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1.
Chinese Journal of Geriatrics ; (12): 1589-1591, 2021.
Article in Chinese | WPRIM | ID: wpr-933018

ABSTRACT

Diffuse neurofibrillary tangles with calcification(DNTC)is a rare neurodegenerative disease and belongs to the category of tauopathies.The main characteristics of DNTC include presenile dementia, symmetrical bilateral calcification in the basal ganglia and cerebral dentate nuclei, and bilateral atrophy of the temporal lobes.At present, the majority of reported cases with DNTC were from Japan, with only a few cases from other countries.Although DNTC is a pathological diagnosis based on autopsy, ante-mortem diagnosis for DNTC can be achieved based on clinical features and presentation of brain tau protein shown on positron emission computed tomography.The clinical characteristics, pathological changes and diagnosis criteria of DNTC are reviewed in this paper.

2.
Chinese Journal of Neurology ; (12): 528-531, 2021.
Article in Chinese | WPRIM | ID: wpr-885457

ABSTRACT

Primary age-related tauopathy (PART) is one of neurodegenerative diseases in the very old people. PART clinically resembles Alzheimer′s disease and its definite diagnose needs pathological identification of neurofibrillary tangles with or without amyloid plaques in the brain. This article reviews the historical evolution, clinical manifestation, pathology and diagnosis of PART in order to provide new insights.

3.
Chinese Journal of Nuclear Medicine and Molecular Imaging ; (6): 321-326, 2021.
Article in Chinese | WPRIM | ID: wpr-884806

ABSTRACT

Objective:To investigate the neuroimaging relationship between tau protein deposition and brain atrophy, and assess their relationships with cognitive decline in Alzheimer′s disease (AD) patients.Methods:From April 2017 to October 2019, 26 AD patients (12 males, 14 females, age (70.7±12.2) years) and 19 cognitively normal controls (CN; 9 males, 10 females, age (65.6±8.1) years) in Chinese PLA General Hospital were retrospectively enrolled. All subjects received (S)-6-[(3- 18F-fluoro-2-hydroxy)propoxy]-2-(4-methylaminophenyl)quinoline ( 18F-THK5317) PET/MR and the standardized uptake value ratio (SUVR) and gray matter volume (GMV) were measured. General linear model (GLM) was used to evaluate the differences of SUVR and GMV between two groups. Pearson correlation analysis was used to assess the relationships between SUVR and GMV, and relationships of SUVR and GMV with Mini-Mental State Examination (MMSE) scores in AD patients. Results:Compared with CN, the AD patients showed significantly increased 18F-THK5317 retention in lateral temporal, frontal, posterior cingulated/precuneus and occipital cortex with significant differences of SUVR between two groups (2.18±0.54 vs 1.78±0.09, 2.13±0.50 vs 1.82±0.06, 2.03±0.45 vs 1.69±0.08, 2.18±0.57 vs 1.76±0.10, t values: 2.58-6.57, all P<0.001). The AD patients also showed decreased GMV in medial temporal, posterior cingulated/precuneus and orbitofrontal cortex ( t values: 3.67-8.85, all P<0.001). In AD patients, SUVR was negatively associated with GMV in bilateral lateral temporal cortex, pre-frontal cortex and orbital frontal cortex ( r values: from -0.52 to -0.43, all P<0.05). Both SUVR ( r=-0.599, P=0.001) and GMV ( r=0.443, P=0.023) were significantly correlated with MMSE scores in AD patients. Conclusion:AD patients have neocortical 18F-THK5317 abnormal uptake and GMV reduction, which are significantly correlated with cognitive decline.

4.
Chinese Journal of Internal Medicine ; (12): 662-667, 2019.
Article in Chinese | WPRIM | ID: wpr-797394

ABSTRACT

Objective@#To investigate the correlation of functional connectivity (FC) and the integrity of connective fibres between hippocampus and thalamus in Alzheimer′s disease(AD) and amnestic mild cognitive impairment (aMCI).@*Methods@#Both resting-state functional magnetic resonance imaging (rs-fMRI) and diffusion tensor imaging (DTI) data of 40 AD patients, 37 aMCI patients and 41 normal control subjects matching with age and educational level were collected. These subjects were all recruited from outpatient Department of Neurology in the Second Medical Center of Chinese PLA General Hospital, as well as poster, from May 2016 to January 2018. The FC strength between bilateral hippocampus and thalamus, as well as the parameters representing integrity of connective fibres, including fractional anisotropy (FA) and mean diffusivity(MD),were analyzed. Also, the correlations between FC strength and FA or MD strength were analyzed in the study.@*Results@#Compared to that of normal control subjects, the FC strength between billateral hippocampus and thalamus in patients with AD, aMCI were not significantly different(P>0.05). The integrity of bilateral connective fibres between hippocampus and thalamus were damaged in AD patients when compared to normal control subjects(P<0.01). A positive correlation of connective fibres integrity with FC strength between hippocampus and thalamus was found in the left side(r=0.25,P<0.05) but rather in the right side.@*Conclusion@#In AD and aMCI patients, structural connectivity between left hippocampus and thalamus affects the functional connectivity between them.

5.
Chinese Journal of Internal Medicine ; (12): 91-101, 2019.
Article in Chinese | WPRIM | ID: wpr-734703

ABSTRACT

Criteria for diagnosis of Alzheimer's disease (AD) is not available in China.The international criteria is not a proper choice due to issues such as translation and lead to low diagnostic rate and high rate of missed diagnosis.The research group of Alzheimer's Disease Chinese (ADC) reviewed knowledge and techniques in neuropsychology,neuroimaging,molecular biology,and clinical neurology,and systematically studied the detection techniques such as memory,language,visuospatial,executive function,and medial temporal lobe visual scores on MRI,and their optimal threshold and diagnostic value for the diagnosis of AD.Through a systematic review and consensus meeting,a diagnostic framework for screeningAD in the Chinese population was established.Among these methods,an operational standard for clinical pathology models increased the diagnostic sensitivity by 15%.The sensitivity and specificity of screening memory impairment increased by 18.1% and 11.6%,respectively.The sensitivity of screening medialtemporal lobe atrophy increased by 24.5% and missed diagnosis was decreased by 34.5%.An operational standard for clinical biology models,incorporating the latest molecular imaging and molecular biology techniques,has enabled the early diagnosis of AD in China.The framework combines a principled diagnostic guideline with an operational screening protocol,which is applicable to all clinical settings and of great significance for the early detection,early diagnosis and early treatment of AD.

6.
Chinese Journal of Internal Medicine ; (12): 10-16, 2019.
Article in Chinese | WPRIM | ID: wpr-734691

ABSTRACT

For lack of cognitive screening standard system and controversy over the value of imaging for cerebrovascular diseases in China, the research group of Alzheimer′s Disease Chinese (ADC) studied the knowledge of neuropsychology, neuroimaging and clinical neurology, systematically reviewed the diagnostic techniques such as memory, language, visuospatial, executive, function, and magnetic resonance imaging (MRI) of cerebrovascular diseases, and their optimal threshold and diagnostic value for vascular dementia. Via a consensus meeting, the diagnostic guidelines and practical screening process are combined to construct a framework in Chinese population, which is based on the objective evidence of medical history and clinical evaluation. The diagnosis of vascular dementia is supported by imaging evidence of cerebrovascular diseases and differentiates from other causes of dementia or comorbidities. This consensus is applicable to medical units in China, and is of great significance for early detection, early diagnosis and early treatment of vascular dementia.

7.
Chinese Journal of Geriatric Heart Brain and Vessel Diseases ; (12): 499-501, 2019.
Article in Chinese | WPRIM | ID: wpr-745486

ABSTRACT

Objective To study the pathological features of argyrophilic grains in normal aging brain, AD, PD and progressive superior nuclear paralysis patients. Methods Brain tissue samples taken from 5 AD, 3 PD, 2 progressive superior nuclear paralysis patients with complete clinico-pathological data and 4 normal aging brain subjects were stained with HE, Luxol fast blue and Gallyas-Braak silver respectively. Aβ, tau, α-synuclein and P62 antibodies were detected by microscopy with immunohistochemical staining. The pathological features of argyrophilic grains were recorded. Results The Gallyas-Braak silver staining showed argyrophilic grain structure in 4 out of the 14 amygdaloid nucleus tissue samples (2 from AD patients, 1 from progressive superior nuclear paralysis patients and 1 from normal aging brain patients) with a positive rate of 28.6%. The immunohistochemical staining showed positive tau and P62 antibodies. Conclusion Argyrophilic grain lesion is not uncommon in aging-related neurodegenerative diseases such as normal aging brain, AD and progressive superior nuclear paralysis and can thus produce its superposition effect on the clinical symptoms of cognitive impairment in AD and progressive superior nuclear paralysis patients.

8.
Chinese Journal of Internal Medicine ; (12): 894-900, 2018.
Article in Chinese | WPRIM | ID: wpr-710109

ABSTRACT

There are no standard diagnostic criteria for Alzheimer′s disease (AD) in China. The copied international criteria has led to a high rate of missed diagnosis due to issues such as translation and cultural discrepancy. Under the principles of semantic equivalence, content equivalence and performance equivalence, the research group of Alzheimer′s Disease Chinese (ADC) adopted several effective methods, such as two-way translation, content conversion, performance evaluation, etc. to systematically study the cognitive, behavioral, functional, and general assessment techniques in dementia screening and diagnosis, as well as their screening thresholds and diagnostic values. We also established a dementia screening and assessment framework in clinical practice through systematic reviews and group consensus. It has improved the early diagnosis rate of dementia in China, been accepted by home and abroad academic institutions, which is of great significance for early diagnosis and treatment of dementia.

9.
Chinese Journal of Experimental and Clinical Virology ; (6): 189-194, 2017.
Article in Chinese | WPRIM | ID: wpr-808300

ABSTRACT

Objective@#To search for biomarkers for human familial prion disease.@*Methods@#Two-dimensional differential gel electrophoresis (2D-DIGE) proteomic analysis has been performed in frontal lobe tissues of 3 patients suffering from human familial prion disease (PrP) and 3 age-and sex-matched patients suffering from sudden death due to heart failure without neurological disease.@*Results@#The maps revealed 14 polypeptide chains differentially modulated in the PrP samples, among those, 7 could be identified upon digestion and MALDI-TOF/MS analysis, of which 6 appeared to be up-regulated, 1 being down-regulated.@*Conclusions@#We highlight Galectin-1(Gal-1), ryanodine receptor 2 (RyR2), ubiquitin, Rab-interacting lysosomes protein-like protein 1 (RILPL-1) profillin 2 (PFN2), in the differential map. These proteins are related to neurogenesis, the clearance of misfolded proteins, stasis of calium channel, myoclonus and so on. These proteins are potential biomarkers or targets for treatment of prion disease.

10.
Chinese Journal of Epidemiology ; (12): 1292-1298, 2017.
Article in Chinese | WPRIM | ID: wpr-737821

ABSTRACT

Objective To investigate the health status,functional ability,mental psychology,health care and other longevity-related characteristics of individuals aged ≥ 100 years as well as risk factors in Hainan province,China.Methods China Hainan Centenarian Cohort Study (CHCCS) is a community-based,prospective cohort study to establish multi-dimensional database consisting of questionnaire findings,anthropometric parameters and biological specimens as well as imaging features.With the household registration information provided by the Department of Civil Affairs of Hainan province,a baseline survey was conducted in centenarians in 18 counties in Hainan with the oldest old in 5 counties as controls between 2014 and 2017.The survey included face to face interview,physical examination and biological specimen collection.After the baseline survey,the participants of CHCCS were followed up at an interval of 2 years to collect the information about their living status,disease status or major death causes.Results According to the information provided by the Department of Civil Affairs of Hainan province in 2014,the survey found that 1 473 centenarians were still living.By December 2016,1 002 of them had agreed to be surveyed.The average age of 722 centenarians with complete information in the baseline survey was (102.7± 2.7) years,the majority of them were females (83.0%),widows (88.8%),in Han ethnic group (84.5%),lived with family members (87.8%),illiterates (89.7%) and farmers (81.0%).Conclusion CHCCS has provided longevity-related information of the large longevity population and collected the valuable and rare biological specimens with great urgency to establish an interdisciplinary platform and base for longevity,senility and healthy aging research.

11.
Chinese Journal of Epidemiology ; (12): 1292-1298, 2017.
Article in Chinese | WPRIM | ID: wpr-736353

ABSTRACT

Objective To investigate the health status,functional ability,mental psychology,health care and other longevity-related characteristics of individuals aged ≥ 100 years as well as risk factors in Hainan province,China.Methods China Hainan Centenarian Cohort Study (CHCCS) is a community-based,prospective cohort study to establish multi-dimensional database consisting of questionnaire findings,anthropometric parameters and biological specimens as well as imaging features.With the household registration information provided by the Department of Civil Affairs of Hainan province,a baseline survey was conducted in centenarians in 18 counties in Hainan with the oldest old in 5 counties as controls between 2014 and 2017.The survey included face to face interview,physical examination and biological specimen collection.After the baseline survey,the participants of CHCCS were followed up at an interval of 2 years to collect the information about their living status,disease status or major death causes.Results According to the information provided by the Department of Civil Affairs of Hainan province in 2014,the survey found that 1 473 centenarians were still living.By December 2016,1 002 of them had agreed to be surveyed.The average age of 722 centenarians with complete information in the baseline survey was (102.7± 2.7) years,the majority of them were females (83.0%),widows (88.8%),in Han ethnic group (84.5%),lived with family members (87.8%),illiterates (89.7%) and farmers (81.0%).Conclusion CHCCS has provided longevity-related information of the large longevity population and collected the valuable and rare biological specimens with great urgency to establish an interdisciplinary platform and base for longevity,senility and healthy aging research.

12.
Chinese Journal of Internal Medicine ; (12): 24-28, 2017.
Article in Chinese | WPRIM | ID: wpr-509036

ABSTRACT

Objective To explore the risk factors for lower extremity amputation in patients with diabetic foot.Methods The clinical data of 1 771 patients with diabetic foot at the Air Force General Hospital of PLA from November 2001 to April 2015 were retrospectively analyzed.The patients were divided into the non-amputation and amputation groups.Within the amputation group , subjects were further divided into the minor and major amputation subgroups.Binary logistic regression analyses were used to assess the association between risk factors and lower extremity amputation.Results Among 1 771 patients with diabetic foot , 323 of them ( 18.24%) were in the amputation group ( major amputation: 41; minor amputation:282 ) and 1 448 ( 81.76%) in the non-amputation group.Compared with non-amputation patients, those in the amputation group had a longer hospital stay and higher estimated glomerular filtration rate(eGFR)levels.Fasting plasma glucose (FPG), glycosylated hemoglobin (HbA1c), C-reaction protein (CRP), ESR, ferritin, fibrinogen and WBC levels of the amputation group were higher , while hemoglobin albumin, transferrin, TC, TG, HDL-C and LDL-C were lower than those of the non-amputation group (all P<0.05 ).The proportion of hypertension ( 52.48% vs 59.98%) , peripheral vascular disease ( PAD ) (68.11% vs 25.04%), and coronary heart disease (21.33% vs 28.71%) were different between the amputation and non-amputation groups (all P<0.05).Multivariable logistic regression analyses showed that Wagner′s grade , PAD and CRP were the independent risk factors associated with lower extremity amputation in hospitalized patients with diabetic foot.Conclusion Wagner′s grade, ischemia of lower limbs and infection are closely associated with amputation of diabetic foot patients.

13.
Chinese Journal of Neurology ; (12): 852-855, 2016.
Article in Chinese | WPRIM | ID: wpr-501767

ABSTRACT

Objective To investigate the striatum neurochemistry in chorea-acanthocytosis(ChAc).Methods The brain samples of autopsy from 4 ChAc patients , 2 Huntington disease patients and 4 normal controls were collected.Immunostainings of enkephalin, substance P, glutamic acid decarboxylase (GAD) and Calbindin-D28k were carried out.Softwares were used to perform image and statistical analyses.Results In ChAc patients , the stainings of enkephalin , substance P and GAD were decreased , while the staining of Calbindin-D28k was increased.In comparison with normal controls , the staining of encephalin in the external segment of globus pallidus (16%(4%) vs 20%(1%),Mann-Whitney U test,Z=-2.337, P=0.029) and the stainings of substance P (12%(3%) vs 22%(1%),Mann-Whitney U test,Z=-2.352, P=0.029 ) and GAD in the internal segment of globus pallidus ( 25% ( 11%) vs 33%( 4%) , Mann-Whitney U test, Z =-2.323, P =0.029 ) were decreased in ChAc patients.Conclusions In ChAc patients, the decrease of substance P was more obvious than encephalin.The increase of Calbindin-D28k may be a protective compensation for the neuron damage.

14.
Clinical Medicine of China ; (12): 912-917, 2016.
Article in Chinese | WPRIM | ID: wpr-503646

ABSTRACT

Objective To identify high?risk groups of Charcot foot( CN) in the people with diabetic foot neuropathic ulcerations( NU) . Methods Twenty cases patients with CN who were diagnosed in General Hospital of the Chinese People Air Force from June 2008 to June 2013 and 58 patients with diabetic neuropathic ulcer who were hospitalized from January 2010 to December 2011 and followed up until June 2014 without foot deform?ity were retrospectively analyzed. All patient's general condition, examination and laboratory results, diabetic chronic complications,complication,diabetes distribution of foot ulcers,and plain features. Results There were no statistically significant differences in terms of patients' average age, sex ratio, proportion of smokers, BMI, HbA1c,blood lipid,dorsalis pedis artery diameter and diabetic nephropathy (Ⅲ?Ⅳperiod) ,chronic kidney dis?ease stage 3 above,proliferation diabetic retinal pathological changes,the prevalence of coronary heart disease between the two groups(P>0. 05). Compared with NU group,patients with single high proportion(40. 00%(8/20) vs. 10. 34%(6/58)),Short duration of diabetes((12. 37±5. 64) years vs. (14. 27±8. 04) years),Feet long numbness(6(5,9) years vs. 4(2,20) years),low rate of hardening of the arteries narrow(ABI<0. 9)( 0 ( 0/20) vs. 39. 66%( 13/58) ) ,high recurrent diabetic foot ulcer prevalence( 70. 00%( 14/20) vs. 25. 86%( 15/58)),more patients with diabetes mellitus autonomic neuropathy(75. 00%(15/20) vs. 39. 66%(23/58)),less combined with hypertension ( 25. 00%( 5/20 ) vs. 58. 62%( 34/58 ) ) , the differences were significant ( t orχ2=6. 981,2. 259,4. 068,3. 887,12. 405,7. 436,6. 724;P<0. 05) . Diabetic foot wound distribution on mesopodi?um of CN group and NU group was 36. 84%(7/19),6. 90%(4/58) respectively,the difference was significant (χ2=11. 443,P=0. 003) . Diabetic foot amputation rate( Wanger 4,5 grade) of CN group and NU group was 44. 44%(4/9),6. 90%(2/29) respectively,the difference was significant(χ2=4. 732,P=0. 020). Conclusion The characteristics of high?risk groups of diabetics Charcot foot in the people with diabetic foot neuropathic ulcerations are middle aged,no foot of ischemia,combine the diabetic autonomic neuropathy and the feet always with recurrent ulcers.

15.
Chinese Journal of Geriatrics ; (12): 578-582, 2015.
Article in Chinese | WPRIM | ID: wpr-475883
16.
Chinese Journal of Internal Medicine ; (12): 31-34, 2015.
Article in Chinese | WPRIM | ID: wpr-468624

ABSTRACT

Objective To understand pathological TDP-43 features in the central nervous systems of patients with clinically and autopsy confirmed motor neuron disease (MND).Methods The clinical and histopathological features of 4 cases with MND confirmed by autopsy were summarized; anti-ubiquitin (Ub) and anti-TDP-43 immunohistochemical staining were carried out on tissue of brains and spinal cords from 4 cases with MND and 3 control cases without history of neurological disorders.Results These 4 cases presented with typical clinical and histologic features of MND.Ub-positive inclusions were observed in brain and spinal cord from 3 cases with the Ub-positive inclusions of skein-round-and lewy body-like structures.Strong TDP-43 pathological staining in brain and spinal cord was identified in 2 cases with MND presented as neuronal and glial cytoplasmic inclusions with various shapes.The TDP-43 positive inclusions were widely distributed in the motor cortex of brain and the anterior horn of spinal cord.TDP-43 weak staining in the spinal cord tissue was observed in 1 case with MND.No Ub-and TDP-43 positive inclusions were found in 3 control cases.Conclusion There is widespread pathological TDP-43 expression in the central nervous system of MND.TDP-43 positive inclusions in MND have relatively high specificity.It is worth further study on their formation mechanism.

17.
Journal of China Medical University ; (12): 448-451, 2015.
Article in Chinese | WPRIM | ID: wpr-463120

ABSTRACT

Objective To establish a diagnostic scoring system for coronary artery disease(CAD)in patients with diabetic foot(DF)and evaluate its application efficiency. Methods The clinical data of 445 DF inpatients were analyzed retrospectively in this case?control study. These patients were divided into the CAD group(n=372,DF with CAD)and the control group(n=73,DF without CAD)according to the presence or absence of CAD. Risk factors were screened from related clinical factors examined through multiple logistic regression analysis for CAD in patients with DF and were assigned according to odds ratio(OR)to establish the scoring system for diagnosis of CAD in patients with DF. Application efficiency of the di?agnostic scoring system was tested by calculating area under the receiver operating characteristic(ROC)curve. Results The multiple logistic re?gression analysis showed that risk factors for CAD in patients with DF were age,male sex,the duration of diabetes≥10 years,the ankle?brachial in?dex(ABI)≤0.9,body mass index(BMI)≥25 kg/m2 and chronic renal insufficiency. According to ORassigned age(0.9=0,≤0.9=2),BMI(<25 kg/m2=0,≥25 kg/m2=3)and chronic renal insufficiency(absent=0,present=3)scores. Area under the ROC curve of the diagnostic score scheme was 0.758(0.682?0.835),the standard error was 0.039,and the point of the diagnosis of CAD was 7. Conclusion The scoring system established in the study is efficacious,simple and practical,which provides an important reference for CAD in patients with DF.

18.
Chinese Journal of Geriatrics ; (12): 676-679, 2015.
Article in Chinese | WPRIM | ID: wpr-466457

ABSTRACT

Objective To investigate the dementia caregivers' awareness of dementia diagnosis for patients aged≥80 years in veterans community in Beijing.Methods Patients aged 80 years and over who were diagnosed as dementia were selected from Dec.2009 to Jul.2011 in the veterans community in Beijing.A face-to-face interview and questionnaire survey were conducted in dementia caregivers individually,including basic social demographic data (gender,age,educational background),awareness rate of dementia,treatment and drug use situation and their attitude to dementia patients.Results A total of 118 caregivers were investigated individually.The caregivers in this study were mainly the spouse of the patients,and most of them were elderly people.46.6% (55/118) of caregivers were the spouse of the patients,and 52.5% (62/118) of caregivers were 65 years old and over.The caregivers' awareness rate of dementia was 32.3% (38/118) when patients were diagnosed as dementia.There was no correlations of gender,age and educational background with the awareness rate of dementia diagnosis (all P>0.05).50% of dementia patients received the dementia treatment,and only 17.8% (21/118) of patients got the first line drug treatment for dementia (acetylcholinesterase inhibitors and / or memantine).98.3 % (116 / 118) of caregivers agreed that the dementia patients should be treated kindly.Conclusions The dementia caregivers are mainly patients' spouse in veterans community and most of them are the elderly.The awareness rate of dementia diagnosis is low among the caregivers.Most patients do not take the formal treatment.The social support for the dementia caregivers should be strengthened.

19.
Chinese Journal of Pathology ; (12): 587-593, 2015.
Article in Chinese | WPRIM | ID: wpr-358960

ABSTRACT

<p><b>OBJECTIVE</b>To investigate histopathology and proteinopathy in the spinal cord of patients with common neurodegenerative diseases.</p><p><b>METHODS</b>Spinal cord tissues from clinically and neuropathologically confirmed neruodegnerative diseases were enrolled in this study, including 3 cases of multiple system strophy, 4 cases of amyotrophic lateral sclerosis, 5 cases of Alzheimer's disease (AD, included 2 cases of AD combined with Parkinson's disease), 2 cases of progressive supranuclear palsy, 1 case of dementia with lewy body and 1 case of corticobasal degeneration from 1955 to 2013 at Chinese People's Liberation Army General Hospital. Four normal control cases were also included. Routine HE and Gallyas-Braak staining, and immunohistochemical stainings for anti-PHF tau (AT8), anti-α-synuclein, anti-TDP-43 and anti-ubiquitin were performed.</p><p><b>RESULTS</b>Examination of the spinal cord in 3 cases with multiple system strophy revealed severe neuron loss in the intermediolateral nucleus of thoracic segment and Onuf's nucleus of the sacral segment, along with moderate neuron loss in the anterior horn of the cervical segment and mild myelin pallor in the anterior funiculus and anterolateral funiculus in the cervical and thoracic segments. Large amount of argentophilic, ubiquitin and synuclein positive oligodendroglial cytoplasmic inclusions were found widely distributed in the anterior horn and the anterior funiculus and anterolateral funiculus of the full spinal cord. Severe neuron loss and several morphological changes with gliosis in the anterior horn and severe loss of myelin in the anterior funiculus and anterolateral funiculus of the full spinal cord were observed in 4 cases of amyotrophic lateral sclerosis, 2 of which were found with Bunina bodies in neurons of the anterior horn. Three amyotrophic lateral sclerosis cases had ubiquitin-positive neuronal inclusions and TDP-43 positive neuronal and glial inclusions in the anterior horn at cervical and lumbar segments. A few argentophilic, tau positive neurofibrillary tangles (NFTs) and neuropil threads in the anterior horn at cervical and lumbar segments were found in 4 AD cases. Examination of spinal cord in 2 cases with Parkinson's disease combined with AD and 1 case with dementia with lewy body revealed severe neuron loss in the intermediolateral nucleus of thoracic segment, and a few synuclein positive lewy bodies and neuritis were also observed. There was mild neuron loss in the anterior horn at cervical and lumbar segments, along with some argentophilic, tau positive globous NFTs and many argentophilic, tau positive neutrophil threads were observed in 2 progressive supranuclear palsy cases and 1 corticobasal degeneration case.</p><p><b>CONCLUSION</b>Each common neurodegenerative diseases of the spinal cord including multiple system strophy, amyotrophic lateral sclerosis and Parkinson's disease has its own specific histopathology and proteinopathy characteristics.</p>


Subject(s)
Humans , Alzheimer Disease , Pathology , Amyotrophic Lateral Sclerosis , Pathology , DNA-Binding Proteins , Metabolism , Immunohistochemistry , Inclusion Bodies , Pathology , Neurodegenerative Diseases , Pathology , Neurofibrillary Tangles , Pathology , Neurons , Pathology , Parkinson Disease , Pathology , Spinal Cord , Pathology , Ubiquitin , Metabolism , alpha-Synuclein , Metabolism
20.
Chinese Journal of Pathology ; (12): 651-656, 2014.
Article in Chinese | WPRIM | ID: wpr-304424

ABSTRACT

<p><b>OBJECTIVE</b>To recognize relationship of protein related neurodegeneration abnormal aggregation in the aged brains with their cognitive and motor functions.</p><p><b>METHODS</b>Brain tissues from the consecutive autopsy cases of the aged from January 2005 to December 2006 in PLA General Hospital were carried out for immunohistochemical staining with beta amyloid, tau, α-synuclein and ubiquitin antibodies. The consortium to establish a registry for Alzheimer's disease (CERAD) was used to semi-quantitatively analyze Aβ positive core plaques density and Braak staging for tau positive neurofibrillary tangles (NFTs) and α-synuclein positive Lewy bodies. In addition, Aβ positive cerebral amyloid angiopathy (CAA), neuritic plaques and various ubiquitin positive structures were also observed. The relationship of these protein abnormal depositions in the aged brains with cognitive and motor functions were analyzed.</p><p><b>RESULTS</b>In brain tissues of 16 consecutive autopsy cases of the aged from 78 to 95 years, there were 13 cases with Aβ positive core plaques, their density was 2 cases with sparse, 2 cases with moderate and 9 cases with frequent, respectively, according to CREAD.Eight cases with Aβ positive CAA were found, including 6 cases of mild CAA and 2 cases of severe CAA. There were 12 cases with tau positive NFTs, including 6 cases with Braak stageI-II, 4 cases with stage III-IV and 2 cases with stage V-VI. There were 5 cases with frequent Aβ core plaques, meanwhile existing numerous tau/ubiquitin positive neuritic plaques and Braak stage IV-VI of tau positive NFTs, all of them presented cognitive dysfunction. Among 4 other cases with frequent Aβ core plaques, only one case coexisted α-synuclein positive Lewy bodies showed moderate cognitive impairment, remaining 3 cases did not present cognitive dysfunction. There were 4 cases with α-synuclein positive Lewy bodies in the brainstem, and all of these cases presented parkinsonian motor dysfunction. 13 cases with ubiquitin positive structures were found.</p><p><b>CONCLUSIONS</b>Beta amyloid protein positive deposit in the aged brain is an important marker of normal brain aging and cognitive impairment; frequent Aβ core plaques in the neocortex plus Braak IV and above tau positive NFTs are closely related to cognitive dysfunction of Alzheimer's disease; α-synuclein positive Lewy bodies in the brainstem is one of the important pathological markers of parkinsonian motor disorders; ubiquitin deposition involves the development of some characteristic structures of several neurodegenerative diseases.</p>


Subject(s)
Aged , Humans , Alzheimer Disease , Metabolism , Pathology , Amyloid beta-Peptides , Autopsy , Brain , Pathology , Brain Chemistry , Cerebral Amyloid Angiopathy , Neurofibrillary Tangles , Chemistry , Pathology , Plaque, Amyloid , Ubiquitin , alpha-Synuclein , tau Proteins
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