Dry Beriberi Research Articles

Dry beriberi mimicking the Guillain–Barré syndrome

A 44-year-old man is described with severe flaccid quadriparesis that evolved over 3 weeks. He had regularly binged on alcohol—up to 20 cans of beer per day with occasional consumption...

The Skinny on a Growing Problem: Dry Beriberi after Bariatric Surgery

The Skinny on a Growing Problem: Dry Beriberi after Bariatric Surgery

Can glucose make you faint?

In April, 2008, a 27-year-old woman came to the emergency department of her local hospital, with a 3-week history of dizziness, low appetite, and occasional vomiting. 2 months before, she had undergone an uncomplicated laparoscopic proximal gastric bypass for obesity; on discharge, she was prescribed multivitamins and lansoprazole.

Deficiência de tiamina como causa de cor pulmonale reversível

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.

Epidemia de beribéri no Maranhão, Brasil

This Editorial addresses the scientific community to report on a beriberi outbreak affecting part of the population in the Tocantins Region in the State of Maranhao, Brazil, with 466 and 543 reported cases and 33 and zero deaths in 2006 and 2007, respectively, predominating among males from 15 to 50 years of age, mainly with the dry beriberi clinical form (data from the Non-Communicable Diseases Division of the Superintendency for Epidemiology and Disease Control, Maranhao State Health Secretariat). During the same period, 30 cases and three deaths were recorded in the neighboring State of Tocantins, according to information provided by its respective State Health Secretariat. Importantly, the last previously recorded beriberi epidemic in Brazil occurred from 1870 to 1910, during the rubber boom in the Amazon Region, as extensively documented by Josue de Castro in Geography of Hunger (1946; English translation, 1952). After numerous patients were hospitalized in Imperatriz, Maranhao, with edema, paresthesia of the lower and upper limbs, difficulty walking, and death from cardio-respiratory failure, an investigation was launched in May 2006 by the Maranhao State Health Secretariat, with participation by the Health Surveillance Secretariat of the Brazilian Ministry of Health. The initial diagnostic hypotheses were: disease of unknown etiology, pesticide poisoning, alcohol intoxication, and Guillain-Barre syndrome. News on the disease had great national repercussion, and the investigation changed directions when a physician from the State of Ceara with experience in the Amazon Region telephoned the head of the regional health department in Imperatriz, suggesting the diagnosis of beriberi. The diagnosis was confirmed by a therapeutic test with thiamine administration. The investigation then focused on identifying the determinant factors, and confirmed the prevalence of alcohol abuse and heavy physical labor among the patients. Current studies are concentrating on the population’s food consumption, pesticide exposure, and possible contamination of rice with fungal microtoxins. Among the actions to quell the beriberi outbreak, emergency measures have been taken such as the purchase and supply of thiamine, as well as short and medium term measures like: distribution of basic food baskets; specific training for health workers; epidemiological, food, and nutritional surveillance; installation of Psychosocial Care Centers for Alcohol and Drug Abuse; investigation of rice processing by local mills; food enrichment; intensification in the inspection of marketing and use of pesticides; and systematization of studies under way on beriberi in the region. The Ministry of Health coordinated the creation of an Inter-Ministerial Task Force aimed at planning and implementing these measures, with representatives from: Office of the Chief of Cabinet; Ministry of Social Development and the Fight Against Hunger; Ministry of Agriculture, Livestock, and Supply; Ministry of Agrarian Development; National Council on Food and Nutritional Safety; National Health Surveillance Agency (ANVISA); and the Government of the State of Maranhao (as reported by the Coordinating Division for Food and Nutritional Policy, Ministry of Health, in 2007). The Task Force allowed greater institutional integration and a more holistic understanding of the problem. Despite difficulties in implementing some measures, the experience has been successful. Some issues in the health sector still need to be defined, like the ideal treatment time, criteria for cure and discharge, thiamine supplementation for patients’ family members, and investigation of other risk groups (children and pregnant women). Another relevant issue is the origin of the epidemic in its ecological, social, and economic dimensions. In recent decades, development in the affected area has focused on extensive deforestation and planting of eucalyptus groves to serve the mining and logging companies operating there, in addition to extensive cattle-raising, to the detriment of production of basic food crops, especially rice, beans, corn, cassava, and native foods like Brazil nuts, babacu, and buriti. The result has been the disorganization of the local economy, thus aggravating the prevailing social inequality and further jeopardizing the population’s access to food and quality of life.

Wernicke Encephalopathy: A Complication of Parenteral Nutrition Diagnosed by Magnetic Resonance Imaging

THIAMIN (vitamin B1) is a water-soluble vitamin involved in glucose metabolism. Its deficiency affects the cardiovascular (wet beriberi) and nervous (dry beriberi) systems, and in industrialized countries, it is commonly linked to chronic alcoholism. Wernicke encephalopathy (WE), a metabolic encephalopathy due to thiamine depletion, is clinically characterized by at least two among four features1: nutritional deficit, oculomotor abnormalities, ataxia, and stupor. Laboratory tests (measurement of serum thiamine or erythrocyte transketolase activity) are diagnostic, but the radiologic confirmation can be achieved by magnetic resonance imaging (MRI) with abnormal hyperintense signal in the periaqueductal gray matter, the mamillary bodies, and around the third ventricle on fluid-attenuated inversion recovery and T2MRI scans.2We report a case of non alcoholic WE complicating postoperative total parenteral nutrition (TPN) lacking micronutrient supply and confirmed by MRI.The diagnosis of benign gastric tumor was established after a gastroscopy with gastric biopsy in a 30-yr-old mother of two children without any relevant medical history but prolonged gastric intolerance and 8 kg weight loss. She underwent an elective laparoscopic partial gastrectomy. On the fourth postoperative day, she had a wound dehiscence and developed peritonitis that required an emergency laparotomy with peritoneal lavage and suture. After this procedure, TPN and adapted antibiotherapy were administered. She developed a digestive tract fistula, and TPN had to be continued. After 30 days, the patient reported fatigue, and hyponatremia (127 mm) and hypophosphatemia developed and were corrected, but she had to be transferred to the intensive care unit for stupor (Glasgow Coma Scale score of 8) without any focal signs, and she was intubated and ventilated. Cerebral computed tomography scan was normal; her sodium concentration went to 150 mm. Micronutrients (polyvitamins and trace elements) were added to TPN. After 3 days, her neurologic status improved, allowing extubation, but the clinical examination showed a horizontal nystagmus and a lateral ophthalmoplegia. MRI showed no sign of central pontine myelinolysis but hyperintense lesions in the medial thalami on fluid-attenuated inversion recovery and T2 MRI scans (fig. 1). After inquiry, it turned out that TPN before intensive care admission had been administered without micronutrient supplementation. Thiamine administration was increased (200 mg/day), and oculomotor and MRI signs regressed, but a severe cognitive deficit along with vertigo and loss of sphincter control persisted. More than 2 yr later, these symptoms are still present, and the patient is totally dependent and disoriented with memory disturbances.In the Western world, thiamine deficiency is characteristically associated with chronic alcoholism, because it affects thiamine uptake and utilization. However, WE may develop in nonalcoholic conditions, as in prolonged starvation,3hyperemesis gravidarum,4bariatric surgery,5and even healthy infants given the wrong formulas.6Thiamine is a cofactor of several enzymes involved in glucose metabolism and cerebral energy utilization, and its depletion could lead to the neuronal damage as seen on MRI, i.e. , T2 and fluid-attenuated inversion recovery hyperintense signaling in the mamillary bodies, periventricular thalamus, and periaqueductal gray matter,7as well as diffusion-weighted imaging to differentiate vasogenic from cytotoxic edema.8Subsequent MRIs may show regression of the signal abnormalities, but there is no correlation with improvement in clinical conditions.7Interestingly, metabolic disturbances such as hyponatremia and hypophosphatemia have been associated with WE and can be misleading,9as in our case. Lactic acidosis, however, is evocative of thiamine deficiency, especially in children.10Our case report emphasizes the diagnostic difficulties leading astray from WE in atypical settings and the usefulness of MRI to correct the diagnosis. However, the main issue is to remind us that TPN is not a harmless and easy technique, and that micronutrients should always be administered on a daily basis during TPN.11

Thiamine deficiency and beriberi features in a patient with hyperemesis gravidarum

Wernicke’s encephalopathy has been sporadically reported in patients with severe hyperemesis gravidarum. We report a new case of Wernicke’s encephalopathy in a patient who had hyperemesis gravidarum associated with signs and symptoms of dry and wet beriberi. The case was managed with very large doses of thiamine. The conclusion was that, in long-lasting hyperemesis gravidarum, recognizing signs of beriberi may help prevent the onset of Wernicke’s encephalopathy, thanks to timely therapy with thiamine supplements. A thiamine therapy similar to the one reported in this article could prove useful in long-lasting hyperemesis gravidarum complicated by Wernicke’s encephalopathy.

THIAMINE ADMINISTRATION IN ALCOHOL-DEPENDENT PATIENTS

( Received 28 August 2004; first review notified 12 September 2004; in revised form 28 September 2004; accepted 7 October 2004 ) Thiamine (vitamin B1) is a water-soluble vitamin that is involved in the metabolism of glucose and lipids as well as in the production of glucose-derived neurotransmitters (see Cook et al ., 1998). Its deficiency leads to a variety of neurological and cardiovascular symptoms and signs. Early symptoms may include fatigue, weakness and emotional disturbance, whereas prolonged gradual deficiency may lead to a form of polyneuritis (known as dry beriberi), cardiac failure or peripheral oedema (wet beriberi) (Thomson, 2000). Severe thiamine deficiency (TD) may result in the development of Wernicke's encephalopathy (WE). The classical signs of WE are ocular motility disorders (nystagmus, ophthalmoplegia), ataxia and mental changes (confusion, drowsiness, obtundation, clouding of consciousness, pre-coma and coma), although minor episodes of 'subclinical' encephalopathies are frequent (Reuler et al ., 1985). An appropriate treatment may correct most of these abnormalities; in contrast, the lack of a diagnosis of WE may result in serious consequences (Reuler et al ., 1985 …

Beriberi after gastric bypass surgery in adolescence

We report three cases of dry beriberi in adolescents who underwent gastric bypass surgery for clinically severe obesity. The key to successful treatment of this problem lies in prompt recognition and thiamine supplementation.

Molecular mechanisms of thiamine utilization.

Thiamine is required for all tissues and is found in high concentrations in skeletal muscle, heart, liver, kidneys and brain. A state of severe depletion is seen in patients on a strict thiamine-deficient diet in 18 days, but the most common cause of thiamine deficiency in affluent countries is alcoholism. Thiamine diphosphate is the active form of thiamine, and it serves as a cofactor for several enzymes involved primarily in carbohydrate catabolism. The enzymes are important in the biosynthesis of a number of cell constituents, including neurotransmitters, and for the production of reducing equivalents used in oxidant stress defenses and in biosyntheses and for synthesis of pentoses used as nucleic acid precursors. Because of the latter fact, thiamine utilization is increased in tumor cells. Thiamine uptake by the small intestines and by cells within various organs is mediated by a saturable, high affinity transport system. Alcohol affects thiamine uptake and other aspects of thiamine utilization, and these effects may contribute to the prevalence of thiamine deficiency in alcoholics. The major manifestations of thiamine deficiency in humans involve the cardiovascular (wet beriberi) and nervous (dry beriberi, or neuropathy and/or Wernicke-Korsakoff syndrome) systems. A number of inborn errors of metabolism have been described in which clinical improvements can be documented following administration of pharmacological doses of thiamine, such as thiamine-responsive megaloblastic anemia. Substantial efforts are being made to understand the genetic and biochemical determinants of inter-individual differences in susceptibility to development of thiamine deficiency-related disorders and of the differential vulnerabilities of tissues and cell types to thiamine deficiency.

Beriberi in a well-nourished Amazonian population

Beriberi is an endemic disease among the Naporuna indigenous people of Ecuadorean Amazonia (annual morbidity rate of 1.5%). A total of 47 patients with dry beriberi were seen at the Franklin Tello Hospital, Napo, during 1995–1996; a similar incidence was observed in previous years. No wet beriberi was found. Although the diagnosis of the disease was made clinically, an effective and quick response to thiamine treatment excluded other differential diagnoses, such as tropical ataxic neuropathy. Although indigenous people have several possible dietetic risk factors, none of them adequately explain the disease’s high incidence. Some suggestions are made for the high incidence of beriberi among this Amazonian people but its cause remains unknown.

Nutritional melalgia, a deficiency vascular disease.

A syndrome of painful burning feet observed among Japanese prisoners of war seemed to have been inadequately reported and referred to as dry beriberi. It was believed the clinical picture with regard to appearance and late effects justified the label nutritional causalgia. Search of the literature revealed that Glusman 1 had reviewed the syndrome proposing the name of nutritional melalgia. Correspondence with former prisoners of war suffering from residual symptoms showed that the physicians they encountered gave varying evaluations of their complaints, usually being unimpressed. The attitude of the physicians was different from that of those who had seen the condition at first hand. It seems appropriate to report the condition in order to disseminate information for the benefit of a misunderstood group of patients. It is also desirable to present this syndrome for the sake of emphasizing a nutritional factor in peripheral vascular disease. I encountered the syndrome of