Visual loss and encephalopathy in a pregnant patient with hyperemesis gravidarum.

A pregnant woman in her 20s at 17 weeks of gestation, presented with symptoms of painless diminution of vision preceded by 8 weeks history of hyperemesis gravidarum. On examination, she was confused, disoriented and had gait ataxia with complete loss of vision in both eyes. Fundus examination revealed grade 4 disc oedema with superficial retinal haemorrhages. Possibilities kept were cerebral venous sinus thrombosis, neuromyelitis optica spectrum disorder, posterior reversible encephalopathy syndrome and Wernicke's encephalopathy (WE). Thiamine levels were low. MRI brain with MR venography revealed symmetrical areas of hyperintensities in bilateral medial thalami, hypothalamus, mammillary body and area postrema. She was managed as a case of WE with intravenous thiamine with complete clinical and radiological resolution within 2 weeks of treatment. Therefore, we conclude that a high index of suspicion of WE in appropriate clinical settings leading to early treatment can potentially reverse its grave clinical symptoms and complications.

Bilateral hearing loss as the initial presentation of reversible Wernicke's encephalopathy with splenial lesion.

BACKGROUND: Wernicke's encephalopathy (WE) is an acute neurological syndrome resulting from thiamine (vitamin B1) deficiency. It has been recognized increasingly in non-alcoholic patients, such as in the condition of malnutrition. Recent literature has shed light on uncommon symptoms and neuroimaging findings. CASE REPORT: We reported a case of a 44-year-old male who initially presented with bilateral hearing loss, and exhibited abnormality in the splenium of the corpus callosum on magnetic resonance imaging (MRI) diffusion-weighted imaging sequence. On the following day the patient developed new symptoms, including unstable walking, double vision and hallucination. The subsequent brain MRI demonstrated lesions involving periaqueductal grey matter and bilateral medial thalamus, indicating the diagnosis of WE. Empirical treatment with intravenous thiamine resulted in complete clinical and radiological resolution. CONCLUSION: To the best of our knowledge, the current case is the first report of WE in literature with uncommon but reversible manifestations. This case warns us to maintain a heightened level of suspicion for WE in malnourished patients with neurological deficits, despite the possibility of atypical presentations encompassing bilateral hearing disturbances and unusual neuroradiological results. Early diagnosis and timely administration of thiamine in WE are likely to lead to a favorable outcome and full recovery.

Presentation of Wernicke encephalopathy in patient secondary to one anastomosis gastric bypass. Case report and literature review.

Wernicke encephalopathy, which is caused by a thiamine deficiency, occurs in 0.8-2% of the population. Only 16% present the typical triad of this disease: nystagmus, confusion and ataxia. We present the case of a postoperative patient with a one anastomosis gastric bypass with reoperation undergoing a Roux-en-Y gastric bypass that begins with confusion and nystagmus on her third postoperative day. The diagnosis of Wernicke encephalopathy is made by imaging, and vitamin B1 is administered with total improvement of nystagmus and altered state of consciousness (lethargy, bradypsychia, bradylalia).

La encefalopatía de Wernicke se produce por una deficiencia de tiamina se presenta en un 0.8-2% de la población. Solo el 16% de los casos presentan la tríada típica de esta enfermedad: nistagmo, confusión y ataxia. Presentamos el caso de una paciente operada de bypass gástrico de una anastomosis con reintervención convirtiendo a bypass gástrico en Y de Roux que en su tercer día de posoperatorio comienza con confusión y nistagmo. Se realiza por imagen el diagnóstico de encefalopatía de Wernicke se administra vitamina B1 con mejoría total del nistagmo y alteración del estado de consciencia (letargia, bradipsiquia, bradilalia).

Use of Thiamine Supplementation in Pregnant Women Diagnosed With Hyperemesis Gravidarum and Wernicke Encephalopathy.

Hyperemesis gravidarum is the most common condition requiring hospital care for women during the first 20 weeks of pregnancy and may lead to malnutrition, dehydration, and vitamin deficiencies. Depletion of vitamins such as thiamine may result in the development of Wernicke encephalopathy, a severe neurological disorder that can increase the risk for mortality and morbidity for the mother and fetus. A lack of awareness regarding the relationship of hyperemesis gravidarum and Wernicke encephalopathy may result in delayed treatment and disease management. Glucose administration in the presence of thiamine deficiency may induce Wernicke encephalopathy; protocols are needed to ensure dextrose is used for women with hyperemesis gravidarum in times of prolonged vomiting and poor oral intake only after first administering thiamine. This article includes a discussion of best practices for thiamine supplementation with hyperemesis gravidarum and Wernicke encephalopathy.

Diagnosis and treatment of Wernicke's encephalopathy: A systematic literature review.

BACKGROUND: Wernicke's encephalopathy (WE) is a serious neurological disorder that is underdiagnosed. Despite limited clinical guidelines, the standard use of intravenous (IV) thiamine is underutilized and remains an area of research deserving much attention. OBJECTIVES: We conducted a systematic review using Medline, Embase, and CENTRAL databases to identify and summarize the literature on IV thiamine treatment in WE. Human studies with WE patients who received ≥100 mg of thiamine IV met inclusion criteria. Randomized controlled trials, cross-sectional studies, and case reports were included. RESULTS: A total of 27 studies were included: 20 case reports, five retrospective studies, one prospective study and one randomized control trial. Of the case reports, 11 (55%) cases were female, and the average age of all cases was 45 years (SD = 15). The other seven studies included 688 patients; the average age was 52 years (SD = 9), and 266 (38.7%) were female. Among the case reports, neurological and clinical findings were used to diagnose WE in 16 (80%) cases. MRI was utilized to diagnose 15 (75%) cases. 500 mg IV thiamine TID was reported in 12 case reports (60%). 18 (90%) of case reports had partial or complete resolution of symptoms following IV thiamine. CONCLUSION: IV thiamine can alleviate neurological symptoms, cognitive dysfunction, and brain imaging lesions associated with WE. We found key limitations in the evidence for IV thiamine and diagnostic standards for WE. Future targeted research should establish clear diagnostic and treatment guidelines for WE to prevent this serious condition from being underdiagnosed or undertreated.

Atypical Wernicke's encephalopathy without mental status changes following bariatric surgery in an adolescent patient.

Morbid obesity is a systemic disease which can result in chronic complications, including hypertension, diabetes mellitus, depression, osteoarthritis and low self-esteem in the adolescent population.Bariatric surgery can be indicated to treat more severe forms of obesity, but these procedures are not without long-term risks. Therefore, adequate preoperative and postoperative care, which includes preoperative psychosocial evaluation for compliance, ongoing nutrition counselling and vitamin and micronutrient supplementation, is required for all patients, especially adolescent patients, who generally may not comply with medical therapies and/or be able to developmentally fully appreciate or comprehend the health consequences of their behaviours, prior to as well as after bariatric surgery to prevent complications.Thiamine pyrophosphate, an active form of thiamine (also known as vitamin B1, a water-soluble vitamin), which functions as a coenzyme in glucose and energy metabolism, is one such vitamin that requires supplementation postoperatively. It is mandatory for glucose to be administered concomitantly with thiamine, as glucose alone can precipitate Wernicke's encephalopathy (WE) in thiamine-deficient individuals. WE is a medical emergency, with a mortality rate of up to 20%. WE is best understood as a classic triad of mental confusion, gait ataxia and eye movement abnormalities, and atypical WE or Wernicke's syndrome (WS) is seen when the classic triad is not present. Cases that meet some, but do not necessarily meet all three criteria, are referred to as atypical WE or WS which can lead to delayed diagnosis. Atypical WE has an incidence of 19% which can lead to misdiagnosis of a preventable medical emergency with fatal complications.The following case reviews the consequences of post-bariatric thiamine supplementation therapy non-adherence and resulting in a deficiency in an adolescent patient.

Encefalopatía de Wernicke secundario a hiperémesis gravídica: la importancia del diagnóstico temprano / Wernicke encephalopathy secondary to hyperemesis gravidarum: importance of early diagnosis

RESUMEN Introducción: La hiperémesis gravídica (HG) es una condición frecuente en el embarazo, que puede resultar en complicaciones potencialmente letales como la encefalopatía de Wernicke (EW), síndrome que al ser reconocido y tratado tardíamente puede traducirse en una alta morbi-mortalidad materna y fetal. Objetivo: Describir el primer caso de EW secundario a HG en Colombia y realizar una revisión de la literatura publicada sobre su diagnóstico y tratamiento. Materiales y métodos: Se describe un caso de EW secundario a HG en el que se brindó un manejo interdisciplinario. Se realizó una revisión de la literatura con los términos "encefalopatía de Wernicke", "hiperémesis gravídica" y "embarazo" incluyendo reportes de casos, series de casos, artículos de revisión, investigaciones originales o cartas al editor en inglés, español y francés, en donde se analizaron el método y tiempo del diagnóstico, pauta de tratamiento y estado funcional final. Resultados: Se incluyeron 69 publicaciones y se identificaron 89 casos. En 23 de ellos se presentó pérdida gestacional, sólo en el 12,4% de los casos se reportó el nivel de tiamina, de los cuales en el 90% se encontraba disminuido y de los casos en donde se reportó estado funcional final en el 5,9% la gestante falleció. Conclusión: La EW secundaria a HG es una complicación potencialmente letal. Debe sospecharse ante cualquier alteración neurológica e historia de emesis persistente. El diagnóstico y tratamiento oportuno interdisciplinario son fundamentales para disminuir el riesgo de secuelas que limitan la capacidad funcional con alto impacto en la calidad de vida.

ABSTRACT Introduction: Hyperemesis gravidarum (HG) is a frequent condition in pregnancy, which can result in potentially lethal complications such as Wernicke encephalopathy (WE), a syndrome that can be translated into a high maternal and fetal morbidity and mortality if it is recognized and treated late. Objective: To describe the first case of WE due to HG in Colombia and to review the published literature about its diagnosis and treatment. Materials and methods: We describe a case of WE due to HG with an interdisciplinary approach. A review of the literature was performed with the terms "Wernicke's encephalopathy", "hyperemesis gravidarum" and "pregnancy" including case reports, case series, review articles, original investigations or letters to the editor in English, Spanish and French, where the method and time of the diagnosis, treatment regimen and sequelae were analyzed. Results: Sixty-nine publications were included and 89 cases were identified. In 23 of them had a gestational loss, only in 12.4% of the cases the thiamine level was reported, in which 90% was diminished and in the cases where the final functional status was reported in 5.9% of the pregnant woman died. Conclusion: WE due to HG is a potentially lethal complication. In any neurological disturbance and history of persistent emesis it should be suspected. Timely interdisciplinary diagnosis and treatment are essential to reduce the risk of sequelae that limit functional capacity with a high impact on quality of life.

La encefalopatía de Wernicke: complicación de la hiperémesis gravídica / Wernicke's encephalopathy: a complication of hyperemesis gravidarum

La encefalopatía de Wernicke (EW) es una patología neurológica ocasionada por un déficit de vitamina B1 o tiamina. Se produce ante una situación que impida la absorción de esta vitamina, como sucede durante la hiperémesis gravídica. Se manifiesta con la tríada clásica de ataxia, oftalmoplejía y confusión mental, aunque en algunas ocasiones la sintomatología es muy inespecífica. Si la enfermedad no es tratada puede ocasionar lesiones irreversibles. Es fundamental establecer un diagnóstico y tratamiento precoz para evitar posibles secuelas tanto maternas como fetales. Presentamos el caso clínico de una gestante con EW como consecuencia de una hiperémesis gravídica. Finalmente, la situación se resolvió de forma favorable tanto para la madre como para el hijo

The Wernicke's encephalopathy (WE) is a neurological disease caused by a deficiency of vitamin B1 or thiamine. It occurs in any situation that prevents the absorption of the vitamin, such as during a hyperemesis gravidarum. It manifests with the classic triad of ataxia, ophthalmoplegia and mental confusion, although sometimes the symptoms are very nonspecific. If the disease is untreated, it can cause irreversible damage. The diagnosis and early treatment remain to be essential to avoid both maternal and fetal sequelae. We report the case of a pregnant woman with WE as a result of hyperemesis gravidarum with good fetal and maternal outcomes

Encefalopatía de Wernicke y polineuropatía asociada a déficit de complejo B después de una cirugía bariátrica / Wernicke´s encephalopathy and polyneuropathy associated with vitamin B complex deficiency after a bariatric surgery

La cirugía bariátrica es un tratamiento que garantiza una pérdida de peso sustancial y duradera, y beneficios tangibles respecto a condiciones médicas asociadas a la obesidad. El aumento del número de cirugías bariátricas ha llevado también a un aumento de las complicaciones relacionadas con ella, incluyendo la encefalopatía de Wernicke y la polineuropatía por deficiencia de vitaminas del complejo B. En este artículo se reporta un caso de encefalopatía de Wernicke siete semanas después de la cirugía, enfatizando en la importancia de reconocer el espectro de la sintomatología para hacer un diagnóstico temprano, que permita intervenir en la fase reversible de esta enfermedad potencialmente letal.

Bariatric surgery is a treatment that guarantees a substantial and lasting weight loss in addition to the tangible benefits relating to obesity-associated medical conditions. The increasing number of bariatric surgeries has revealed an increasing number of complications related to this procedure, including Wernicke´s encephalopathy and vitamin B deficiency polyneuropathies. Herein, a 7-week post-surgery case of Wernicke´s encephalopathy is presented that emphasizes the importance of an early recognition of these symptoms so as to initiate intervention during the reversible phase of these potentially lethal pathologies.

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.

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.

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