis of synaptic and neuronal dysfunction in AD.

Hallazgos por RM en la encefalopatía de Wernicke aguda: a propósito de un caso / MRI findings in acute Wernicke's encephalopathy: a case report

Si bien la enfermedad de Wernicke es un desorden neurológico poco frecuente que se produce por el déficit de tiamina, cuando ocurre es una emergencia médica, ya que la ausencia de tratamiento conduce a una psicosis de Korsakoff e, incluso, a la muerte. Presentamos el caso de un paciente en el cual la resonancia magnética (RM), que tiene imágenes características, fue fundamental para el diagnóstico precoz...

Vitamina B1

1. Vitamina B1 é essencial para o metabolismo dos hidratos de carbono. 2. O homem depende da ingestão de alimentos para suprir suas necessidades de tiamina. 3. Pequenas deficiências de vitamina B1 podem ser repostas através de alimentação adequada. 4. Grandes deficiências de tiamina ocorrem no alcoolismo e grandes déficits nutricionais.5. Carência crônica de tiamina pode levar a insuficiência cardíaco-congestiva.

A Case of Wernicke's Encephalopathy Following Fluorouracil-based Chemotherapy

The pyrimidine antimetabolite 5-fluorouracil (5-FU) is a chemotherapeutic agent used widely for various tumors. Common side effects of 5-FU are related to its effects on the bone marrow and gastrointestinal epithelium. Neurotoxicity caused by 5-FU is uncommon, although acute and delayed forms have been reported. Wernicke's encephalopathy is an acute, neuropsychiatric syndrome resulting from thiamine deficiency, and has significant morbidity and mortality. Central nervous system neurotoxicity such as Wernicke's encephalopathy following chemotherapy with 5-FU has been reported rarely, although it has been suggested that 5-FU can produce adverse neurological effects by causing thiamine deficiency. We report a patient with Wernicke's encephalopathy, reversible with thiamine therapy, associated with 5-FU-based chemotherapy.

Deficiência de tiamina como causa de cor pulmonale reversível / Thiamin deficiency as a cause of reversible cor pulmonale

A deficiência de tiamina pode apresentar quatro formas clássicas de apresentação clínica: polineuropatia periférica, anorexia e fraqueza muscular (beribéri seco); insuficiência cardíaca de alto débito com sinais congestivos (beribéri úmido); beribéri associado ao choque (Shoshin beribéri) e encefalopatia de Wernicke. Neste relato, descrevemos quadro sugestivo de hipertensão pulmonar grave e cor pulmonale, com estase jugular, hepatite congestiva e edema generalizado, que apresentou reversão completa após administração de tiamina.

Thiamine deficiency may present four classic clinical forms: peripheral polyneuropathy, anorexia and muscular weakness (dry beriberi); high output heart failure with signs of congestion (wet beriberi); beriberi associated with shock (Shoshin beriberi) and Wernicke's encephalopathy. In this report we describe a picture that is suggestive of severe pulmonary hypertension and cor pulmonale, with jugular stasis, congestive hepatitis and generalized edema that reversed completely after the administration of thiamine.

Influência da terapia com diuréticos nos níveis séricos de tiamina em pacientes com insuficiência cardíaca / Influence of diuretic therapy on thiamin blood levels in patients with heart failure

Fundamento - A importância de micronutrientes como tiamina há muito é conhecida, visto que sua deficiência associa-se com insuficiência cardíaca (IC) de alto débito. Vários trabalhos mostram a influência dos diuréticos nos níveis de tiamina. Objetivos - Avaliar se o uso de furosemida isolada ou associada a espironolactona (espiro) determina níveis de tiamina diferentes em pacientes (pc) com IC. Verificar se a tiamina é influenciada por fatores nutricionais, comorbidades e outros fármacos. Métodos - Estudo em duas etapas: Estudo 1 - análise transversal (uso prévio de furosemida isoladamente ou em associação com espiro) de 22 pc com IC (classes III/IV da NYHA) divididos em: grupo I - com epiro (n=11) e grupo II - sem espiro (n=11); Estudo 2 - ensaio clínico randomizado, aberto, com três grupos paralelos de 53 pc com IC divididos em grupo "sem diuréticos" (n=15), "com diurético" (n=15) (uso de furosemida até o dia 90 e depois associar espiro até o dia 180) e "com espiro" (n=23) (usar furosemida e espiro desde a visita inicial até o dia 180). Os pc realizaram três visitas: inicial, em 90 dias e 180 dias, sendo coletadas amostras de sangue para tiamina e outros exames, assim como análise clínica. Determinamos os níveis de tiamina por recombinação de apoenzima e por cromatografia líquida de alta performance. Utilizamos testes t de Student, qui-quadrado, Mann-Whitney, Kruskal-Wallis, Spearman e como a variância foi cerca de três vezes maior que a estimada, usamos a simulação robusta de Monte Carlo. Resultados: no estudo 1, os grupos I e II eram similares em relação à ingesta alimentar, doses diárias de furosemida (I=110,9 +- 30,2 vs II=105,5 +-26,9mg; p>0,05) e dados demográficos. Os pacientes do grupo I mostraram níveis maiores de tiamina (277,2+-89,8), comparados aos do grupo II (154,7+-35,7) (p<0,001). Nenhuma outra variável esteve associada com a tiamina. No estudo 2, os grupos também eram semelhantes quanto às características clínicas e laboratoriais...

Background: The importance of micronutrients such as thiamine known since its deficiency is associated with the development of high-output heart failure (HF). Several studies show the influence of diuretic therapy in thiamine blood levels. Objectives: assess whether the use of furosemide alone or in combination with spironolactone (spiro) can determine different thiamine blood levels in patients (pt) with HF. To determinat whether thiamine blood levels were influenced by nutritional factors, comorbidities and other drugs. Methods: Study 1 - cross-sectional analysis (previous use of furosemide alone or with spiro) of 22 HF pt (NYha class III/IV) divided in two groups: I - with spiro (n=11) and II - without spiro (n=11); Study 2 - randomized, open-label, with three parallel groups of 53 HF pt divided into group "without diuretics" (n=15), "with diuretic" (n=15) (use of furosemide until day 90 and then adding spiro from day 90 until day 180) and "with spiro" (n=23) (using furosemide and spiro through all study). Patients were scheduled to three visits: day 0, day 90 and day 180, and blood samples were collected for thiamine and other tests, as well as clinical analysis. Thiamine levels were determined using an apoenzyme recombination method and high-performance liquid chromatography. The tests used were t-Student, chi-square, Mann-Whitney, Kruskal-Wallis, Spearman correlation, and as the variance was about three times higher than estimated, we used a robust Monte Carlo simulation. Results: in study 1, group I and II were similar regarding food intake, daily furosemide doses (I=110.9+-30.2 vs II=105.5+-26.9 mg; p>0.05) and demographics. Pts in group I (277.2+-89.8) showed significantly higher thiamine levels when compared to pts in group II (154.7+-35.7) (p<0.001). No other variables were associated with thiamine. In study 2, the groups were also similar regarding clinical and laboratory assessments. We found no statistical differences in thiamin blood levels...

Encefalopatía de Wernicke / Wernick's encephalopathy

La Encefalopatía de Wernicke (EW) es un síndrome neurológico reversible causado por un déficit de tiamina (vitamina B). Se le adjudica una frecuencia de 0,8 a 2,8 por ciento en la población general la que se eleva hasta un 12 por ciento si se considera sólo los pacientes alcohólicos. Su principal factor etiológico es el alcoholismo; sin embargo, se han descrito casos asociados a malnutrición de diversos orígenes. Clásicamente se caracteriza por la tríada de signos oculares, ataxia y confusión; pero esta asociación tiene una muy baja frecuencia (10 por ciento) por lo cual se han propuesto algunos criterios diagnósticos operacionales para reducir el subdiagnóstico. No existen en la actualidad métodos de laboratorio útiles para la confirmación de los casos, por lo cual es necesario mantener un elevado índice de sospecha clínica. El tratamiento y la profilaxis con tiamina están bien establecidos, sin embargo no existe evidencia actual que avale el empleo de un esquema en particular. No obstante se conoce la evolución natural de esta patología, la que de no medir un tratamiento oportuno conlleva una elevada proporción de pacientes desarrollen alteraciones neurológicas de carácter irreversible que dan origen a una psicosis de Korsakoff y en otros casos aislados incluso a la muerte.

Thiamine deficiency and parasitic infection in rural Thai children.

A study was carried out to determine whether thiamine deficiency in northeast Thailand is linked with parasitic infection. We assessed the thiamine status and tested for parasitic infections in 231 children between 6 and 12 years of age in Nam Phong District of Khon Kaen Province, Thailand. Thiamine deficiency [thiamine pyrophosphate effect (TPPE) > 20%] and parasitic infections (mostly Strongyloides stercoralis) were identified in 16 and 45% of the children, respectively. No association between thiamine deficiency and parasitic infection was found. The general health of the children, according to Thai standard anthropometric curves, was within the normal range. The suboptimal thiamine status and the high prevalence of parasitic infection require attention.

Polyneuropathy from thiamin deficiency associated with thyrotoxicosis.

Polyneuropathy from thiamin deficiency can occur in persons who consume a diet consisting mainly of polished rice with low protein and thiamin content in the setting of excessive physical activity or hypermetabolic states. The authors report here a 17-year-old fisherman who presented with a 3-month history of symptoms and signs consistent with polyneuropathy. There were also clinical features of thyrotoxicosis which was confirmed by thyroid function test. His dietary intake consisted mainly of polished rice and fish both of which contain a small amount of thiamin. This could not cope with his hypermetabolic condition from thyrotoxicosis resulting in thiamin deficiency with polyneuropathy.

Edema generalizado e circulação hiperdinâmica: um possível caso de beribéri / Generalized edema and hyperdynamic circulation: a possible case of beriberi

Paciente com insuficiência cardíaca causada pelo beribéri, apresentando regressão dos sinais e sintomas adquiridos após medicação com tiamina.

Treatable cardiomyopathies.

Cardiomyopathy is defined as primary myocardial dysfunction which is not due to hypertensive, valvular, congenital, coronary or pulmonary vascular disease. This term usually denotes a dismal prognosis short of cardiac transplantation. However, several organic diseases of the heart can result in right or left ventricular dysfunction resulting in congestive heart failure and prompting the physician to label them as cardiomyopathy; the etiological factor is overlooked as it produces very subtle features. Therefore, before labelling any child as cardiomyopathic, all possible causes of ventricular dysfunction must be excluded by clinical and investigative means. The causes of "treatable cardiomyopathy" include mechanical factors as critical aortic stenosis and pulmonic stenosis, severe coarctation of aorta in an infant and aortaarteritis is an older child. Some of the persistent arrhythmias like atrial tachycardia, fibrillation, paroxysmal junctional re-entrant tachycardia are also known for causing ventricular dysfunction producing tachycardiomyopathy. Treatment of arrhythmia improves the ventricular function. Myocardial ischemia as a result of congenital coronary anomaly (commonest being anomalous origin of left coronary artery from pulmonary artery) can also present with a cardiomyopathy like picture. Early surgical correction is very rewarding. Finally, some of the metabolic conditions like creatinine and thiamine deficiency can also produce ventricular dilatation and dysfunction. In conclusion, the so called cardiomyopathy like picture can be produced because of several reasons and an attempt must be made to identify them.

Un brote de beriberi en una cárcel colombiana / An outbreak of beri-beri in Colombia jail

Se realizó una investigación de campo en Leticia (Amazonas) con el fín de esclarecer la causa del fallecimiento de un joven recluso en la cárcel municipal. Después de revisar la evidencia clínica y epidemiológica, concluimos que la causa de muerte fue una insuficiencia cardiaca con gasto alto, causada muy probablemente por déficit de tiamina (beriberi húmedo). Se hallaron cuatro casos más de beriberi mixto con manifestaciones moderadas, que pasaron sin diagnóstico, dentro de los reclusos. La dieta suministrda regularmente a los internos solo cubría la mitad de los requerimientos diarios de tiamina para personas adultas expuestas a un clima tropical. Este es el segundo informe publicado recientemente en Colombia sobre casos de beriberi y debe servir para que se extremen la vigilancia y las medidas dietarias de control en poblaciones altamente expuestas tales como aquellas institucionalizadas

Encefalopatía de Wernicke como complicación de hemodiálisis crónica: caso clínico / Wernicke's encephalopathy as an hemodialysis complication: clinical case

Wernicke encephalopathy, due to the depletion of thiamine, can be a complication of chronic hemodialysis. We report a 43 years old diabetic male in chronic hemodialysis, who two weeks after an infra-condyleal amputation of his left leg, was admited to the hospital due to an episode of vomiting and abdominal pain lasting 5 days, where confusion and ocular motor signs appeared. Parenteral thiamine administration was started and the confusional state abated. Dialysis can be a predisposing factor for Wernicke encaphalopathy and this diagnosis must be considered in confuse patients

Parálisis cordal y alcoholismo / Vocal cord paralysis and alcoholism

La parálisis recurrencial por alcoholismo es un hallazgo muy raro, si bien la literatura mundial menciona algunos casos. La inercia de las cuerdas vocales es debido a una neuropatía por probable déficit de vitamina B1 (tiamina). El siguiente trabajo relata una interesante manifestación clínica en un paciente alcohólico quien presentó disfonía, diagnosticándose paresia recurrencial izquierda cuya etiología, luego de descartar las causas más frecuentes y considerando que evolucionó favorablemente con vitamina B1, resultó ser una neuritis recurrencial alcohólica.

Encefalopatia de Wernicke: control de la enzima tiamina pirofosfato en sangre, en 30 pacientes con alteración del estado de conciencia, que consultaron en el Departamento de Medicina Interna del Hospital General San Juan de Dios

En nuestro pais, Guatemala, nunca se habia investigado lo que es la encefalopatia de Wernicke que traducido se refiere a la deficiencia de la Tiamina (Vitamina B1)/. Para poder realizar nuestra investigación tomamos una muestra de 30 pacientes, adultos de uno y otro sexo, excluyéndose los pacientes que presentaban alteraciones del estado de conciencia con diagnóstico de hipertensión arterial, accidente cerebro vascular, alcohólicos, abcesos cerebrales, (alcohólicos) o traumatismo cerebral. Que ingresaron por bez primera al hospital sin diagnóstico; al llegar el paciente a la emergencia, inmediatametne se procedió extraer 5 cc de sangre, la que se centrifugaba, se extraia el suero. El cual se hacia reaccionar posteriormente con 4 reactivos (blanco muestra Standar y control) específico para medir el incremento en sangre de la enzima Tiamina pirofosfato (ver metododologia)

Efeito da deficiência aguda de tiamina no epitélio jejunal proximal de ratos / Effect acute deficiency thiamine in jejunal epithelium of rats

Ratos alimentados com dieta sem tiamina desenvolveram inicialmente adipsia, em seguida anorexia e finalmente perda de peso corporal. Do ponto de vista morfométrico constatou-se hipotrofia do epitélio da vilosidade jejunal, enquanto que do ponto de vista cinético observou-se que a taxa de produçäo celular da cripta se manteve inalterada nos animais deficientes em vitamina. A adipsia e a hipotrofia observada poderiam ser responsáveis, pelo menos em parte, pela diminuiçäo da absorçäo de nutrientes. A manutençäo da taxa de produçäo celular da cripta, apesar da hipotrofia do epitélio da vilosidade, no grupo experimental, resultou provavelmente do balanço entre três fatores: nutriçäo luminal, produçäo adequada de DNA a presença de células epiteliais imaturas na vilosidade