Putamina involvement in Wernicke encephalopathy induced by Janus Kinase 2 inhibitor.

OBJECTIVE: The aim of the study was to report a case of Wernicke encephalopathy (WE) due to fedratinib (Janus Kinase 2 inhibitor) treatment with atypical neuroimaging findings. METHODS: We present a detailed report of the case and literature review. RESULTS: A 68-year-old woman under treatment with fedratinib (investigational JAK2 inhibitor) developed memory impairment, diplopia, and ataxia compatible with WE. Brain magnetic resonance imaging showed extensive lesions involving medial thalami, periaqueductal gray, caudate nuclei, and putamina. Thiamine supplementation provided clinical recovery and radiological improvement of the lesions described. Basal ganglia lesions have been previously described in children with this disease, but this is rarely found in adults. Clinical trials including fedratinib have been recently discontinued, and its involvement in pathogenesis of WE may be related to thiamine-transporter inhibition. CONCLUSIONS: Our case represents an example of drug-related WE, with a rare radiological pattern. Precocious diagnosis and treatment are essential to prevent irreversible brain injury.

Wernicke-Korsakoff syndrome in Sydney hospitals after 6 years of thiamin enrichment of bread.

OBJECTIVES: To estimate the incidence of Wernicke's encephalopathy (WE) and Korsakoff's psychosis (KP) before and after the introduction of thiamin enrichment of bread in Australia. DESIGN AND SUBJECTS: Inpatient records were examined in 17 major public general hospitals in greater Sydney for the 4 years 1993-96 (inclusive) with the International Classification of Diseases (ICD) 9 diagnoses 265.1 (WE), 291.1 and 294.0 (KP). Relevant clinical data were recorded on a specially designed form so that cases could be classified as confirmed or probable WE, confirmed or probable KP, confirmed or probable Wernicke-Korsakoff syndrome (WE + KP) or not WE or KP. The average number of cases of WE + KP was 38 acute (new) cases and 69 total (acute + chronic) cases per annum for all the hospitals combined. RESULTS: This study used the same methods as our earlier retrospective examination of Wernicke-Korsakoff cases in essentially the same hospitals for 1978-93. Records for 1993 were thus pulled twice and, while individual cases (using hospital index number) did not always coincide, the total numbers for 1993 were 69 and 70. For the 5 years after 1991 the number of acute cases of WE and KP in Sydney hospitals was 61% of the number for the 5 years before 1991 (P<0.01). There is, however, no continuing downward trend. CONCLUSIONS: These results are consistent with a 40% reduction of the incidence of acute WE and KP since bread has been enriched with thiamin. The disease complex has, however, not been eliminated. To achieve this, further public health action would be needed, such as addition of thiamin to beer.

Thiamin in beer: a health promotion perspective.

Wernicke's encephalopathy (WE) is a serious disease resulting from excessive alcohol intake. As well as the disastrous consequences for the individual, patients with WE place a heavy burden for continuing care on the community. Australia appears to have a higher rate of WE than other western countries, probably due to differences in the consumption patterns of alcoholic beverages. Unlike most other western countries, Australia does not fortify flour with thiamin. On the other hand the USA does fortify flour and has higher thiamin intakes and lower rates of WE. In Australia it has been proposed that thiamin be added to alcoholic beverages. Given that technical problems related to stability, taste and absorption can be solved, the fortification of a substance which is potentially harmful, raises serious problems for health educators. The labeling of alcoholic beverages as 'vitamin enriched' could result in changes in the community's beliefs about alcohol and in increased alcohol consumption. The nutrition and education implications of this proposal are discussed. The conclusion is drawn that Australia should follow the example of other western countries and fortify flour with thiamin.