Abstract:
:Rare metabolic diseases may sometimes arise acutely and endanger human life if not immediately recognized and treated. Marchiafava Bignami disease is an uncommon neurologic disorder described in alcohol abusers and characterized by an acute severe damage of brain white matter. Even more rarely, it has been reported in non-alcohol addicted patients, but never in vegetarian people. This is a case report of a young vegetarian woman, accustomed to drink high amounts of tea, who, three weeks after her first natural childbirth, developed serious motor and cognitive disturbances. A timely brain magnetic resonance (MR) allowed us to identify Marchiafava Bignami disease and she healed few hours after the administration of parenteral steroids and vitamins. We advise to suspect Marchiafava Bignami Disease in all patients presenting with non-obvious acute generalized motor and cognitive disturbances, also if non alcoholics, and to collect the nutritional habits in all patients with suspected symptoms. In these cases a timely brain MRI is warranted, since brain imaging is typical and patients may recover after a prompt treatment.
journal_name
Metab Brain Disjournal_title
Metabolic brain diseaseauthors
Fiacco F,Barbato L,Pecoraro MG,Maggio Pdoi
10.1007/s11011-016-9893-zsubject
Has Abstractpub_date
2017-02-01 00:00:00pages
271-274issue
1eissn
0885-7490issn
1573-7365pii
10.1007/s11011-016-9893-zjournal_volume
32pub_type
杂志文章abstract::Glutaric aciduria type 1 is a neurometabolic disorder, caused by riboflavin-dependent glutaryl-CoA dehydrogenase deficiency. As its consequence, accumulation of the putatively neurotoxic metabolites (glutaric and 3-hydroxyglutaric acids) in body tissues, but especially within the brain, is observed. Estimated incidenc...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-018-0357-5
更新日期:2019-04-01 00:00:00
abstract::In view of the protective effect of brain derived neurotrophic factor (BDNF) against metabolic/excitotoxic insults in vitro, we investigated whether BDNF could limit infarct size after permanent occlusion of the middle cerebral artery in rat (MCAO). BDNF was delivered into the territory of the middle cerebral artery v...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/BF02674671
更新日期:1997-12-01 00:00:00
abstract::While risk factors for konzo are known, determinants of cognitive impairment in konzo-affected children remain unknown. We anchored cognitive performance (KABC-II scores) to serum levels of free-thyroxine (free-T4), thyroid-stimulating hormone (TSH), albumin, and motor proficiency (BOT-2 scores) in 40 children includi...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-014-9492-9
更新日期:2014-06-01 00:00:00
abstract::Schizophrenia is known to be associated with metabolic disturbances including diabetes mellitus, obesity and cardiovascular diseases. A growing body of evidence has suggested abnormal cytokine levels in schizophrenia. In the present study, we explored the effects of low-grade chronic inflammation on behavioral stereot...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-014-9630-4
更新日期:2015-06-01 00:00:00
abstract::Cyclic AMP is part of an endogenous mechanism that downregulates inflammatory response, and its intracellular concentration is regulated chiefly by cyclic nucleotide phosphodiesterases type 4. The goal of the present study was to determine whether phosphodiesterases 4 are involved in the inflammatory response of astro...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-009-9150-9
更新日期:2009-09-01 00:00:00
abstract::The aim of the present work was to investigate the role of ondansetron on the high fat diet (HFD) induced obese mice for behavioral and biochemical alterations using chronic unpredictable mild stress (CUMS) model of depression. Animals were fed with high fat diet for 14 weeks and subjected to different stress procedur...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-014-9574-8
更新日期:2014-09-01 00:00:00
abstract::In patients with hepatic encephalopathy (HE) the blood concentration of ammonia is usually highly elevated. Ammonia readily enters brain cells from the blood, and toxic effects of ammonia on brain metabolism and neurotransmission are believed to play a key role in the pathogenesis of HE. It has, however, been a matter...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-008-9126-1
更新日期:2009-03-01 00:00:00
abstract::Hypoxic-ischemia (HI) is a widely used animal model to mimic the preterm or perinatal sublethal hypoxia, including hypoxic-ischemic encephalopathy. It causes diffuse neurodegeneration in the brain and results in mental retardation, hyperactivity, cerebral palsy, epilepsy and neuroendocrine disturbances. Herein, we exa...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-016-9816-z
更新日期:2016-08-01 00:00:00
abstract::There is evidence to suggest that, in acute liver failure (ALF), brain ammonia and proinflammatory cytokines may act synergistically to cause brain edema and its complications (intracranial hypertension, brain herniation). However, the molecular mechanisms involved remain to be established. In order to address this is...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-010-9185-y
更新日期:2010-03-01 00:00:00
abstract::α-asarone (ASR) is a major bioactive compound isolated from the rhizome of Acorus tatarinowii Schott and it has extensive biological effects. Clinically, anxiety disorder is a common comorbidity of chronic pain. However, limited information is available regarding the effects of ASR on chronic pain-related anxiety. Thi...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0108-z
更新日期:2017-12-01 00:00:00
abstract::At the present time alcoholism is recognized as a metabolic disease exhibiting the clinical features of craving for alcohol, loss of control over drinking, tolerance and physical dependence on alcohol, while both epidemiological and experimental studies have demonstrated that genetic factors may be important in determ...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/BF01999765
更新日期:1994-06-01 00:00:00
abstract::To characterize an Egyptian patient with glutaric acidemia type I (GA I) and to identify the causative mutation(s) that may be responsible for the disease phenotype. MRI was performed on the patient using the 1.5 T magnet, biochemical analysis was carried out using gas chromatography/mass spectrometry on the patient's...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-016-9879-x
更新日期:2017-02-01 00:00:00
abstract::Iron is an important micronutrient for neuronal function and survival. It plays an essential role in DNA and protein synthesis, neurotransmission and electron transport chain due to its dual redox states. On the contrary, iron also catalyses the production of free radicals and hence, causes oxidative stress. Therefore...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/s11011-014-9522-7
更新日期:2014-09-01 00:00:00
abstract::Traumatic brain injury (TBI) is accompanied with enhanced matrix metalloproteinase-9 (MMP-9) activity and elevated levels of plasma fibrinogen (Fg), which is a known inflammatory agent. Activation of MMP-9 and increase in blood content of Fg (i.e. hyperfibrinogenemia, HFg) both contribute to cerebrovascular disorders ...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-014-9550-3
更新日期:2015-04-01 00:00:00
abstract::Substantial evidence demonstrated that maternal dietary nutrients can significantly determine the susceptibility to developing metabolic disorders in the offspring. Therefore, we aimed to investigate the later-life effects of maternal and postweaning diets interaction on epigenetic modification of the central nervous ...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-015-9678-9
更新日期:2015-10-01 00:00:00
abstract::Sleep disturbances are prevalent among patients with Alzheimer's disease (AD) and often precede the onset and progression of dementia. However, there are no reliable animal models for investigating sleep disturbances in patients with sporadic AD (sAD), which accounts for more than 90% of all AD cases. In the present s...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0125-y
更新日期:2018-02-01 00:00:00
abstract::Because of the importance of cholesterol metabolism in the physiopathogenesis of dementia, and knowing the function of ATP-binding cassette A1 transporter (ABCA1) as a cholesterol flow pump at the cellular and cerebral level, it has been noted that the ABCA1 gene may be a good candidate for disease study. In order to ...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-020-00577-4
更新日期:2020-08-01 00:00:00
abstract::The lifetime prevalence rate for major depressive disorder (MDD) is approximately 17 % for most developed countries around the world. Dietary polyphenols are currently used as an adjuvant therapy to accelerate the therapeutic efficacy on depression. Ferulic acid (FA) or 4-hydroxy-3-methoxy-cinnamic acid (Fig. 1a) is a...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-015-9704-y
更新日期:2015-12-01 00:00:00
abstract::Homocystinuria is an inborn error of sulfur amino acid metabolism characterized predominantly by vascular and nervous system dysfunction. In this study we determined the in vitro effects of homocysteine and methionine, metabolites which accumulate in homocystinuria, on Na+, K+-ATPase, and Mg2+-ATPase activities in syn...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1023/a:1015594111778
更新日期:2002-06-01 00:00:00
abstract::The regional energy status and the availability of metabolic substrates during brain development are important, since a variety of fetal metabolic insults have been increasingly implicated in the evolution of neonatal brain disorders. The response of the brain to a metabolic insult is determined, in large part, by the...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1023/a:1025503115837
更新日期:2003-09-01 00:00:00
abstract::The integrity of the blood-brain barrier (BBB) is an important aspect of normal central nervous system (CNS) function. Recently, it was shown that the BBB breakdown is one of the predisposing factors in the pathogenesis of thiamine-deficiency encephalopathy. The result is discussed along with some reviews on previous ...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/BF02080931
更新日期:1996-03-01 00:00:00
abstract::Antipsychotics, such as risperidone, increase food intake and induce alteration in glucose and lipid metabolism concomitantly with overweight and body fat increase, these biological abnormalities belong to the metabolic syndrome definition (high visceral adiposity, hypertriglyceridemia, hyperglycemia, low HDL-choleste...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0133-y
更新日期:2018-02-01 00:00:00
abstract::Tissue methylmalonic acid (MMA) accumulation is the biochemical hallmark of methylmalonic acidemia. Clinically, the disease is characterized by progressive neurological deterioration and renal failure, whose pathophysiology is still undefined. In the present study we investigated the effect of acute MMA administration...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-013-9396-0
更新日期:2013-09-01 00:00:00
abstract::Transient bilateral common carotid artery occlusion (tBCCAO), followed by reperfusion, is a model of transient global hypoperfusion. In the present study we aimed to investigate the probable effects of Vanillic acid (VA) on some physiological parameters including cerebral hyperemia, blood-brain barrier (BBB) disruptio...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-018-0187-5
更新日期:2018-06-01 00:00:00
abstract::Increasing evidence indicates that fetal metabolic stress may result in a variety of post-natal perturbations during brain development. The goal of the study was to determine the duration of hypoxia/ischemia that would elicit a demonstrable regional depression of metabolism in the fetal brain and further to examine se...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-006-9031-4
更新日期:2006-12-01 00:00:00
abstract::Depression, common in chronic medical conditions, and hepatic encephalopathy (HE), a reversible neuropsychiatric syndrome due to liver dysfunction, are associated with impaired health-related quality of life (HRQOL) in cirrhosis and hepatitis C (HCV). This study investigated the impact of depression and HE on HRQOL in...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-016-9817-y
更新日期:2016-08-01 00:00:00
abstract::Spontaneous intracerebral hemorrhage (ICH) represents a partially-understood cerebrovascular disease of high incidence, morbidity and mortality. We, herein, report the findings of our study concerning the role of two important adenosinetriphosphatases (ATPases) in a porcine model of spontaneous ICH that we have recent...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-013-9380-8
更新日期:2013-09-01 00:00:00
abstract::Several studies have examined neonatal diabetes, a rare disease characterized by hyperglycemia and low insulin levels that is usually diagnosed in the first 6 month of life. Recently, the effects of diabetes on the brain have received considerable attention. In addition, hyperglycemia may perturb brain function and mi...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0170-6
更新日期:2018-02-01 00:00:00
abstract::Glutamate neurotoxicity was studied in primary neuronal cultures prepared from rat cerebral cortex and hippocampal CA1 sector. Neurons were cultivated with 5% native horse serum and then exposed to 0.1 or 1.0 mM glutamate for 5 min. Subsequently, neurons were allowed to recover for 24 hours either in the presence or i...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/BF02098880
更新日期:1994-12-01 00:00:00
abstract::Acute and chronic administration of Al-gluconate (12.7% Al) at the concentration of 1 mg/kg produces edema in the rat brain, as reflected by the increase in water and Na+ content. The permeability for Evans blue is also increased, which indicates the opening of the blood-brain barrier. Higher concentrations of the Al-...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/BF00996974
更新日期:1990-03-01 00:00:00