In the last decades,the role of the prion protein(PrP) in neurodegenerative diseases has been intensively investigated,initially in prion diseases of humans(e.g., Creutzfeldt-J akob disease) and animals(e.g.,scrapie i...In the last decades,the role of the prion protein(PrP) in neurodegenerative diseases has been intensively investigated,initially in prion diseases of humans(e.g., Creutzfeldt-J akob disease) and animals(e.g.,scrapie in sheep,chronic wasting disease in deer and elk,or "mad cow disease" in cattle).Templated misfolding of physiological cellular prion protein(PrPC) into an aggregation-prone isoform(termed PrP "Scrapie"(PrPSc)),self-re plication and spreading of the latter inside the brain and to peripheral tissues,and the associated formation of infectious proteopathic seeds(termed "prions")are among the essential pathogenic mechanisms underlying this group of fatal and transmissible spongiform encephalopathies.Late r,key roles of the correctly folded PrPCwere identified in more common human brain diseases(such as Alzheimer s disease or Parkinson’s disease) associated with the misfolding and/or accumulation of other proteins(such as amyloid-β,tau or α-synuclein,respectively).PrPChas also been linked with n euro protective and regenerative functions,for instance in hypoxic/ischemic conditions such as stroke.However,despite a mixed "bouquet" of suggested functions,our understanding of pathological and,especially,physiological roles played by PrPCin the brain and beyond is ce rtainly incomplete.Interactions with various other proteins at the cell surfa ce or within intracellular compartments may account for the functional diversity linked with PrPC.Moreover,conserved endogenous proteolytic processing of PrPCgenerates seve ral defined PrPCfragments,possibly holding intrinsic functions in physiological and pathological conditions,thus making the "true and complete biology" of this protein more complicated to be elucidated.Here,we focus on one of those released PrPCfragments,namely shed PrP(sPrP),generated by a membrane-proximate ADAM10-mediated cleavage event at the cell surfa ce.Similar to other soluble PrP fragments(such as the N1 fragment representing PrP’s released N-terminal tail upon the major α-cleavage event)or expe rimentally employed recombinant PrP,sPrP is being suggested to act n euro protective in Alzheimer’s disease and other protein misfolding diseases.Seve ral lines of evidence on extracellular PrPC(fragments) suggest that induction of PrPCrelease co uld be a future therapeutic option in various brain disorders.Our recent identification of a substrate-specific approach to stimulate the shedding by ADAM 10,based on ligands binding to cell surface PrPC,may further set the stage for research into this direction.展开更多
BACKGROUND Wernicke encephalopathy is a neurological disorder caused by thiamine deficiency,commonly seen in alcoholic populations but also involving other circumstances that may lead to thiamine deficiency.The recogn...BACKGROUND Wernicke encephalopathy is a neurological disorder caused by thiamine deficiency,commonly seen in alcoholic populations but also involving other circumstances that may lead to thiamine deficiency.The recognition of Wernicke encephalopathy often depends on clinicians’keen ability to detect its typical triad of features;however,most cases do not present with the full constellation of signs,which complicates the timely identification of Wernicke encephalopathy.CASE SUMMARY This case report describes a patient with nasopharyngeal carcinoma who developed abnormal ocular function and ataxia following concurrent chemoradiotherapy,without a history of alcohol abuse.With the aid of radiological examinations,he received a timely diagnosis and treatment;however,his symptoms did not fully resolve during follow-up.CONCLUSION For patients with malignant tumors exhibiting neurological symptoms,clinicians should consider the possibility of Wernicke encephalopathy and provide prophylactic thiamine therapy.展开更多
AIM: To evaluate the association between serum concentrations of S100β in patients with cirrhosis and the presence of low grade hepatic encephalopathy (HE).METHODS: This was a cross-sectional study. The po...AIM: To evaluate the association between serum concentrations of S100β in patients with cirrhosis and the presence of low grade hepatic encephalopathy (HE).METHODS: This was a cross-sectional study. The population was categorized into four groups healthy subjects, cirrhosis without HE, cirrhosis with covert hepatic encephalopathy (CHE) and cirrhosis with overt HE. Kruskal-Wallis, Mann Whitney’s U with Bonferroni adjustment Spearman correlations and area under the ROC were used as appropriate.RESULTS: A total of 61 subjects were included, 46 cirrhotic patients and 15 healthy volunteers. S100β values were different among all groups, and differences remained significant between groups 1 and 2 (P < 0.001), and also between groups 2 and 3 (P = 0.016), but not between groups 3 and 4. In cirrhotic patients with HE S100β was higher than in patients without HE [0.18 (0.14-0.28) ng/mL vs 0.11 (0.06-0.14) ng/mL, P < 0.001]. There was a close correlation between serum concentrations of S100β and psychometric hepatic encephalopathy score in patients with cirrhosis without HE compared to the patients with cirrhosis with CHE (r = -0.413, P = 0.019). ROC curve analysis yielded > 0.13 ng/mL as the best cutoff value of S100β for the diagnosis of HE (sensitivity 83.3%, specificity 63.6%).CONCLUSION: Serum concentrations of S100β are higher in patients with cirrhosis than in healthy volunteers, and are further increased in the presence of hepatic encephalopathy. The results suggest that serum biomarkers such as S100β could help in the correct characterization of incipient stages of HE.展开更多
AIMTo present the typical and atypical magnetic resonance (MR) imaging findings of alcoholic and non-alcoholic Wernicke’s encephalopathy.METHODSThis study included 7 patients with Wernicke’s encephalopathy (2 men, 5...AIMTo present the typical and atypical magnetic resonance (MR) imaging findings of alcoholic and non-alcoholic Wernicke’s encephalopathy.METHODSThis study included 7 patients with Wernicke’s encephalopathy (2 men, 5 women; mean age, 52.3 years) that underwent brain MR examination between January 2012 and March 2016 in a single institution. Three patients were alcoholics and 4 patients were non-alcoholics. MR protocol included a T2-weighted sequence, a fluid attenuation inversion recovery (FLAIR) sequence, a diffusion-weighted sequence (b = 0 and 1000 s/mm<sup>2</sup>), and a contrast-enhanced MR sequence. All MR images were retrospectively reviewed at baseline and follow-up by two radiologists.RESULTSAll patients with Wernicke’s encephalopathy had bilateral areas showing high signal intensity on both T2-weighted and FLAIR MR images in the typical sites (i.e., the periaqueductal region and the tectal plate). Signal intensity abnormalities in the atypical sites (i.e., the cerebellum and the cerebellar vermis) were seen in 4 patients, all of which had no history of alcohol abuse. Six patients had areas with restricted diffusion in the typical and atypical sites. Four patients had areas showing contrast-enhancement in the typical and atypical sites. Follow-up MR imaging within 6 mo after therapy (intravenous administration of thiamine) was performed in 4 patients, and demonstrated a complete resolution of all the signal intensities abnormalities previously seen in all patients.CONCLUSIONMR imaging is valuable in the diagnosis of Wernicke’s encephalopathy particularly in patients presenting with atypical clinical symptoms, or with no history of alcohol abuse.展开更多
This paper presents the limiting expression for the gen calized inverse A T.S(2) and itscorresgonding projectors Since comonon imnortors inverses,such as and AT.S(2) etc are all generalized in e e AT.S(2) In fact,we g...This paper presents the limiting expression for the gen calized inverse A T.S(2) and itscorresgonding projectors Since comonon imnortors inverses,such as and AT.S(2) etc are all generalized in e e AT.S(2) In fact,we give a unified limiting formula of computine such imporiant generalined inverses and its corresponding proiectors,Based on this we estalish and imbedling method fire compoting the generalized in verse AT.S(2) The results extend earlier work by various authors.展开更多
Fibrillar proteins form structural elements of cells and the extracellular matrix. Pathological lesions, of fibrillar microanatomical structures, or secondary fibrillar changes in globular proteins are well known. A s...Fibrillar proteins form structural elements of cells and the extracellular matrix. Pathological lesions, of fibrillar microanatomical structures, or secondary fibrillar changes in globular proteins are well known. A special group concerns histologically amorphous deposits, amyloid. The major characteristics of amyloid are: apple green birefringence after Congo red staining of histological sections, and non-branching 7–10 nm thick fibrils on electron microscopy revealing a high content of cross beta pleated sheets. About 25 different types of amyloid have been characterised. In animals, AA-amyloid is the most frequent type. Other types of amyloid in animals represent: AIAPP (in cats), AApoAI, AApoAII, localised AL-amyloid, amyloid in odontogenic or mammary tumors and amyloid in the brain. In old dogs Aβ and in sheep APrPsc-amyloid can be encountered. AA-amyloidosis is a systemic disorder with a precursor in blood, acute phase serum amyloid A (SAA). In chronic inflammatory processes AA-amyloid can be deposited. A rapid crystallization of SAA to amyloid fibrils on small beta-sheeted fragments, the ‘amyloid enhancing factor’ (AEF), is known and the AEF has been shown to penetrate the enteric barrier. Amyloid fibrils can aggregate from various precursor proteins in vitro in particular at acidic pH and when proteolytic fragments are formed. Molecular chaperones influence this process. Tissue data point to amyloid fibrillogenesis in lysosomes and near cell surfaces. A comparison can be made of the fibrillogenesis in prion diseases and in enhanced AA-amyloidosis. In the reactive form, acute phase SAA is the supply of the precursor protein, whereas in the prion diseases, cell membrane proteins form a structural source. Aβ-amyloid in brain tissue of aged dogs showing signs of dementia forms a canine counterpart of senile dementia of the Alzheimer type (ccSDAT) in man. Misfolded proteins remain potential food hazards. Developments concerning prevention of amyloidogenesis and therapy of amyloid deposits are shortly commented.展开更多
INTRODUCTIONHepatic encephalopathy ( HE) is a frequent complication of chronic liver disease .It is defined as a characteristic functional and reversible alteration of the mental state ,due to impaired liver function ...INTRODUCTIONHepatic encephalopathy ( HE) is a frequent complication of chronic liver disease .It is defined as a characteristic functional and reversible alteration of the mental state ,due to impaired liver function and / or increased portosystemic shunting .展开更多
Ding's Manor is a Hakka's folk dwelling characterized by both Hakka's cultures on the Central Plain and also cultures of north Sichuan, and a typical Hakka's architecture in Sichuan Province. Design of...Ding's Manor is a Hakka's folk dwelling characterized by both Hakka's cultures on the Central Plain and also cultures of north Sichuan, and a typical Hakka's architecture in Sichuan Province. Design of the manor concentrates rich cultures, arts and beliefs of the Hakka, thus exploration of Ding's Manor, specifically, its site layout showing Fengshui doctrines of the Hakka, architectural style and watchtower with strict structures, and exquisite decorative arts, show architectural technology and wisdom of the Hakka, and also artistic characteristics of the Hakka's folk dwellings in a certain historical environment, which provides a favorable reference for the construction of modern folk dwellings.展开更多
Hashimoto’s encephalopathy(HE)is a rare form of reversible encephalopathy characterized by the presence of anti-thyroid antibodies in serum and/or cerebrospinal fl uid.The syndrome is more common in women and the pre...Hashimoto’s encephalopathy(HE)is a rare form of reversible encephalopathy characterized by the presence of anti-thyroid antibodies in serum and/or cerebrospinal fl uid.The syndrome is more common in women and the presentation varies considerably.Here,we report a case of an elderly male with a history of Hashimoto’s thyroiditis,presenting with acute cognitive decline.A diagnosis of HE was established based on the presence of antithyroid antibodies in the serum,diffuse electroencephalography changes and lack of an alternative explanation.The patient promptly responded to steroids and was discharged on the 8th day of admission.We suggest that an assessment of thyroid antibodies should be included in anyone presenting with acute cognitive decline in the absence of alternative explanation.展开更多
Posterior reversible encephalopathy syndrome(PRES)is a clinico-ragiological syndrome presenting with neurological symptoms and characteristic radiologic findings.PRES occurs in the setting of various clinical conditio...Posterior reversible encephalopathy syndrome(PRES)is a clinico-ragiological syndrome presenting with neurological symptoms and characteristic radiologic findings.PRES occurs in the setting of various clinical conditions and requires prompt management of the causative factor for a full recovery.This is a case report of a Crohn’s disease patient who developed PRES syndrome during a complicated post-operative course.In the presence of multiple causative factors,sepsis was considered as the predominant one.After prompt management,the patient recovered with no permanent neurological damage.展开更多
Rationale:Prolonged undernutrition may arise out of depression and lead to Wernicke's encephalopathy if timely diagnosis and intervention are missed.Wernicke's encephalopathy is potentially treatable,and appro...Rationale:Prolonged undernutrition may arise out of depression and lead to Wernicke's encephalopathy if timely diagnosis and intervention are missed.Wernicke's encephalopathy is potentially treatable,and appropriate treatment may revert clinical depression and cognitive dysfunction to some extent.Patient's concern:A 69-year-old female who had been taking escitalopram for one year developed tremor,ophthalmoplegia,ataxia,progressive cognitive decline,and convulsions.Diagnosis:Non-alcoholic Wernicke's encephalopathy and hypomagnesemia due to psychogenic anorexia.Interventions:High dose intravenous thiamine and magnesium were supplemented.Outcomes:The patient showed remarkable improvement in neurological complications and even in depressive features.Lessons:Wernicke's encephalopathy should not be ignored in the treatment of depression.展开更多
The pathogenesis of Hashimoto’s encephalopathy has not been clearly elucidated and involvement of autoimmune damage has been proposed. We report a 23-year-old man who was emergently hospitalized for paroxysmal syncop...The pathogenesis of Hashimoto’s encephalopathy has not been clearly elucidated and involvement of autoimmune damage has been proposed. We report a 23-year-old man who was emergently hospitalized for paroxysmal syncope and involuntary tremor with normal physical examination except for low heart rate. The patient was characterized by neurological symptoms, mild hypothyroidism, enlarged pituitary gland and extremely elevated thyrotropin, which all were reversed with levothyroxine alone. The case indicated that the pituitary-thyroid dysfunction may play a role in the pathogenesis of Hashimoto’s encephalopathy, hence, evaluation of pituitary gland should be recommended in diagnosis and treatment of Hashimoto’s encephalopathy.展开更多
Introduction: Hashimoto encephalopathy (HE) is an autoimmune disease with different neurological and psychiatric characteristics. It is associated with increased thyroperoxidase autoantibodies. The diagnosis of HE is ...Introduction: Hashimoto encephalopathy (HE) is an autoimmune disease with different neurological and psychiatric characteristics. It is associated with increased thyroperoxidase autoantibodies. The diagnosis of HE is clinical and involves the detection of serum thyroperoxidase (TPO) antibodies and the exclusion of other etiologies. A clinical response to corticosteroid therapy supports the diagnosis. We report a case of HE revealed by convulsive seizures and cognitive and behavioral disorders. Observation: She is a 7-year-old child followed in a neurology unit for convulsive seizures for one year. Clinical examination performed in the endocrinology unit later found tachycardia without goiter, behavioral disorders, difficulties to concentrate as well as psychomotor agitation. Biological work up revealed subclinical hypothyroidism, positive anti-TPO antibodies with normal thyroid ultrasound and brain MRI. The cerebrospinal fluid was clear, normotensive and absence of pleocytosis. The sleep-wake electroencephalogram was well organized for her age with a paroxysmal discharge in the left parieto-temporal region. Based on these clinical, biological and morphological data, the diagnosis of HE was retained. Corticosteroid therapy with prednisone at 1 mg/kg/day was initiated for 6 weeks while reducing the dose progressively. There was a good clinical and biological progress under treatment. Conclusion: Hashimoto’s encephalopathy is a rare, but probably unrecognized entity. Over the past decades, knowledge about thyroid, brain, immunity interactions has increased. This indicates the importance of a multidisciplinary approach of endocrinologists, neurologists, psychiatrists and pediatricians for early recognition and treatment.展开更多
目的探讨缺氧缺血性脑病(HIE)新生儿血清生物标志物神经元特异性烯醇酶(NSE)、S-100B、Tau蛋白、激活素A等指标变化及诊断效能。方法选取2020年1月至2022年2月在温州医科大学附属台州医院出生或就诊的75例HIE足月新生儿为研究对象,按照...目的探讨缺氧缺血性脑病(HIE)新生儿血清生物标志物神经元特异性烯醇酶(NSE)、S-100B、Tau蛋白、激活素A等指标变化及诊断效能。方法选取2020年1月至2022年2月在温州医科大学附属台州医院出生或就诊的75例HIE足月新生儿为研究对象,按照HIE严重程度分为轻度HIE组30例,中度HIE组25例,重度HIE组20例。选取同期本院出生的75名健康足月新生儿为对照组。检测并比较4组研究对象血清生物标志物及神经发育指标[包括新生儿神经行为(NBNA)、发育商(DQ)],采用Pearson相关分析血清生物标志物与神经发育指标的相关性,ROC曲线分析各项血清生物标志物单独或联合检测对HIE的诊断效能。结果4组研究对象血清生物标志物及神经发育指标比较,差异均有统计学意义(均P<0.05),其中轻、中、重度HIE组患儿血清NSE、S-100B、Tau蛋白、激活素A水平均明显高于对照组(均P<0.05),且轻度HIE组<中度HIE组<重度HIE组(均P<0.05);生后7 d NBNA评分、生后35周DQ、生后52周DQ均明显低于对照组(均P<0.05),且轻度HIE组>中度HIE组>重度HIE组(均P<0.05)。血清NSE、S-100B、Tau蛋白、激活素A水平与生后7 d NBNA评分均呈负相关(r=-0.779、-0.788、-0.714、-0.800,均P<0.05);与生后35周DQ均呈负相关(r=-0.662、-0.700、-0.626、-0.674,均P<0.05),与生后52周DQ均呈负相关(r=-0.636、-0.684、-0.619、-0.632,均P<0.05)。血清NSE、S-100B、Tau蛋白、激活素A单独检测和4项联合检测诊断HIE的AUC分别为0.923、0.946、0.942、0.939和0.995,4项联合检测的诊断效能最佳。结论血清NSE、S-100B、Tau蛋白、激活素A与HIE新生儿神经发育有关,4项联合检测诊断HIE的效能较高。展开更多
We discuss the safety and controversy regarding the administration of glucose before thiamine with regards to precipitation of Wernicke’s Encephalopathy in the severely malnourished and alcoholic patient population. ...We discuss the safety and controversy regarding the administration of glucose before thiamine with regards to precipitation of Wernicke’s Encephalopathy in the severely malnourished and alcoholic patient population. Herein we review clinical features, pathophysiology and the relevant literature to provide an evidenced-based recommendation that thiamine replacement should not delay glucose administration acutely in the malnourished patient population.展开更多
He designed the national emblem of thePeople’s depublic of China, was a primary painterof the mural in the Beijing Capital Airport, ananimator of "Ne Zha Makes Havoc of the Sea", arenowned cartoonist, one o...He designed the national emblem of thePeople’s depublic of China, was a primary painterof the mural in the Beijing Capital Airport, ananimator of "Ne Zha Makes Havoc of the Sea", arenowned cartoonist, one of the founders of展开更多
基金supported by funding from the Creutzfeldt-Jakob Disease FoundationInc.(USA)+4 种基金the Alzheimer Forschung Initiative (AFI e.V.,Germany)the Werner-Otto-Stiftung (Hamburg,Germany)(all to HCA)the China Scholarship Council (to FS)European Union’s Horizon 2020 Research and Innovation Program under the Marie Sklodowska-Curie Grant Agreement N°101030402 (to AMA)Deutsche Forschungsgemeinschaft (DFG) Collaborative Research Center (CRC) 877"Proteolysis as a regulatory event in pathophysiology"(to MG)。
文摘In the last decades,the role of the prion protein(PrP) in neurodegenerative diseases has been intensively investigated,initially in prion diseases of humans(e.g., Creutzfeldt-J akob disease) and animals(e.g.,scrapie in sheep,chronic wasting disease in deer and elk,or "mad cow disease" in cattle).Templated misfolding of physiological cellular prion protein(PrPC) into an aggregation-prone isoform(termed PrP "Scrapie"(PrPSc)),self-re plication and spreading of the latter inside the brain and to peripheral tissues,and the associated formation of infectious proteopathic seeds(termed "prions")are among the essential pathogenic mechanisms underlying this group of fatal and transmissible spongiform encephalopathies.Late r,key roles of the correctly folded PrPCwere identified in more common human brain diseases(such as Alzheimer s disease or Parkinson’s disease) associated with the misfolding and/or accumulation of other proteins(such as amyloid-β,tau or α-synuclein,respectively).PrPChas also been linked with n euro protective and regenerative functions,for instance in hypoxic/ischemic conditions such as stroke.However,despite a mixed "bouquet" of suggested functions,our understanding of pathological and,especially,physiological roles played by PrPCin the brain and beyond is ce rtainly incomplete.Interactions with various other proteins at the cell surfa ce or within intracellular compartments may account for the functional diversity linked with PrPC.Moreover,conserved endogenous proteolytic processing of PrPCgenerates seve ral defined PrPCfragments,possibly holding intrinsic functions in physiological and pathological conditions,thus making the "true and complete biology" of this protein more complicated to be elucidated.Here,we focus on one of those released PrPCfragments,namely shed PrP(sPrP),generated by a membrane-proximate ADAM10-mediated cleavage event at the cell surfa ce.Similar to other soluble PrP fragments(such as the N1 fragment representing PrP’s released N-terminal tail upon the major α-cleavage event)or expe rimentally employed recombinant PrP,sPrP is being suggested to act n euro protective in Alzheimer’s disease and other protein misfolding diseases.Seve ral lines of evidence on extracellular PrPC(fragments) suggest that induction of PrPCrelease co uld be a future therapeutic option in various brain disorders.Our recent identification of a substrate-specific approach to stimulate the shedding by ADAM 10,based on ligands binding to cell surface PrPC,may further set the stage for research into this direction.
文摘BACKGROUND Wernicke encephalopathy is a neurological disorder caused by thiamine deficiency,commonly seen in alcoholic populations but also involving other circumstances that may lead to thiamine deficiency.The recognition of Wernicke encephalopathy often depends on clinicians’keen ability to detect its typical triad of features;however,most cases do not present with the full constellation of signs,which complicates the timely identification of Wernicke encephalopathy.CASE SUMMARY This case report describes a patient with nasopharyngeal carcinoma who developed abnormal ocular function and ataxia following concurrent chemoradiotherapy,without a history of alcohol abuse.With the aid of radiological examinations,he received a timely diagnosis and treatment;however,his symptoms did not fully resolve during follow-up.CONCLUSION For patients with malignant tumors exhibiting neurological symptoms,clinicians should consider the possibility of Wernicke encephalopathy and provide prophylactic thiamine therapy.
基金Supported by CONACYT/UNAM and FUNDACIóN PARA LA SALUD Y LA EDUCACIóN DR. SALVADOR ZUBIRáN A.C (To Macias-Rodriguez RU)CONACYT/UNAM (To Ruiz-Margáin A) Ramón y Cajal Researcher, No. RYC-2014-15242 (To Cubero FJ)
文摘AIM: To evaluate the association between serum concentrations of S100β in patients with cirrhosis and the presence of low grade hepatic encephalopathy (HE).METHODS: This was a cross-sectional study. The population was categorized into four groups healthy subjects, cirrhosis without HE, cirrhosis with covert hepatic encephalopathy (CHE) and cirrhosis with overt HE. Kruskal-Wallis, Mann Whitney’s U with Bonferroni adjustment Spearman correlations and area under the ROC were used as appropriate.RESULTS: A total of 61 subjects were included, 46 cirrhotic patients and 15 healthy volunteers. S100β values were different among all groups, and differences remained significant between groups 1 and 2 (P < 0.001), and also between groups 2 and 3 (P = 0.016), but not between groups 3 and 4. In cirrhotic patients with HE S100β was higher than in patients without HE [0.18 (0.14-0.28) ng/mL vs 0.11 (0.06-0.14) ng/mL, P < 0.001]. There was a close correlation between serum concentrations of S100β and psychometric hepatic encephalopathy score in patients with cirrhosis without HE compared to the patients with cirrhosis with CHE (r = -0.413, P = 0.019). ROC curve analysis yielded > 0.13 ng/mL as the best cutoff value of S100β for the diagnosis of HE (sensitivity 83.3%, specificity 63.6%).CONCLUSION: Serum concentrations of S100β are higher in patients with cirrhosis than in healthy volunteers, and are further increased in the presence of hepatic encephalopathy. The results suggest that serum biomarkers such as S100β could help in the correct characterization of incipient stages of HE.
文摘AIMTo present the typical and atypical magnetic resonance (MR) imaging findings of alcoholic and non-alcoholic Wernicke’s encephalopathy.METHODSThis study included 7 patients with Wernicke’s encephalopathy (2 men, 5 women; mean age, 52.3 years) that underwent brain MR examination between January 2012 and March 2016 in a single institution. Three patients were alcoholics and 4 patients were non-alcoholics. MR protocol included a T2-weighted sequence, a fluid attenuation inversion recovery (FLAIR) sequence, a diffusion-weighted sequence (b = 0 and 1000 s/mm<sup>2</sup>), and a contrast-enhanced MR sequence. All MR images were retrospectively reviewed at baseline and follow-up by two radiologists.RESULTSAll patients with Wernicke’s encephalopathy had bilateral areas showing high signal intensity on both T2-weighted and FLAIR MR images in the typical sites (i.e., the periaqueductal region and the tectal plate). Signal intensity abnormalities in the atypical sites (i.e., the cerebellum and the cerebellar vermis) were seen in 4 patients, all of which had no history of alcohol abuse. Six patients had areas with restricted diffusion in the typical and atypical sites. Four patients had areas showing contrast-enhancement in the typical and atypical sites. Follow-up MR imaging within 6 mo after therapy (intravenous administration of thiamine) was performed in 4 patients, and demonstrated a complete resolution of all the signal intensities abnormalities previously seen in all patients.CONCLUSIONMR imaging is valuable in the diagnosis of Wernicke’s encephalopathy particularly in patients presenting with atypical clinical symptoms, or with no history of alcohol abuse.
基金This project is supported by the National Natural Science Foundation of China.
文摘This paper presents the limiting expression for the gen calized inverse A T.S(2) and itscorresgonding projectors Since comonon imnortors inverses,such as and AT.S(2) etc are all generalized in e e AT.S(2) In fact,we give a unified limiting formula of computine such imporiant generalined inverses and its corresponding proiectors,Based on this we estalish and imbedling method fire compoting the generalized in verse AT.S(2) The results extend earlier work by various authors.
文摘Fibrillar proteins form structural elements of cells and the extracellular matrix. Pathological lesions, of fibrillar microanatomical structures, or secondary fibrillar changes in globular proteins are well known. A special group concerns histologically amorphous deposits, amyloid. The major characteristics of amyloid are: apple green birefringence after Congo red staining of histological sections, and non-branching 7–10 nm thick fibrils on electron microscopy revealing a high content of cross beta pleated sheets. About 25 different types of amyloid have been characterised. In animals, AA-amyloid is the most frequent type. Other types of amyloid in animals represent: AIAPP (in cats), AApoAI, AApoAII, localised AL-amyloid, amyloid in odontogenic or mammary tumors and amyloid in the brain. In old dogs Aβ and in sheep APrPsc-amyloid can be encountered. AA-amyloidosis is a systemic disorder with a precursor in blood, acute phase serum amyloid A (SAA). In chronic inflammatory processes AA-amyloid can be deposited. A rapid crystallization of SAA to amyloid fibrils on small beta-sheeted fragments, the ‘amyloid enhancing factor’ (AEF), is known and the AEF has been shown to penetrate the enteric barrier. Amyloid fibrils can aggregate from various precursor proteins in vitro in particular at acidic pH and when proteolytic fragments are formed. Molecular chaperones influence this process. Tissue data point to amyloid fibrillogenesis in lysosomes and near cell surfaces. A comparison can be made of the fibrillogenesis in prion diseases and in enhanced AA-amyloidosis. In the reactive form, acute phase SAA is the supply of the precursor protein, whereas in the prion diseases, cell membrane proteins form a structural source. Aβ-amyloid in brain tissue of aged dogs showing signs of dementia forms a canine counterpart of senile dementia of the Alzheimer type (ccSDAT) in man. Misfolded proteins remain potential food hazards. Developments concerning prevention of amyloidogenesis and therapy of amyloid deposits are shortly commented.
基金This work was supported by Deutsche Forschungsgemeinschaft (Sonderforschungsbereich 503"Molekulare und zellulare Mechanismen exogener Noxen"and SFB 575"Experimentelle Hepatologie")
文摘INTRODUCTIONHepatic encephalopathy ( HE) is a frequent complication of chronic liver disease .It is defined as a characteristic functional and reversible alteration of the mental state ,due to impaired liver function and / or increased portosystemic shunting .
文摘Ding's Manor is a Hakka's folk dwelling characterized by both Hakka's cultures on the Central Plain and also cultures of north Sichuan, and a typical Hakka's architecture in Sichuan Province. Design of the manor concentrates rich cultures, arts and beliefs of the Hakka, thus exploration of Ding's Manor, specifically, its site layout showing Fengshui doctrines of the Hakka, architectural style and watchtower with strict structures, and exquisite decorative arts, show architectural technology and wisdom of the Hakka, and also artistic characteristics of the Hakka's folk dwellings in a certain historical environment, which provides a favorable reference for the construction of modern folk dwellings.
文摘Hashimoto’s encephalopathy(HE)is a rare form of reversible encephalopathy characterized by the presence of anti-thyroid antibodies in serum and/or cerebrospinal fl uid.The syndrome is more common in women and the presentation varies considerably.Here,we report a case of an elderly male with a history of Hashimoto’s thyroiditis,presenting with acute cognitive decline.A diagnosis of HE was established based on the presence of antithyroid antibodies in the serum,diffuse electroencephalography changes and lack of an alternative explanation.The patient promptly responded to steroids and was discharged on the 8th day of admission.We suggest that an assessment of thyroid antibodies should be included in anyone presenting with acute cognitive decline in the absence of alternative explanation.
文摘Posterior reversible encephalopathy syndrome(PRES)is a clinico-ragiological syndrome presenting with neurological symptoms and characteristic radiologic findings.PRES occurs in the setting of various clinical conditions and requires prompt management of the causative factor for a full recovery.This is a case report of a Crohn’s disease patient who developed PRES syndrome during a complicated post-operative course.In the presence of multiple causative factors,sepsis was considered as the predominant one.After prompt management,the patient recovered with no permanent neurological damage.
文摘Rationale:Prolonged undernutrition may arise out of depression and lead to Wernicke's encephalopathy if timely diagnosis and intervention are missed.Wernicke's encephalopathy is potentially treatable,and appropriate treatment may revert clinical depression and cognitive dysfunction to some extent.Patient's concern:A 69-year-old female who had been taking escitalopram for one year developed tremor,ophthalmoplegia,ataxia,progressive cognitive decline,and convulsions.Diagnosis:Non-alcoholic Wernicke's encephalopathy and hypomagnesemia due to psychogenic anorexia.Interventions:High dose intravenous thiamine and magnesium were supplemented.Outcomes:The patient showed remarkable improvement in neurological complications and even in depressive features.Lessons:Wernicke's encephalopathy should not be ignored in the treatment of depression.
文摘The pathogenesis of Hashimoto’s encephalopathy has not been clearly elucidated and involvement of autoimmune damage has been proposed. We report a 23-year-old man who was emergently hospitalized for paroxysmal syncope and involuntary tremor with normal physical examination except for low heart rate. The patient was characterized by neurological symptoms, mild hypothyroidism, enlarged pituitary gland and extremely elevated thyrotropin, which all were reversed with levothyroxine alone. The case indicated that the pituitary-thyroid dysfunction may play a role in the pathogenesis of Hashimoto’s encephalopathy, hence, evaluation of pituitary gland should be recommended in diagnosis and treatment of Hashimoto’s encephalopathy.
文摘Introduction: Hashimoto encephalopathy (HE) is an autoimmune disease with different neurological and psychiatric characteristics. It is associated with increased thyroperoxidase autoantibodies. The diagnosis of HE is clinical and involves the detection of serum thyroperoxidase (TPO) antibodies and the exclusion of other etiologies. A clinical response to corticosteroid therapy supports the diagnosis. We report a case of HE revealed by convulsive seizures and cognitive and behavioral disorders. Observation: She is a 7-year-old child followed in a neurology unit for convulsive seizures for one year. Clinical examination performed in the endocrinology unit later found tachycardia without goiter, behavioral disorders, difficulties to concentrate as well as psychomotor agitation. Biological work up revealed subclinical hypothyroidism, positive anti-TPO antibodies with normal thyroid ultrasound and brain MRI. The cerebrospinal fluid was clear, normotensive and absence of pleocytosis. The sleep-wake electroencephalogram was well organized for her age with a paroxysmal discharge in the left parieto-temporal region. Based on these clinical, biological and morphological data, the diagnosis of HE was retained. Corticosteroid therapy with prednisone at 1 mg/kg/day was initiated for 6 weeks while reducing the dose progressively. There was a good clinical and biological progress under treatment. Conclusion: Hashimoto’s encephalopathy is a rare, but probably unrecognized entity. Over the past decades, knowledge about thyroid, brain, immunity interactions has increased. This indicates the importance of a multidisciplinary approach of endocrinologists, neurologists, psychiatrists and pediatricians for early recognition and treatment.
文摘目的探讨缺氧缺血性脑病(HIE)新生儿血清生物标志物神经元特异性烯醇酶(NSE)、S-100B、Tau蛋白、激活素A等指标变化及诊断效能。方法选取2020年1月至2022年2月在温州医科大学附属台州医院出生或就诊的75例HIE足月新生儿为研究对象,按照HIE严重程度分为轻度HIE组30例,中度HIE组25例,重度HIE组20例。选取同期本院出生的75名健康足月新生儿为对照组。检测并比较4组研究对象血清生物标志物及神经发育指标[包括新生儿神经行为(NBNA)、发育商(DQ)],采用Pearson相关分析血清生物标志物与神经发育指标的相关性,ROC曲线分析各项血清生物标志物单独或联合检测对HIE的诊断效能。结果4组研究对象血清生物标志物及神经发育指标比较,差异均有统计学意义(均P<0.05),其中轻、中、重度HIE组患儿血清NSE、S-100B、Tau蛋白、激活素A水平均明显高于对照组(均P<0.05),且轻度HIE组<中度HIE组<重度HIE组(均P<0.05);生后7 d NBNA评分、生后35周DQ、生后52周DQ均明显低于对照组(均P<0.05),且轻度HIE组>中度HIE组>重度HIE组(均P<0.05)。血清NSE、S-100B、Tau蛋白、激活素A水平与生后7 d NBNA评分均呈负相关(r=-0.779、-0.788、-0.714、-0.800,均P<0.05);与生后35周DQ均呈负相关(r=-0.662、-0.700、-0.626、-0.674,均P<0.05),与生后52周DQ均呈负相关(r=-0.636、-0.684、-0.619、-0.632,均P<0.05)。血清NSE、S-100B、Tau蛋白、激活素A单独检测和4项联合检测诊断HIE的AUC分别为0.923、0.946、0.942、0.939和0.995,4项联合检测的诊断效能最佳。结论血清NSE、S-100B、Tau蛋白、激活素A与HIE新生儿神经发育有关,4项联合检测诊断HIE的效能较高。
文摘We discuss the safety and controversy regarding the administration of glucose before thiamine with regards to precipitation of Wernicke’s Encephalopathy in the severely malnourished and alcoholic patient population. Herein we review clinical features, pathophysiology and the relevant literature to provide an evidenced-based recommendation that thiamine replacement should not delay glucose administration acutely in the malnourished patient population.
文摘He designed the national emblem of thePeople’s depublic of China, was a primary painterof the mural in the Beijing Capital Airport, ananimator of "Ne Zha Makes Havoc of the Sea", arenowned cartoonist, one of the founders of