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thiamine in neurology

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https://www.readbyqxmd.com/read/29770345/biotin-thiamine-responsive-basal-ganglia-disease-case-report-and-follow-up-of-a-patient-with-poor-compliance
#1
Muneera A Alabdulqader, Sumayah Al Hajjaj
Background: Biotin-thiamine-responsive basal ganglia disease (BTBGD) is a rare treatable autosomal recessive neurometabolic disorder characterized by progressive encephalopathy that eventually leads to severe disability and death if not treated with biotin and thiamine supplements. Objectives: We aimed to determine the optimal management of BTBGD presenting in acute encephalopathic episodes. Method: Case report. Results: An 8-year-old girl born to consanguineous parents was diagnosed with BTBGD at the age of 3 years after presenting with acute encephalopathy and ataxia...
2018: Child Neurology Open
https://www.readbyqxmd.com/read/29714153/thiamine-and-alcohol-for-brain-pathology-super-imposing-or-different-causative-factors-for-brain-damage
#2
Rita Moretti, Paola Caruso, Matteo Dal Ben, Silvia Gazzin, Claudio Tiribelli
Drinking more than the recommended limits is a worldwide emerging problem, difficult to circumscribe, and alcohol related brain damages are an under-recognized health problem. Alcohol-cognitive disruption can be considered as transient and recoverable if the alcohol consumption is limited and occasional; if not, it can progress to the so-called alcohol-related dementia (ARD), or to the Wernicke encephalopathy, or it can even induce the Korsakoff syndrome, an irreversible and long-lasting medical condition. ARD still remains poorly diagnosed and addressed, even if it had recently raised increased research interest...
April 2, 2018: Current Drug Abuse Reviews
https://www.readbyqxmd.com/read/29694308/severe-neurological-complication-following-adjustable-gastric-banding
#3
G Martines, N Musa, F Aquilino, P Capuano
AIM: In the last years with the increase of bariatric surgery, first of all as a result of new indications, a rise in the incidence of nutrient-related complications has been observed. Currently little is known about the impact of post-bariatric malnutrition and neurological complications. Wernicke's encephalopathy is a severe neurological syndrome which occurs as a result of thiamine deficiency. Wernicke-Korsakoff syndrome must be considered a serious neurological complication of bariatric surgery with significant morbidity and mortality, with rapidly progressing neurological symptoms, and must be treated immediately...
March 2018: Il Giornale di Chirurgia
https://www.readbyqxmd.com/read/29686552/myocardial-ischemia-in-an-adolescent-secondary-to-nutritional-thiamine-deficiency
#4
Cassandra Lefevre, Lea H Mallett, Lori Wick
Thiamine (vitamin B1) is an essential vitamin found in most food products in developed nations. Deficiencies in this crucial vitamin lead to diseases related to nutritional needs, metabolic demands, enzymatic function, and neurological inflammation. We present an 18-year-old patient with a history of nasopharyngeal carcinoma and total parenteral nutrition (TPN) dependence who experienced neurological manifestations and heart failure, likely due to underlying thiamine deficiency, dehydration, and vasodilation...
January 2018: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/29602891/high-dose-thiamine-and-essential-tremor
#5
Antonio Costantini
Essential tremor is a common neurological disease. The medical treatment of this affection currently involves the use of propranolol, primidone and other drugs. These drugs, however, are often not effective in reducing tremor and cause side effects in a large share of the patients treated. The treatment with intramuscular high-dose thiamine has led to a rapid, remarkable and persistent improvement of the symptoms in two patients with essential tremor. This result suggests the possibility that high doses of intramuscular thiamine may be an affordable alternative, highly effective and long-lasting medical treatment that has shown no relevant side effect...
March 30, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29596284/a-case-of-wernicke-encephalopathy-developing-after-ileal-bypass-surgery
#6
Tülay Kamaşak, Sibel Kul, Mustafa Tuşat, Nezir Ozgun, Ali Cansu
Wernicke encephalopathy is an acute neurological problem resulting from thiamine deficiency and manifesting with mental confusion, oculomotor dysfunction, and ataxia. It is associated with alcohol dependence in adults. Preparatory factors include hyperemesis gravidarum, prolonged diarrhea, prolonged parental nutrition without vitamin support, absorption disorders, anorexia, cancer, and chemotherapy. Failure to consider the clinical findings and preparatory factors of this disease, which is rare in children, can delay diagnosis...
March 28, 2018: Pediatric Emergency Care
https://www.readbyqxmd.com/read/29593640/vestibular-dysfunction-in-wernicke-s-encephalopathy-predominant-impairment-of-the-horizontal-semicircular-canals
#7
Seung-Han Lee, Sang-Hoon Kim, Ji-Min Kim, Alexander Andrea Tarnutzer
Background: Wernicke's encephalopathy (WE), a metabolic disorder due to thiamine deficiency, manifests with various neurological symptoms and signs. It has been known as a cause of vestibular dysfunction. Preliminary reports have proposed predominant involvement of the horizontal semicircular canals (HSCs). Objective: To better characterize the pattern of vestibular impairment in patients with WE using quantitative video head-impulse testing and to review the literature regarding this topic...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29545765/clinical-characteristics-and-etiology-of-bilateral-vestibular-loss-in-a-cohort-from-central-illinois
#8
Jorge C Kattah
Background: Previous series of bilateral vestibular loss (BVL) identified numerous etiologies, but surprisingly, a cause in a significant number of cases remains unknown. In an effort to understand possible etiology and management strategies, a global effort is currently in progress. Here, I contribute my 10-year experience with both acute and chronic BVL during the 2007-2017 decade. Methods: This is a retrospective review of the charts and EMR of patients diagnosed with BVL in the last 10 years...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29465574/simultaneous-acute-marchiafava-bignami-disease-and-central-pontine-myelinolysis-a-case-report-of-a-challenging-diagnosis
#9
Chun-Yi Tsai, Po-Kai Huang, Poyin Huang
RATIONALE: Marchiafava-Bignami disease (MBD) is a rare disease characterized by demyelination of the corpus callosum. It is most commonly seen in patients with chronic alcoholism. The clinical diagnosis of MBD can be difficult due to its nonspecific manifestation. Central pontine myelinolysis (CPM) occurs mostly as a complication of severe and prolonged hyponatremia, especially when corrected too rapidly. However, CPM can be associated with chronic alcoholism and its clinical presentation can be heterogeneous...
February 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29386493/-a-case-of-wernicke-encephalopathy-with-hypoacusia-and-mr-high-intensity-of-the-inferior-colliculi-that-normalized-after-thiamine-administration
#10
Takuma Nakamura, Keisuke Imai, Masashi Hamanaka, Hidekazu Yamazaki, Takehiro Yamada, Toshiki Mizuno
A 61-year-old man was admitted to our institution with progressive hypoacusia, double vision, and lightheadedness. Neurological examination on day 6 of his illness showed severe hypoacusia, mild confusion, ocular motility disorder, truncal ataxia and absence of a deep tendon reflex. MRI fluid-attenuated inversion recovery imaging revealed symmetrical high intensities in the tectum of the midbrain, involving the bilateral inferior colliculi and the bilateral medial thalami, which suggested Wernicke encephalopathy (WE)...
February 28, 2018: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/29373764/-wernicke-korsakoff-syndrome-secondary-to-cytomegalovirus-encephalitis-a-case-report
#11
Luis Guillermo Uribe, María Alejandra Pérez, Camilo Andrés Lara, Natalia Rueda, Javier Augusto Hernández
Cytomegalovirus (CMV) is one of the opportunistic microorganisms with the highest prevalence in immunocompromised patients. Reactivation has decreased after the introduction of highly active antiretroviral therapy (HAART). Encephalitis has been reported in the coinfection as one of the most frequent presentations.We present the case of a young adult patient with HIV infection and rapid neurological deterioration due to classic clinical symptoms and signs of the Wernicke-Korsakoff syndrome, with no risk factors for thiamine deficiency, with images by nuclear magnetic resonance typical of the syndrome, and identification of cytomegalovirus in cerebrospinal fluid...
December 1, 2017: Biomédica: Revista del Instituto Nacional de Salud
https://www.readbyqxmd.com/read/29360523/thiamine-and-cardiovascular-disease-a-literature-review
#12
REVIEW
James J DiNicolantonio, Jing Liu, James H O'Keefe
Thiamine is a water-soluble vitamin that plays an important role in the energy metabolism in the human body. Deficiency in thiamine can lead to neurological abnormalities and congestive heart failure (HF), known as dry beriberi and wet beriberi respectively. Several populations are at higher risk for thiamine deficiency, most notably persons with chronic alcoholism. This article aims to provide a review of current literature on the role of thiamine in the human body, the current scope of thiamine deficiency, and explore the specific effects of thiamine deficiency and supplementation on the cardiovascular system...
January 31, 2018: Progress in Cardiovascular Diseases
https://www.readbyqxmd.com/read/29243370/neurological-nutritional-and-alcohol-consumption-factors-underlie-cognitive-and-motor-deficits-in-chronic-alcoholism
#13
Rosemary Fama, Anne-Pascale Le Berre, Cheshire Hardcastle, Stephanie A Sassoon, Adolf Pfefferbaum, Edith V Sullivan, Natalie M Zahr
Variations in pattern and extent of cognitive and motor impairment occur in alcoholism (ALC). Causes of such heterogeneity are elusive and inconsistently accounted for by demographic or alcohol consumption differences. We examined neurological and nutritional factors as possible contributors to heterogeneity in impairment. Participants with ALC (n = 96) and a normal comparison group (n = 41) were examined on six cognitive and motor domains. Signs of historically determined subclinical Wernicke's encephalopathy were detected using the Caine et al...
December 15, 2017: Addiction Biology
https://www.readbyqxmd.com/read/29128903/impairment-of-thiamine-transport-at-the-gut-bbb-axis-contributes-to-wernicke-s-encephalopathy
#14
P M Abdul-Muneer, Saleena Alikunju, Heather Schuetz, Adam M Szlachetka, Xiaotang Ma, James Haorah
Wernicke's encephalopathy, a common neurological disease, is caused by thiamine (vitamin B1) deficiency. Neuropathy resulting from thiamine deficiency is a hallmark of Wernicke-Korsakoff syndrome in chronic alcohol users. The underlying mechanisms of this deficiency and progression of neuropathy remain to be understood. To uncover the unknown mechanisms of thiamine deficiency in alcohol abuse, we used chronic alcohol consumption or thiamine deficiency diet ingestion in animal models. Observations from animal models were validated in primary human neuronal culture for neurodegenerative process...
November 11, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/29045486/thiamine-deficiency-activates-hypoxia-inducible-factor-1%C3%AE-to-facilitate-pro-apoptotic-responses-in-mouse-primary-astrocytes
#15
Kristy Zera, Jason Zastre
Thiamine is an essential enzyme cofactor required for proper metabolic function and maintenance of metabolism and energy production in the brain. In developed countries, thiamine deficiency (TD) is most often manifested following chronic alcohol consumption leading to impaired mitochondrial function, oxidative stress, inflammation and excitotoxicity. These biochemical lesions result in apoptotic cell death in both neurons and astrocytes. Comparable histological injuries in patients with hypoxia/ischemia and TD have been described in the thalamus and mammillary bodies, suggesting a congruency between the cellular responses to these stresses...
2017: PloS One
https://www.readbyqxmd.com/read/28951891/thiamine-acquisition-strategies-impact-metabolism-and-competition-in-the-gut-microbe-bacteroides-thetaiotaomicron
#16
Zachary A Costliow, Patrick H Degnan
Thiamine (vitamin B1) is an essential cofactor for all organisms. Humans primarily acquire thiamine through their diet, and thiamine deficiencies have adverse neurological effects. However, the role gut microbes play in modulating thiamine availability is poorly understood, and little is known about how thiamine impacts the stability of microbial gut communities. To investigate thiamine's role in the gut, we utilized the model gut microbe Bacteroides thetaiotaomicron. Transcriptome sequencing (RNA-seq) revealed a global downregulation of thiamine and amino acid biosynthesis, glycolysis, and purine metabolism when thiamine was present...
September 2017: MSystems
https://www.readbyqxmd.com/read/28944253/psychological-assessment-of-patients-with-biotin-thiamine-responsive-basal-ganglia-disease
#17
Majid Alfadhel, Amal Al-Bluwi
Biotin-thiamine-responsive basal ganglia disease is a devastating autosomal recessive inherited neurological disorder. We conducted a retrospective chart review of all patients with biotin-thiamine-responsive basal ganglia disease who underwent a formal psychological assessment. Six females and 3 males were included. Five patients (56%) had an average IQ, two patients (22%) had mild delay, and two (22%) had severe delay. A normal outcome was directly related to the time of diagnosis and initiation of treatment...
January 2017: Child Neurology Open
https://www.readbyqxmd.com/read/28794205/the-human-serum-metabolome-of-vitamin-b-12-deficiency-and-repletion-and-associations-with-neurological-function-in-elderly-adults
#18
Alex Brito, Dmitry Grapov, Johannes Fahrmann, Danielle Harvey, Ralph Green, Joshua W Miller, Sergey N Fedosov, Setareh Shahab-Ferdows, Daniela Hampel, Theresa L Pedersen, Oliver Fiehn, John W Newman, Ricardo Uauy, Lindsay H Allen
Background: The specific metabolomic perturbations that occur in vitamin B-12 deficiency, and their associations with neurological function, are not well characterized.Objective: We sought to characterize the human serum metabolome in subclinical vitamin B-12 deficiency and repletion.Methods: A before-and-after treatment study provided 1 injection of 10 mg vitamin B-12 (with 100 mg pyridoxine and 100 mg thiamin) to 27 community-dwelling elderly Chileans (∼74 y old) with vitamin B-12 deficiency, as evaluated with serum vitamin B-12, total plasma homocysteine (tHcy), methylmalonic acid (MMA), and holotranscobalamin...
August 9, 2017: Journal of Nutrition
https://www.readbyqxmd.com/read/28765488/wernicke%C3%A3-%C3%A2-%C3%A2-s-encephalopathy-associated-with-liver-abscess
#19
Rajesh Verma, Vipul Garg
Wernicke's encephalopathy is a rare neurological disorder caused by thiamine deficiency, characterised by ocular motor dysfunction, ataxia and impairment in consciousness. It predominantly affects brain regions with a high metabolic rate such as mammillary bodies, medial thalamic nuclei, the tectal region and the cerebellum. Although chronic alcoholism is the most common cause of Wernicke's encephalopathy, various other conditions not related to alcohol consumption such as bariatric surgery, acute pancreatitis, hyperemesis gravidarum, prolonged fasting and gastrointestinal surgery have been implicated in its aetiology...
July 31, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28706281/thiamine-metabolism-is-critical-for-regulating-correlated-growth-of-dendrite-arbors-and-neuronal-somata
#20
Huimin Liu, Shaoming Sang, Yuan Lu, Zhongfeng Wang, Xiang Yu, Chunjiu Zhong
Thiamine is critical for cellular function, as its phosphorylated and active form, thiamine diphosphate (TDP), acts as coenzyme for three key enzymes in glucose metabolism. Mutations in thiamine transporter, TDP synthesizing enzyme or carrier, including solute carrier family 19 member 3 (SLC19A3), thiamine pyrophosphokinase (TPK1) and solute carrier family 25 member 19 (SLC25A19), have been associated with developmental neurological disorders, including microcephaly and Leigh syndrome. However, little is known about how thiamine metabolism regulates neuronal morphology at the cellular level...
July 13, 2017: Scientific Reports
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