Paper 2
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw in The Final FRCR, 2020
Wernicke encephalopathy is caused by thiamine (vitamin B1) deficiency and is typically seen in alcoholics or those who self-neglect. It presents with the classic triad of confusion, ataxia and ophthalmoplegia. Korsakoff psychosis is the chronic form of the condition and is characterised by confabulation and memory loss. MRI findings include symmetrical high signal on T2 and FLAIR sequences with post-contrast enhancement in the mammillary bodies, basal ganglia, paraventricular/medial thalamic regions, brain stem and periaqueductal grey matter. Korsakoff syndrome is associated with mammillary body atrophy and dilatation of the third ventricle.
Korsakoff Syndrome
Alexander R. Toftness in Incredible Consequences of Brain Injury, 2023
Wernicke encephalopathy is caused by a lack of the vitamin thiamine, also called vitamin B1, which is one of the vitamins that the human body does not make and therefore must be obtained through the diet (Martin et al., 2003). The two main ways to develop a deficiency of thiamine are via excessive alcohol intake or via malnourishment, and these often overlap, with alcoholics often lacking proper nourishment. There are, however, other ways to get it, such as when a person receives weight loss surgery that makes it difficult for them to intake and absorb thiamine (Shahani et al., 2015).
Nutritional Diseases
Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang in Roxburgh's Common Skin Diseases, 2022
Management: Oral thiamine supplementation is used to manage beriberi with 5–30 mg per dose daily to 3 times daily (TID) depending on severity of illness. Wernicke encephalopathy is immediately managed with 500 mg IV thiamine TID in combination with other B vitamins. Glucose administration must follow thiamine supplementation in severely malnourished patients. Korsakoff encephalopathy is treated with oral vitamin B1 supplementation to help prevent further deterioration of mental status. Lifetime abstinence from alcohol is crucial in Wernicke-Korsakoff syndrome.
Red Flags in the Assessment of Adult Ophthalmoplegia
Published in Journal of Binocular Vision and Ocular Motility, 2018
Kimberly S. Merrill, Michael S. Lee, Collin M. McClelland
While starvation is rare in developed nations, medical conditions hindering nutrient absorption, alcohol abuse impairing healthy dietary choices, restrictive diets, and the rise of bariatric surgery all contribute to the risk of thiamine deficiency.10 Thiamine (vitamin B1) is a vital component of energy synthesis and deficiency leads to central nervous system manifestations collectively termed Wernicke encephalopathy.10 The classic triad of Wernicke encephalopathy includes eye movement abnormalities, confusion, and gait ataxia, although most patients do not present with all of these features, thus complicating early diagnosis. In a literature review of autopsy proven cases of Wernicke encephalopathy, 62 of 256 (24.2%) of patients exhibited ophthalmoplegia, nystagmus, or gaze palsy on examination.11
Therapeutic options for COVID-19: a quick review
Published in Journal of Chemotherapy, 2020
Muhammad Sani Ismaila, Faruku Bande, Aminu Ishaka, Aminatu Abubakar Sani, Karla Georges
Fedratinib (SAR302503, TG101348) is an antiviral JAK2 inhibitor drug that was tested in vitro and approved by FDA for myeloproliferative neoplasms. The drug got its first approval in August 2019 at the USA for the treatment of adult patients with intermediate-2 or high-risk primary or secondary myelofibrosis.77 It was tested on TH17 cell cytokine production.44 Fedratinib is specific for JAK2 but did not affect JAK1, JAK3, and TYK2. They found out that Fedratinib treatment decreased the expression of IL-17 by murine TH17 cells. Fedratinib has been reported to have an effect in vitro on SARS-COVID though the regulation of JAK-STAT Signaling Pathway in cytokine release storm (CRS). In severe coronavirus disease 2019 (COVID-19), an increase in the level of cytokine release is observed, leading to increased interleukin (IL)-6, IL-2, IL-7, and IL-10 and severe inflammation.78 Although this drug showed a promising effect in SARS-COVID management. However, caution should be observed due to reported side effects in a clinical trial conducted to assess its safety in patients with myelofibrosis where treatment with fedratinib 500-mg caused anemia, gastrointestinal symptoms, and increased levels of liver transaminases, serum creatinine, and pancreatic enzymes. Encephalopathy was reported in 4 women who received fedratinib 500 mg/d. Wernicke encephalopathy was diagnosed using magnetic resonance imaging in 3 cases and suspected clinically in one case.79
Emerging drugs for the treatment of myelofibrosis: phase II & III clinical trials
Published in Expert Opinion on Emerging Drugs, 2021
Douglas Tremblay, Ronald Hoffman
Importantly, the development of fedratinib was halted as a result of four patients who experienced encephalopathy in the JAKARTA study, with imaging consistent with Wernicke encephalopathy in 3 patients [11]. Further review of 670 patients in the fedratinib program, 8 possible cases of Wernicke encephalopathy were identified. Only one was confirmed, with two additional likely cases and the remaining five either being either inconclusive or inconsistent with the diagnosis of Wernicke encephalopathy [42]. Therefore, this complication is likely very rare. Regardless, there is a black-box warning for Wernicke encephalopathy for US prescribing of fedratinib. Thiamine levels are recommended to be checked prior to starting treatment. Any encephalopathy should prompt immediate discontinuation of fedratinib and initiation thiamine supplementation. Many clinicians avoid this complication by prophylactically administering thiamine to patients receiving fedratinib.
Related Knowledge Centers
- Ataxia
- B Vitamins
- Central Nervous System
- Confusion
- Korsakoff Syndrome
- Neurological Disorder
- Ophthalmoparesis
- Thiamine
- Thiamine Deficiency
- Wernicke–Korsakoff Syndrome