Two Cases of Wernicke's Encephalopathy That Developed during Total Parenteral Nutrition in Colon Cancer Patients Treated with 5-Fluorouracil-based Chemotherapy / 대한소화기학회지

Wernicke's encephalopathy (WE) caused by thiamine deficiency is an acute neurological disorder. Clinically, the classic triad of WE consists of ophthalmoplegia, ataxia, and mental status changes. Thiamine deficiency is known to occur commonly in chronic alcoholic patients. Sometimes, it can occur in patients after gastrointestinal surgery and in those with malabsorption. In addition, patients undergoing renal dialysis, suffering from hyperemesis gravidarum, receiving total parenteral nutrition (TPN), and being treated with chemotherapeutic agents are also prone to develop thiamine deficiency. Herein, we report two cases of WE that developed following simultaneous 5-fluorouracil (5-FU) chemotherapy and TPN in colon cancer patients which was successfully treated with thiamine administration.

Acute administration of Zn, Mg, and thiamine improves postpartum depression conditions in mice

Background: Postpartum depression [PPD] affects approximately half of new mothers. Chronic exposure to progesterone during pregnancy and its withdrawal following delivery increases depression and anxiety. In addition, there are complex interactions between hormones, neurotransmitters, and trace elements. Zinc [Zn] and magnesium [Mg] in Auence the nervous system by impacting synaptic neurotransmis-sion in the brain. Thiamine [Vit B[1]] deficiency results in a high percentage of depressive behaviors. Elevated levels of reactive oxygen species in pregnancy are implicated in the pathogenesis of major depression

Methods: We examined the effects of different combinations of Zn, Mg, and Vit B[1] in an animal model of PPD. ZnCl, MgCl, and thiamine-HCl were administered to PPD-induced mice. Depression, anxiety-related behavior, and total antioxidant capacity [TAC] were assessed. Depression and anxiety-like behavior were evaluated by the forced swimming test [FST] and elevated plus-maze, respectively

Results: The acute combined administration of Zn, Mg, and Vit B[1] significantly decreased immobility time in FST, increased the percentage of both time spent in-and entries to open arms in the elevated plus-maze, and augmented TAC

Conclusion: Our data suggest that acute administration of combined treatment with Zn, Mg, and Vit B[1] on postpartum day 3 improves depressive symptoms and anxiety-like behaviors. Our evaluation of TAC is in accordance with behavioral results

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.

Vitamina B en paciente alcohólico: relato de un caso / Vitamine B in alcoholic patient: case report

Métodos: A partir del año 2007 se efectúan estudios en nuestro Instituto para precisar una dosis adecuada de complejo B a aplicar en el Síndrome de Deprivación Alcohólico (Ibáñez y Bustamante) Resultados: Después de tres semanas de tratamiento con benzodiacepina y complejo B (tiamina) según el nuevo protocolo se logra una recuperación motora y mental de un paciente con un Síndrome Korsakoiwideo alcohólico y un cuadro de paraparesia. Conclusión: Los resultados sugieren que la encefalopatía de Wernicke tratada con dosis superiores a 300 mg/diarios de tiamina puede tener un resultado altamente beneficioso para el paciente con síndrome de deprivación alcohólico.

Method: Since 2007, different studies have been made in our Institute, in order to find the right dose of Vitamin B Complex in cases of Alcohol Withdrawal Syndrome (Ibáñez y Bustamante). Results: After a 3 weeks treatment with Benzodiazepine and Vitamin B Complex (Thiamine), according to the new protocol, a patient with Alcoholic Korsakow Syndrome and Paraparesis, recovers his mental and motor functions. Conclusions: Wernicke’s encephalopathy can be treated with high doses of Thiamine (around 300 mg/a day), on patients with Alcohol Withdrawal Syndrome with highly good results.

Encefalopatia de Wernicke em criança com doença de Crohn / Wernicke encephalopathy in a child with Crohn disease

OBJETIVO: Descrever um caso de encefalopatia de Wernicke associada à doença de Crohn na infância. DESCRIÇÃO DO CASO: Menino de cinco anos, com diagnóstico de doença de Crohn por colonoscopia com biópsia há um ano; desde então, fez uso de diversos medicamentos sem resultados terapêuticos. Evoluiu com pancreatite há três meses, quando foi iniciada nutrição parenteral. Apresentou subitamente sonolência, episódios de frases desconexas e alteração da movimentação ocular. O exame neurológico evidenciou nistagmo vertical para cima em todas as posições e nistagmo horizontal no desvio conjugado do olhar. A ressonância magnética do encéfalo mostrou alterações compatíveis com a encefalopatia de Wernicke, sendo instituído o tratamento com tiamina parenteral imediatamente, com reversão completa dos sintomas por volta do 30º dia de tratamento. COMENTÁRIOS: Embora seja uma entidade rara, deve-se suspeitar de encefalopatia de Wernicke frente à presença de fatores de risco, uma vez que o tratamento imediato evita as sequelas neurológicas.

OBJECTIVE: To report a case of Wernicke encephalopathy associated with Crohn disease in childhood. CASE DESCRIPTION: A five year-old boy with Crohn disease, diagnosed by colonoscopy and biopsy one year ago; he has been treated with many different medications without results. During the past year, the patient was diagnosed with pancreatites and has received parenteral nutrition since then. After three months, the child suddenly presented drowsiness, meaningless speech and ocular movement disturbance. Neurologic examination disclosed upbeat nystagmus in all positions and horizontal nystagmus during conjugate deviation of the eyes. Magnetic resonance showed abnormalities consistent with Wernicke encephalopathy. Parenteral thiamine has been administered soon after diagnosis and by the 30th day of treatment, recovery of symptoms was complete. COMMENTS: Despite being a rare entity, neurological symptoms associated to the presence of risk factors such as prolonged parenteral nutrition should suggest Wernicke encephalopathy. Immediate treatment is important to avoid neurological disabilities.

Thiamine responsive megaloblastic anemia syndrome.

Thiamine responsive megaloblastic anemia syndrome (TRMA) is a clinical triad characterized by thiamine-responsive anemia, diabetes mellitus and sensorineural deafness. We report a 4-year-old girl with TRMA whose anemia improved following administration of thiamine and this case report sensitizes the early diagnosis and treatment with thiamine in children presenting with anemia, diabetes and deafness.

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.

Pediatric cardiac beriberi: 3 different presentations.

The authors report 3 cases of pediatric cardiac beriberi at Queen Sirikit National Institute of Child Health during the last 10-year-period. The first two cases presented classically while the third case came with an unusual presentation. Cardiac beriberi is an old disease in modern Bangkok which can present in as several different clinical patterns. The diagnosis needs a high index of suspicion. Echocardiographic findings of right heart dysfunction and dramatic response to intravenous thiamine are diagnostic.

Marchiafava-Bignami disease.

Marchiafava- Bignami disease is the symmetrical demyelination of the middle portion of the corpus callosum observed in people with chronic alcoholism. We report two male patients who had history of chronic alcoholism, different clinical presentation and MRI findings consistent with the diagnosis of Marchiafava-Bignami disease.

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.

Parotiditis crónica recidivante con trastornos inmunológicos asociados / Parotiditis chronic recidivante with immune inconveniences associates

En el departamento de ultrasonido diagnóstico fue atendida una paciente, de tres años de edad, que presentaba inflamación de ambas glándulas parótidas y que había presentado varios episodios de dolor, tumefacción unilateral o bilateral y fiebre. Había sido valorada en varias ocasiones por pediatría y había recibido múltiples tratamientos con antibióticos. Se le indicó ultrasonido de partes blandas y se valoró por inmunología. Se encontró un retardo en la fagocitosis y una disminución de la inmunidad celular. Se puso tratamiento con inmunomoduladores y antihistamínicos.

A 3-year-old female patient with swelling of both parotid glands that had presented various episodes of pain, unilateral or bilateral tumefaction and fever was seen at the diagnostic ultrasound department. She had been evaluated on several occasions by the pediatric department and had received multiple treatments with antibiotics. An ultrasound of soft tissues was indicated and she was assessed at the immunology service. A phagocytosis retardation and a reduction of cellular immunity were found. A treatment with immunomodulators and antihistamines was applied.

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.

A Korean Female Patient with Thiamine-responsive Pyruvate Dehydrogenase Complex Deficiency Due to a Novel Point Mutation (Y161C)in the PDHA1 Gene

Pyruvate dehydrogenase complex (PDHC) deficiency is mostly due to mutations in the X-linked E1alpha subunit gene (PDHA1). Some of the patients with PDHC deficiency showed clinical improvements with thiamine treatment. We report the results of biochemical and molecular analysis in a female patient with lactic acidemia. The PDHC activity was assayed at different concentrations of thiamine pyrophosphate (TPP). The PDHC activity showed null activity at low TPP concentration (1 x 10(-3) mM), but significantly increased at a high TPP concentration (1 mM). Sequencing analysis of PDHA1 gene of the patient revealed a substitution of cysteine for tyrosine at position 161 (Y161C). Thiamine treatment resulted in reduction of the patient's serum lactate concentration and dramatic clinical improvement. Biochemical, molecular, and clinical data suggest that this patient has a thiamine-responsive PDHC deficiency due to a novel mutation, Y161C. Therefore, to detect the thiamine responsiveness it is necessary to measure activities of PDHC not only at high but also at low concentration of TPP.

Wernicke's encephalopathy: report of a case.

A case of Wernicke's encephalopathy is reported. A 53-year-old alcoholic man was presented with acute confusion, upward gaze palsy of the left eye and gait ataxia for 5 days. The typical findings upon MRI of the brain showed multiple, scattered, small non-enhanced low signal intensity on T1WI, high signal intensity on T2WI involving at bilateral basal ganglias, and thalami, midbrain, pons, and periventricular regions with evidence of atrophic changes of brain and the mamillary bodies. After treatment with intravenous thiamine 100 mg/day, the left upward gaze palsy was fully recovered at the 8th day of treatment. The mental status and gait ataxia gradually improved.

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.

Marchiafava-Bignami disease: a case report.

Marchiafava-Bignami Disease (MBD) is a rare, severe and usually fatal neurological disorder associated with chronic alcoholism. Previously, the definite diagnosis was confirmed at the autopsy. After the era of modern imaging technology, diagnosis was based on clinical profiles, history of alcoholism and specific location of pathology in corpus the callosum demonstrated by MRI. The authors reported a case of MBD in a 41 year-old alcoholic Thai male who presented with acute confusion and ataxia. MRI of the brain demonstrated demyelination, edema and necrosis of the corpus callosum with extensive symmetrical subcortical white matter lesions. He had a dramatic recovery after treatment with intravenous thiamine. Follow-up MRI revealed atrophic and cystic changes of the corpus callosum and almost complete resolution of the subcortical lesions. Recently, 15 cases of MBD with specific corpus callosal lesion, demonstrated by MRI, were published in the English literature. All had a favorable outcome after treatment with thiamine. Only one case had extensive extracallosal lesions and this case also had a good recovery after treatment. Now, MBD is not a fatal disease and early diagnosis and treatment are crucial.

Polioencefalomalacia em bovinos nos estados de Mato Grosso do Sul e Säo Paulo / Polioencephalomalacia in cattle in the states of Mato Grosso do Sul and Säo Paulo

Trinta focos de polioencefalomalacia (PEM) foram diagnosticados no período de agosto de 1993 a outubro de 1997. Vinte e nove focos ocorreram no estado de Mato Grosso do Sul (MS) e um no estado de São Paulo (SP). Foram afetados bovinos de 4 a 84 meses de idade. A morbidade dos rebanhos afetados variou de 0,02% a 14,28% e a letalidade de 42,5% a 100%. A doença não apresentou uma sazonalidade e ocorreu em vários municípios do MS. Em todos os focos estudados os animais afetados eram criados em regimes de criação extensiva, com exceção de um foco. Os sinais clínicos observados foram exclusivamente nervosos e a evolução dos casos variou de 12 horas a 4 dias. A maioria dos animais tratados com tiamina e dexametasona recuperou-se. Histologicamente, as lesões consistiam de necrose laminar do córtex cerebral. Adicionalmente em 2 casos observaram-se hemorragias submeningeanas e corticais, e em 9 casos presença de infiltrado de eosinófilos. A dosagem de sódio no líquor apresentou-se elevada em um caso. A etiologia da PEM não está esclarecida, porém em alguns casos a intoxicação por cloreto de sódio/privação de água pode estar envolvida na etiologia da enfermidade. A PEM representa 4,78% dos casos de enfermidades de bovinos diagnosticadas no Laboratório de Anatomia Patológica da UFMS. Com bases nos resultados, verifica-se a importância da enfermidade no diagnóstico diferencial de outras doenças com quadro clínico neurológico no MS, principalmente a raiva, a meningoencefalite por herpesvírus bovino tipo 5 (HVB-5) e o botulismo.

Encefalopatia de Wernicke: a propósito de um caso com síndrome pilórica obstrutiva / Wernicke's encephalopathy: apropos of a case with obstructive pyloric syndrome

Os autores apresentam um caso de encefalopatia de Wernicke em um paciente com síndrome de obstruçäo pilórica por adenocarcinoma gástrico, sem história de alcoolismo e desnutriçäo. Comentam aspectos fisiológicos da importância da tiamina como co-enzima da transcetolase e da piruvato-descarboxilase na rota metabólica para produçäo de ATP e a possibilidade da existência de interaçäo de fatores genéticos e ambientais no surgimento do quadro clínico. Chamam a atençäo para a importância do diagnóstico clínico, que deve ser precoce para que a terapêutica específica com tiamina possa surtir efeito, principalmente em pacientes sem história prévia de alcoolismo e desnutriçäo