[Wernicke encephalopathy : a neuro-ophthalmological side effect of bariatric surgery]. / L'encéphalopathie de Gayet-Wernicke : une complication neuro-ophtalmologique de la chirurgie bariatrique.

Due to an increase in the worldwide prevalence of obesity and the efficiency of bariatric surgery, this procedure is more often performed. Besides its benefits, it has also disadvantages and may be the cause of nutritional deficiencies. Thiamin deficiency is particularly important to diagnose and to treat early as it can lead to major sequelae and even to death. Wernicke's encephalopathy is the most frequent presentation associating confusion, ataxia, ophtalmoplegia and nystagmus. The full triad is not usually observed, which may lead to sub-diagnosis of this affection. The diagnosis is clinical, biological and radiologic thanks to the brain MRI. Intravenous thiamin supplementation therapy must be administered as fast as possible in order to avoid long-term damages. In the ophthalmological field, the potential sequelae are ophthalmoplegia, nystagmus and optic neuropathy. Therapeutics for nystagmus are pharmacological, surgical and/or optical. We illustrate this condition with a case report of an 18-year-old man developing Wernicke's encephalopathy as early as six weeks after a sleeve gastrectomy.

Suite à une augmentation de la prévalence de l'obésité dans le monde et à l'efficacité de la chirurgie bariatrique, cette technique est pratiquée de plus en plus fréquemment. Malgré ses avantages, elle n'est pas sans risque et peut être responsable de déficits nutritionnels multiples. Le déficit en vitamine B1 ou thiamine est particulièrement important à connaître et, à rapidement diagnostiquer en raison des nombreuses séquelles invalidantes, voire le décès du patient, dont il peut être responsable. Le tableau classique est l'encéphalopathie de Gayet-Wernicke associant confusion, ataxie et troubles oculomoteurs. Néanmoins, il n'est pas toujours complet, ce qui participe au sous-diagnostic de cette pathologie. Le diagnostic est clinique, biologique et/ou radiologique grâce à l'IRM cérébrale. La supplémentation vitaminique intraveineuse doit être instaurée le plus rapidement possible afin d'éviter des séquelles à long terme. D'un point de vue ophtalmologique, les séquelles potentielles sont les ophtalmoplégies, les nystagmus et les neuropathies optiques. Les thérapies envisageables du nystagmus, outre la supplémentation en thiamine en aigu, sont pharmacologiques, chirurgicales et/ou optiques. Nous illustrons cette pathologie par un cas clinique d'encéphalopathie de Gayet-Wernicke dès la 6ème semaine post-opératoire d'une chirurgie bariatrique de type «sleeve¼ chez un patient de 18 ans.

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).

Altered Mental Status and Cardiac Failure Due to Thiamine Deficiency in an Overweight Teen.

We describe an overweight but otherwise previously healthy 17-year-old female who presented with altered mental status and rapidly progressive weakness. She was ultimately diagnosed with Wernicke encephalopathy and wet beriberi resulting from severe thiamine deficiency. She required admission to the pediatric ICU because of hypoventilation with progressive weakness and worsening encephalopathy and was found to have impaired cardiac function as assessed by echocardiography. Her heart function and encephalopathy improved on initiation of thiamine repletion. She remained in inpatient rehabilitation for 10 months but still remained weak at discharge. Thiamine deficiency is not commonly considered in the United States as a diagnosis other than in patients with severe alcohol use disorder. However, thiamine may be depleted in as little as 2 weeks if nutrition is inadequate. In such a setting, thiamine deficiency is an important etiology to consider early in the pediatric patient with altered mentation especially because it can be readily and safely treated.

Nystagmus in a child with nephrotic syndrome.

We report a child with steroid-dependent nephrotic syndrome presenting with excessive irritability, double vision and inability to walk for 5 days. On examination, the child was irritable with Glasgow coma sccale (GCS of 12/15, had bilateral convergent squint (R>L), vertical nystagmus, ataxia without any focal neurological deficits and normal fundus. MRI brain with venogram showed bilateral symmetric FLAIR hyperintensity in the medial thalamus and periaqueductal grey matter showing diffuse restriction with normal venogram. A possibility of Wernicke encephalopathy (WE) was considered and the child was started on thiamine supplementation, following which he had significant improvement in his symptoms. His irritability reduced with significant improvement in the range of eye movements and vertical nystagmus. At 3-month follow-up, the child is asymptomatic with normal gait. Although WE is uncommon in children with nephrotic syndrome, the possibility has to be kept in mind when a child presents with atypical neurological symptoms.

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.

Uncommon bilateral optic neuropathy in Wernicke's encephalopathy complicating gravidarum hyperemesis.

Wernicke encephalopathy (WE) is a rare neurological disorder that results from vitamin B1 (Thiamin) deficiency, classically characterized by the triad of ophtalmoplagia, altered consciousness, and ataxia. WE is often associated with alcoholism, malnutrition, or gastrointestinal diseases with malabsorption. The association of «gravidarum hyperemesis¼ and WE seems to be underestimated. We report a 24-year-old pregnant woman with hyperemesis gravidarum, who presented with decreased visual acuity of both eyes. Fundus examination showed a bilateral stage 2 papillary edema. brain magnetic resonance imaging (MRI) showed bilateral and symmetrical hyper intense lesions on T2-weighted and FLAIR sequences in periaqueductal gray matter, thalamus, and mammillary bodies, which confirmed WE complicated by bilateral optic neuropathy. Her symptoms resolved after thiamine treatment. This case raises of the possibility of optic neuropathy in WE, which is a diagnostic emergency requiring early treatment to prevent complications.

Síndrome de Wernicke-Korsakoff em paciente gestante de 18 anos / Wernicke-Korsakoff syndrome in an 18-year-old pregnant patient

A síndrome de Wernicke-Korsakoff é uma rara encefalopatia desencadeada pela deficiência de tiamina, uma vitamina do complexo B, que atua como importante cofator de enzimas responsáveis pela manutenção da homeostase da energia cerebral. Apresentamos o caso de uma paciente de 18 anos, gestante, com diagnóstico prévio de pancreatite aguda biliar, que evoluiu à hiperêmese gravídica e à Wernicke-Korsakoff. Objetivamos, com este trabalho, chamar a atenção para a importância do diagnóstico imediato dessa síndrome diante de seu potencial em causar danos cerebrais irreversíveis, caso não tratada precocemente.

Wernicke-Korsakoff syndrome is a rare encephalopathy triggered by deficiency of thiamine, a B-complex vitamin, which acts as an important cofactor of enzymes responsible for maintaining brain energy homeostasis. We present the case of an 18-year-old pregnant woman with previous diagnosis of acute biliary pancreatitis, who developed hyperemesis gravidarum and Wernicke-Korsakoff. With this work, we aim to draw attention to the importance of the immediate diagnosis of this syndrome in view of its potential to cause irreversible brain damage if not treated early.

Wernicke encephalopathy as a complication of hyperemesis gravidarum: case report / Encefalopatia de Wernicke como complicação de hiperêmese gravídica: relato de caso

Abstract Wernicke encephalopathy (WE) is an acute neurological disorder resulting from vitamin B1 deficiency, which is common in chronic alcoholism. We report a rare case of WE due to hyperemesis gravidarum in a 25-year-old pregnant patient at 13 weeks and 5 days of gestation. Initially, the disease manifested as weakness, mental confusion, anterograde amnesia, and visual and auditory hallucinations. The diagnosis was established after the detection of suggestive findings of WE in the thalamus by magnetic resonance imaging (MRI) and a rapid improvement in the patient's clinical status subsequent to treatment with thiamine. Hyperemesis is a rare cause of WE, which makes the reported case important in the literature and reinforces the need for attention in clinical practice to rare but important complications of this common condition (hyperemesis gravidarum).

Resumo A encefalopatia de Wernicke (EW) é uma condição neurológica aguda resultada da deficiência de vitamina B1, muito comum em etilistas crônicos. Relatamos um caso de EW secundário a um quadro de hiperêmese gravídica em uma gestante de 25 anos de idade e 13 semanas e 5 dias de idade gestacional. Inicialmente essa desordem se manifestou como fraqueza, confusão mental, amnésia anterógrada, e alucinações auditivas e visuais. O diagnóstico foi estabelecido depois da detecção de achados sugestivos de EW na ressonância nuclear magnética e da melhora do quadro clínico com reposição de tiamina. A hiperêmese gravídica não é uma causa comum de EW, o que faz com que o presente relato de caso tenha importância na literatura e reforça a necessidade de atenção na prática clínica para complicações raras mas importantes desse quadro tão comum (hiperêmese gravídica).

Coma irreversível em paciente portadora de esquizofrenia paranóide: encefalopatia de Wernicke após síndrome de realimentação: um relato de caso / Irreversible coma in a patient with paranoid schizophrenia: Wernicke encephalopathy after refeeding syndrome: a case report

Encefalopatia de Wernicke (EW) e síndrome de realimentação (SR) são duas condições frequentemente coexistentes, subdiagnosticadas e que podem implicar prognóstico reservado após sua instalação. Sua identificação precoce representa um desafio para os intensivistas, haja vista a falta de sensibilidade e especificidade das manifestações clínicas. Apresenta-se um relato de caso de uma paciente portadora de esquizofrenia paranoide, sem histórico de abuso de álcool, que desenvolveu quadro de coma irreversível após greve de fome, sendo feito diagnóstico tardio de EW associada a SR. Descreve-se a evolução clínica e neuropsiquiátrica com o intuito de enfatizar a necessidade crucial de alta suspeição diagnóstica, com reposição vitamínica imediata, vigilância de distúrbios eletrolíticos e progressão parcimoniosa do aporte nutricional. (AU)

Wernicke encephalopathy (WE) and refeeding syndrome (RFS) are two often coexisting, underdiagnosed conditions that may involve a poor prognosis after their onset. Early identification represents a challenge for intensivists, given the lack of sensitivity and specificity of clinical manifestations. We report a case of a patient with paranoid schizophrenia, without a history of alcohol abuse, who developed irreversible coma after a hunger strike, with a late diagnosis of WE associated with RFS. The clinical and neuropsychiatric outcomes are described herein in order to emphasize the crucial need for a high diagnostic suspicion, with immediate vitamin replacement, monitoring of electrolyte disorders, and gradual progression of nutritional support. (AU)

Desnutrición en el anciano y encefalopatía de Wernicke / Malnutrition in the elderly and Wernicke encephalopathy

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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.

Encefalopatía progresiva de origen carencial: identificar a los pacientes en riesgo de déficit de tiamina / Progressive encephalopathy associated with nutritional deficiencies: Identifying patients at high risk for developing thiamine deficiency

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Psicosis de Korsakoff, a propósito de un caso interesante / Korsakoff's psychosis, about an interesting case

La psicosis de Korsakoff (PK) es una de las causas más frecuentes de amnesia. Se caracteriza por confusión mental, deterioro de la memoria reciente y confabulación. Se presenta el caso de un paciente masculino de 53 años de edad, fumador de un paquete al día, durante más de 35 años, bebedor de riesgo, con antecedentes de hipertensión arterial e hipercolesterolemia. Se diagnosticó como psicosis de Korsakoff debido al déficit de tiamina o vitamina B1. La psicosis de Korsakoff es un síndrome amnésico que puede presentarse precedido o no de encefalopatía de Wernicke (EW), por lo que se diagnostican menos casos de los que en realidad existen. Por ello, es un problema frecuentemente infradiagnosticado en los centros de salud lo que resulta interesante el conocimiento de esta patología (AU).

Korsakoff psychosis is one of the most frequent causes of amnesia. It is characterized by mental confusion, impairment of the recent memory and confabulation. It is presented the case of a male patient, aged 53 years, who smoked 1 packet of cigarettes a day during more than 35 years, risk drinker with antecedents of arterial hypertension and hypercholesterolemia. He was diagnosed as Korsakoff psychosis due to the thiamine or B1 vitamin deficit. Korsakoff psychosis is an amnesic syndrome that may be preceded or not by Wernicke encephalopathy, so there are diagnosed fewer cases than those truly existing. That is why it is a problem frequently underdiagnosed in health care institutions, making interesting this disease´s knowledge (AU).