The effectiveness of using thiamine in the Wernicke-Korsakoff syndrome

Introduction: The Wernicke-Korsakoff syndrome is a condition caused by thiamine (vitamin B1) deficiency in the brain, being a debilitating and potentially fatal factor. It is characterized by a classic triad: delirium, ophthalmoparesis and ataxia. Objectives: Analyze the possible effectiveness of using thiamine in the prognostic change of patients with the syndrome, as well as the ideal dose and identification of possible secondary outcomes of the use of thiamine. Methods: A systematic review made in March 2021, included studies published between 2011-2021. The descriptors selected according to the MeSH platform, were inserted in the SCIELO, Lilacs and PubMed databases, resulting in a total of 323 studies, of which only 8 were selected. Results: Among the 8 evaluated articles, 5 reinforce the effectiveness of thiamine therapy, with prognostic changes in those patients, and only 4 of these studies describe their clinical evolution, showing mostly a gradual regression of the ocular manifestations and ataxia, while neurological symptoms tend to develop later. Thus 62,5% of the articles show improvement of patients with these therapeutics. Other studies do not refer to the prognosis after the institution of the treatment. About the dose, it was observed that the therapeutic effectiveness was related to higher doses of thiamine. Conclusion: Most of the analyzed studies were favorable to the hypothesis of the early use of thiamine in regression of the symptoms. Regarding the most effective dose, the topic still needs studies with high scientific evidence, as it hasn’t yet been thoroughly discussed in the literature.

Importance of Immediate Thiamine Therapy in Children with Suspected Thiamine-Responsive Megaloblastic Anemia—Report on Two Patients Carrying a Novel SLC19A2 Gene Mutation

Thiamine-responsive megaloblastic anemia (TRMA) is an autosomal recessive disorder characterized by the development of megaloblastic anemia, diabetes mellitus, and sensorineural deafness. We report on the first two Croatian patients with TRMA, compound heterozygotes for nonsense, c.373C > T; p.(Gln125Ter) and novel missense variant, c.1214C > G; p.(Thr405Arg) in SLC19A2 gene. The first was diagnosed at 4 months with diabetes mellitus and severe anemia requiring transfusions. As TRMA was suspected, thiamine therapy was immediately started to prevent further transfusions and insulin therapy. His brother developed extreme anemia at 3 weeks of age while waiting for the results of the genetic test. Severe anemia in this sibling may have been prevented if thiamine had been initiated earlier.

Wernicke’s Encephalopathy: Typical Disease with an Atypical Clinicoradiological Manifestation

Wernicke’s encephalopathy is an acute neurological disorder caused due to thiamine deficiency. It is characterized by a triad of confusion, ataxia, and ophthalmoplegia. Wernicke’s encephalopathy can present with atypical clinical manifestations, particularly in setting of nonalcoholic background. Our patient manifested with clinical complaints of acute drowsiness, speech difficulty, and ataxia. Magnetic resonance imaging of the brain revealed T2 hyperintensity in bilateral medial thalami, globus pallidus, periaqueductal gray matter, dorsal pons, and dorsal medulla. The diagnosis of Wernicke’s encephalopathy was entertained and he well responded to parental thiamine therapy. The clinician should be aware about atypical presentation of Wernicke’s encephalopathy.

Incidence of Adverse Reactions to Parenteral Thiamine in the Treatment of Wernicke’s Encephalopathy, and Recommendations

Aim To offer an estimate of the incidence of anaphylactic reactions to parenteral products containing thiamine used in the treatment of Wernicke’s encephalopathy (WE) and make recommendations. Method Review of previously released data on some older products and parenteral thiamine use in some other countries; analysis of sales and adverse incident data on anaphylaxis for a contemporary parenteral product used in the UK, Pabrinex. Results It was difficult to estimate the incidence of related anaphylactic reactions to Pabrinex in the UK because the number of doses given is unknown. Sales data are only an approximation to doses given because for products with a limited shelf life not all product sold is administered. However, available data indicate that there have been 10 anaphylactic reactions to Pabrinex from between 5,431,235–6,651,947 patient-days (14,880–16,080 years) of treatment. Conclusion It is reasonable to assume that the risk of anaphylaxis is low, and lower than for many other drugs. The risk-benefit ratio for administration is favourable given the potential severity of brain damage in Wernicke–Korsakoff (WK) syndrome. There is a need for international agreement on the reporting of anaphylaxis and on the optimum thiamine therapy for the treatment of WK syndrome. We make recommendations on how this might be achieved.

Marchiafava-Bignami disease with haemophagocytic lymphohistiocytosis as a postoperative complication of cardiac surgery

Marchiafava-Bignami disease (MBD) is a rare complication of chronic alcoholism; however, MBD in a non-alcoholic diabetic patient has rarely been reported. The aetiology or pathophysiology of MBD is still unknown. A 50-year-old man with a history of untreated diabetes mellitus underwent on-pump beating coronary artery bypass graft surgery (CABG) surgery for three-vessel and left main coronary disease. 3 days after the surgery, he developed a fever over 40°C and entered a coma state. MRI revealed multiple lesions, including in the corpus callosum, globus pallidus, brain stem and upper cervical spinal cord, which suggested MBD. The patient did not respond to thiamine therapy, but partly responded to steroid therapy. He ultimately died of respiratory failure. The autopsy revealed MBD and haemophagocytic lymphohistiocytosis. It is rare, but systemic inflammatory response syndrome induced by on-pump beating CABG could develop these complication.