The level of vitamin B1 in 20 patients with the mild and typical the "numb-unsteady" syndrome and 20 healthy persons with ages of 20-50 was quantified during 10-12/1999. The participants did use the vitamin B1 within the previous 3 weeks. The level of vitamin B1 was quantified by the high performance liquid chromatography (HPLC). The results have shown that the level of blood vitamin B1 in 100% of participants was lower than normal level. Although patients used continuously the vitamin B1 at high dose, the blood vitamin B1 has still lower than normal range and control. The level of vitamin B1, B2 and PP in the diet of both patients and control groups did not meet the wanted level
Thiamine ; Hypesthesia

Pyruvate dehydrogenase complex (PDHC) deficiency is mostly due to mutations in the X-linked E1alpha subunit gene (PDHA1). Some of the patients with PDHC deficiency showed clinical improvements with thiamine treatment. We report the results of biochemical and molecular analysis in a female patient with lactic acidemia. The PDHC activity was assayed at different concentrations of thiamine pyrophosphate (TPP). The PDHC activity showed null activity at low TPP concentration (1 x 10(-3) mM), but significantly increased at a high TPP concentration (1 mM). Sequencing analysis of PDHA1 gene of the patient revealed a substitution of cysteine for tyrosine at position 161 (Y161C). Thiamine treatment resulted in reduction of the patient's serum lactate concentration and dramatic clinical improvement. Biochemical, molecular, and clinical data suggest that this patient has a thiamine-responsive PDHC deficiency due to a novel mutation, Y161C. Therefore, to detect the thiamine responsiveness it is necessary to measure activities of PDHC not only at high but also at low concentration of TPP.
Thiamine Pyrophosphate/metabolism ; Thiamine/*therapeutic use ; Pyruvate Dehydrogenase Complex Deficiency Disease/drug therapy/*genetics ; Pyruvate Dehydrogenase (Lipoamide)/*genetics ; *Point Mutation ; Infant, Newborn ; Humans ; Female ; Cells, Cultured

Thiamine deficiency is known to lead to certain neurological sequelae including Wernicke-Korsakoff syndrome. Signs attributable to this condition include ataxia, ophthalmoplegia, nystagmus, and mental confusion. The prompt use of thiamine prevents progression of the disease and reverses those lesions that have not yet progressed to the point of fixed structual change. We have experienced a case of Wernicke-Korsakoff syndrome associated with hyperemesis gravidarum, which seemed to be developed by prolonged thiamine-free dextrose therapy, we emphasise the need for thiamine supplementation in hyperemesis gravidarum patient.
Ataxia ; Female ; Glucose ; Humans ; Hyperemesis Gravidarum* ; Korsakoff Syndrome* ; Ophthalmoplegia ; Pregnancy ; Thiamine ; Thiamine Deficiency

Wernicke's encephalopathy, characterized by ataxia, nystagmus, confusion, due to thiamine deficiency, can be a lethal disease without appropriate treatment such as thiamine supplement. The thiamine defect could be from poor oral intake because of hyperemesis gravidarum as well as alcoholic disease. Radiologically, Wernicke's encephalopathy presents the abnormality around the 3rd and the 4th ventricle and periaqueductal area. We now present the case of Wernicke's encephalopathy associated with hyperemesis gravidarum with the radiologic finding and reviewing other literature.
Alcoholics ; Ataxia ; Female ; Humans ; Hyperemesis Gravidarum* ; Pregnancy ; Thiamine ; Thiamine Deficiency ; Wernicke Encephalopathy*

Wernicke's encephalopathy is an acute-onset illness characterized by mental confusion, ataxia and ophthalmoplegia due to a thiamine deficiency. Immediate administration of thiamine prevents progression of the disease and reverses brain lesions. We have experienced a case of Wernicke's encephalopathy associated with hyperemesis gravidarum. As Wernicke's encephalopathy is mostly associated with chronic alcoholism, the possibility of Wernicke's encephalopathy may be ignored in young women. We emphasize the need for thiamine supplementation in the patient requiring intravenous alimentation such as hyperemesis gravidarum.
Alcoholism ; Ataxia ; Brain ; Female ; Humans ; Hyperemesis Gravidarum* ; Ophthalmoplegia ; Pregnancy ; Thiamine ; Thiamine Deficiency ; Wernicke Encephalopathy*

Alcohol-induced cognitive disorder is a very severe problem in problem alcohol drinker and alcohol itself seems to be one of the main causalities in the development of senile dementia. However, the spectrum of alcohol induced cognitive disorder is quite broad, for example, it covered from alcohol-induced persistent amnestic disorder to Wernicke-Korsakoff syndrome and alcohol-induced persistent dementia. By that reason, broad spectrum of cognitive impairment by excessive alcohol drinking is regarded as alcohol related dementia. The pharmacological treatment is not well established yet in alcohol related dementia, except Wernicke-Korsakoff syndrome which is definitely related to thiamine deficiency. Therefore we introduced that some reports about the clinical efficacies by rivastigmine or donepezil trial and recent outcomes of memantine trial by authors in this review.
Alcohol Drinking ; Alzheimer Disease ; Dementia ; Indans ; Korsakoff Syndrome ; Memantine ; Phenylcarbamates ; Piperidines ; Rivastigmine ; Thiamine ; Thiamine Deficiency

Wernicke's encephalopathy (WE) is an acute neuropsychiatric syndrome resulting from thiamine deficiency. Traditionally, diagnosis of WE rests on a clinical symptom triad consisting of ocular signs, altered consciousness, and ataxia. However, the complete triad is only present in a fraction of cases, which means that WE tends to be under-diagnosed, especially in nonalcoholic patients. Brain MRI of WE patients usually shows symmetrical signal intensity alterations in the thalami, mammillary bodies, and periaqueductal area, because of cytotoxic edema in the same region. These typical findings are useful diagnostic leads in WE patients with atypical symptoms. However, atypical findings can occasionally be seen in the vermis of cerebellum and cerebellar nuclei. Notably, alterations of signal intensity in the cerebellar dentate nuclei, which is a typical finding of metronidazole-induced encephalopathy (MIE), need to be distinguished according to medication history and response to thiamine.
Ataxia ; Brain ; Cerebellar Nuclei ; Cerebellum ; Consciousness ; Edema ; Humans ; Mamillary Bodies ; Metronidazole ; Thiamine ; Thiamine Deficiency ; Wernicke Encephalopathy

Thiamine deficiency can cause peripheral polyneuropathy and Wernicke's encephalopathy. Wernicke's encephalopathy is characterized by ataxia, ophthalmoplegia, nystagmus, and confusion, and typically presents acute and rapidly progressive course, whereas peripheral neuropathy associated with thiamine deficiency manifests chronic and slowly progressive one. However, acute and rapidly progressive axonal polyneuropathy combined with Wernicke's encephalopathy is quite rare and unusual. Here, we describe a patient with Wernicke's encephalopathy who presented with acute bilateral axonal neuropathy.
Ataxia ; Axons ; Humans ; Ophthalmoplegia ; Peripheral Nervous System Diseases ; Polyneuropathies ; Thiamine ; Thiamine Deficiency ; Wernicke Encephalopathy* ; Wrist*

Thiamine (vitamin B1) serves as an important cofactor in body metabolism and energy production. It is related with the biosynthesis of neurotransmitters and the production of substances used in defense against oxidant stress. Thus, a lack of thiamine affects several organ systems, in particular the cardiovascular and nervous system. The cardiac insufficiency caused by thiamine deficiency is known as cardiac beriberi, with this condition resulting from unbalanced nutrition and chronic excessive alcohol intake. Given that the disease is now very rare in developed nations such as Korea, it is frequently missed by cardiologists, with potentially fatal consequences. Herein, we present a case study in order to draw attention to cardiac beriberi. We believe that this case will be helpful for young cardiologists, reminding them of the importance of this forgotten but memorable disease.
Beriberi ; Cardiovascular System ; Developed Countries ; Korea ; Nervous System ; Thiamine ; Thiamine Deficiency

Wernicke's encephalopathy is caused by thiamine deficiency, and is characterized by acute mental confusion, ataxia, and ophthalmoplegia. It is also a rare neurologic complication of hematopoietic stem cell transplantation (HSCT). However, because of its rare incidence, Wernicke's encephalopathy can easily be overlooked in HSCT patients, and a few misleading steps in the early stage of the disease may result in permanent neurologic disability or even mortality. We recently encountered a case of Wernicke's encephalopathy in a patient who underwent allogeneic HSCT. Based on our own experience and previously published documents, we suggest early radiologic surveillance and treatment for patients with findings compatible with Wernicke's encephalopathy following HSCT.
Ataxia ; Hematopoietic Stem Cell Transplantation ; Hematopoietic Stem Cells ; Humans ; Incidence ; Ophthalmoplegia ; Thiamine ; Thiamine Deficiency ; Wernicke Encephalopathy