Long-Term Mortality of Patients with an Alcohol-Related Wernicke-Korsakoff Syndrome.

AIMS: To characterize a series of contemporary patients with alcohol-related Wernicke's encephalopathy (WE) or Korsakoff's syndrome (KS) and to update the current prognosis of disease. METHODS: Retrospective and prospective study of patients diagnosed with an alcohol-related WE or KS between 2002 and 2011 in a tertiary hospital. Socio-demographic, alcohol use characteristics, signs and symptoms, co-morbidity and blood parameters were obtained at admission. Patients were followed up until 2013 and causes of death were ascertained through the review of charts. RESULTS: Sixty-one patients were included (51 with WE and 10 with KS). Among patients with WE, 78% were men and age at diagnosis was 57 years (interquartile range (IQR): 49-66). Twenty-three percent fulfilled the classic WE triad. Regarding Caine's criteria for WE, 70.6% presented with at least two out of four signs or symptoms. Median follow-up of patients with WE syndrome was 5.3 years (IQR: 2.6-8.8), the cumulated mortality was 45% and death rate of 7.4 × 100 person-years (95% confidence interval (CI): 4.8-10.9). Overall, 50% of patients would be expected to die within 8 years of WE episode and main causes of death included serious bacterial infections (44.5%) and cancer (33.3%). CONCLUSIONS: Survival of patients with an alcohol-related Wernicke-Korsakoff syndrome is poor; pursuing treatment of alcohol use disorder and early diagnosis of thiamine deficiency is a priority for improving clinical outcomes.

Wernicke's encephalopathy: a preventable cause of maternal death.

Wernicke's encephalopathy is a rare cause of maternal death. It is a difficult diagnosis to make but prevention and treatment is straightforward. Severe thiamine deficiency causes Wernicke-Korsakoff syndrome. Correct diagnosis and treatment with thiamine will decrease the case fatality rate.

[Gayet-Wernicke's encephalopathy. A study of 13 cases observed in a refugee population hospitalized at the Conakry Teaching Hospital]. / Encéphalopathie de Gayet-Wernicke. Etude de 13 observations dans une population de patients réfugiés hospitalisés pour affections neurologiques au CHU de Conakry.

The authors report 13 cases of Gayet-Wernicke's encephalopathy observed in 13 patients of a refugee population. 11 presented the classical triad: oculomotor signs, cerebral ataxia and state of confusion and in 2 patients, only 2 symptoms were noted. The etiological factors: chronic alcoholism, malnutrition, uncontrollable vomiting, HIV and tuberculosis were identified. The outcome was evaluated on the basis of the disappearance of symptoms after treatment with 500 mg of thiamine in 7 patients, 1 death and 5 patients progressed toward Korsakoff amnesic syndrome.

Parenteral thiamine and Wernicke's encephalopathy: the balance of risks and perception of concern.

Wernicke's encephalopathy, a disorder with significant mortality and high morbidity, is common amongst alcohol-dependent patients. Thiamine deficiency appears to play a key role in its aetiology, and parenteral high-dose thiamine is effective in prophylaxis and treatment. Unfortunately, reports of rare anaphylactoid reactions have led to a dramatic reduction in the use of parenteral thiamine, and it is possible that this change in treatment has led, or will lead, to an increase in morbidity and mortality. There is a need for education of doctors who treat alcohol-dependent patients, in order to ensure appropriate use of parenteral thiamine in prophylaxis and treatment of this disorder.

Coma and death in unrecognized Wernicke's encephalopathy: an autopsy study

Numa série de 36 casos de encefalopatia de Wenicke autopsiados, 11 haviam desenvolvido coma. Em nenhum desses pacientes comatosos o diagnóstico foi estabelecido durante a vida. Seis desses pacientes eram homens e 5 mulheres, com idades entre 26 a 50 anos (média de 36,6). História de alcoolismo crônico foi positiva em 7 casos, sinais de desnutriçäo grave ocorreram em 3; uma paciente apresentava câncer gástrico e uma, hiperemese gravídica. Dois pacientes foram admitidos ao hospital após terem sido encontrados em coma em suas residências. O exame neuropatológico revelou alteraçöes macroscópicas nos corpos mamilares em 8 casos - atrofia, descoloraçäo e espongiose. Atrofia da porçäo anterior e superior do vermis do cerebelo foi observada em um caso. Em 5 casos havia hemorragias petequiais no diencáfalo, principalmente próximas às paredes do terceiro ventrículo. A microscopia revelou além das hemorragias, proliferaçäo glial, hipertrofia do endotélio vascular e necrose de neurônios e mielina. Mielinólise pontina central ocorreu em um paciente. A encefalopatia de Wernicke é pouco diagnosticada clinicamente. O coma pode mascarar as outras manifestaçöes clínicas da doença ou pode constituir sua única manfiestaçäo. Embora seja um sinal de mau prognóstico, o coma pode ser revertido pela administraçäo de tiamina. É enfatizado que todo paciente em coma de causa näo estabelecida deve receber altas doses de tiamina

Coma and death in unrecognized Wernicke's encephalopathy. An autopsy study.

Eleven out of 36 autopsied cases of Wernicke's encephalopathy had developed coma. None of these patients had the diagnosis during life. There were six men and five women with ages ranging from 26 to 50 years (mean 36.6). Seven of these patients were heavy drinkers, three exhibited signs of severe malnutrition, whereas one was being evaluated for a disseminated gastric cancer and one was in treatment of hyperemesis gravidarum. Two patients were brought to the hospital after found unconscious at home. Neuropathological examination disclosed gross changes in the mammillary bodies in eight cases and microscopic changes in all cases. In one case there was atrophy of the anterior superior part of the vermis. Petechial hemorrhages were observed particularly in the walls of the third ventricle. Microscopically there were in addition to hemorrhages, glial proliferation, endothelial hypertrophy and necrosis of nerve cells and myelin. Central pontine myelinolysis was observed in one case. Wernicke's encephalopathy is a clinically underdiagnosed condition. Coma may mask its classical clinical picture or even be the sole manifestation. Although coma points to a poor outlook it may be reversed by thiamine administration. Any patient with coma of unknown etiology should be given parenteral thiamine.

The prevalence of the Wernicke-Korsakoff syndrome in Sydney, Australia: a prospective necropsy study.

In a prospective necropsy study, the prevalence of the Wernicke-Korsakoff syndrome (WKS) in Sydney, Australia was 2.1% of adults over the age of 15 years. The population studied encompassed a wide spectrum of socio-economic and cultural backgrounds. Abuse of alcohol appeared to be the major predisposing factor to the development of the WKS in cases which were adequately documented. This high prevalence rate is in line with other clinical and pathological Australian studies and provides additional support for the idea of prevention of the WKS by the use of thiamin supplements in the Australian diet in flour, bread and perhaps alcoholic beverages.