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Guillain-Barré syndrome in northern China. The spectrum of neuropathological changes in clinically defined cases.

The pathology of the Guillain-Barré syndrome remains controversial, and autopsied cases available for study by contemporary techniques are uncommon. Large numbers of cases clinically diagnosed as Guillain-Barré syndrome occur in northern China. In this study we examined the neuropathological changes in 12 autopsied cases from Hebei Province, China. Eleven died early in the course of their disease. In all cases tissue was specially handled and fixed for electron microscopy and for immunocytochemistry. Three of these 12 cases had typical acute inflammatory demyelinating polyneuropathy (AIDP) wit

Preliminary NINDS neuropathologic criteria for Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy).

We present the preliminary neuropathologic criteria for progressive supranuclear palsy (PSP) as proposed at a workshop held at the National Institutes of Health, Bethesda, MD, April 24 and 25, 1993. The criteria distinguish typical, atypical, and combined PSP. A semiquantitative distribution of neurofibrillary tangles is the basis for the diagnosis of PSP. A high density of neurofibrillary tangles and neuropil threads in the basal ganglia and brain-stem is crucial for the diagnosis of typical PSP. Tau-positive astrocytes or their processes in areas of involvement help to confirm the diagnosis.

Neuropathologic analysis of postmortal brain samples of HIV-seropositive and -seronegative i.v. drug addicts.

Between 1987 and 1992 we performed forensic-pathologic and neuropathologic examinations on 70 cases of i.v. drug addicts who died of an overdose. In almost 25% of the cases HIV-positivity was revealed by serological examinations. In all the cases, irrespective of the result of the serological examination, a diffuse, reactive astrocytosis was the most prominent histologic alteration, accompanied by more or less pronounced regressive changes of this cell type in the deep layers of the white matter. Furthermore, also in all the cases, a slight to moderate, reactive proliferation of microglia coul

[An autopsy case of Degos disease with neurological symptoms--neuropathological observations and increased platelet aggregation].

We reported clinical and neuropathological observations of a 41-year-old man with Degos disease. He first noted painless skin lesions over the upper extremities in January, 1982. Three years later he was diagnosed as Degos disease by skin biopsy, and treatment with aspirin was started. In September, 1985, he complained of paresthesia on his right arm, followed by a series of new neurological manifestations suggesting multifocal spinal cord lesions. On October 28, examination of admission showed papules with central umblication over the whole body except the head, face, palms, soles and scrotum

Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases.

In this consecutive series of 230 patients with neuropathologically verified Creutzfeldt-Jakob disease (CJD), the disease was found to affect men and women with approximately equal frequency in a peak plateau between the ages of 55 and 75 years (mean, 61.5 years). Familial cases accounted for 4 to 8% of the total series. Nonspecific prodromal symptoms occurred in one third of the patients, and the neurological presentation, although usually a gradually evolving mental deterioration, was of rapid onset in 20% of patients and in 36% of patients consisted exclusively of neurological symptoms. The

Neuropathological study and chemico-pathological correlation in sibling cases of Sanfilippo syndrome type B.

Pathological and biochemical studies of two sibling cases (18-year-old girl and 16-year-old boy) with Sanfilippo syndrome type B are presented. Pathological study revealed distended neurons and neuronal processes with inclusions throughout the CNS and the visceral organs, and neuronal loss and corresponding gliosis. The authors paid particular attention to atrophic changes in the thalamus and to "dissociation gliomyélinique" in the cerebral white matter. Histochemistry and electron microscopy revealed regional differences in stored materials. Biochemical studies revealed an accumulation of gly

Astragalus polysaccharide alleviates neuropathology and cognitive deficits by modulating gut microbiota and neuroinflammation in an Alzheimer's disease model.

BACKGROUND: Emerging evidence indicates that the neuroprotective effects of Astragalus polysaccharides (APS), an extract compound and bioactive constituent derived from traditional Chinese herbs, may be relevant to an effective prescription for delaying progression of Alzheimer's disease (AD), yet the underlying mechanisms remain to be fully elucidated. This study aimed to investigate the therapeutic efficacy of APS in alleviating cognitive impairment and neuropathology in 5×FAD transgenic mice, with a specific focus on the regulatory role of the gut-brain axis. METHODS: Male 5×FAD mice were …

Traumatic brain injury and late-life Alzheimer's disease neuropathology: Quantitative investigation in the Adult Changes in Thought study.

BackgroundTraumatic brain injury (TBI) is associated with increased dementia risk, yet its relationship to Alzheimer's disease (AD) neuropathology is unclear. Prior autopsy studies show inconsistent results, constrained by limited TBI ascertainment, semi-quantitative neuropathology methods, and selection bias.ObjectiveTo examine whether TBI with loss of consciousness (TBI-LOC) is associated with quantitative measures of tau and amyloid-β42 (Aβ42) pathology in a community-based autopsy cohort.MethodsThe analytic sample included 810 Adult Changes in Thought study brain donors with baseline TBI-L…

Silmitasertib, an FDA-designated orphan CK2 Inhibitor, ameliorates neuropathology and motor dysfunction in a Huntington's disease mouse model.

Huntington's disease (HD) is a devastating autosomal dominant neurodegenerative disease that manifests with progressive motor, cognitive, and psychological impairments. HD is caused by a polyQ (CAG) repeat expansion in the huntingtin (HTT) gene, leading to the misfolding and aggregation of mutant HTT protein (mHTT) and the preferential degeneration of the striatum. Previously in our lab, we identified Protein Kinase CK2 as an important kinase involved in the pathophysiology of HD. Specifically, the alpha prime catalytic subunit of CK2 (CK2α') is upregulated in HD, and genetic depletion of CK2α

Disruption of BAG3-mediated BACE1 stabilization alleviates neuropathology and memory deficits in a mouse model of Alzheimer's disease.

β-Site amyloid precursor protein (APP)-cleaving enzyme 1 (BACE1) is the rate-limiting enzyme for amyloid-β (Aβ) generation and is considered promising drug target for Alzheimer's disease (AD). The co-chaperone BAG3 (Bcl-2-associated athanogene 3) plays an important role in maintaining intracellular protein homeostasis by regulating heat shock protein 70 (HSP70). Here, we reported that BAG3 expression was significantly elevated in AD. It interacted with and stabilized BACE1 by delaying its degradation through ubiquitin-proteasome and autophagy-lysosomal pathways. BAG3E455K and BAG3R480A mutatio

Association of [18F]Flortaucipir-PET and plasma p-tau217 with tau neuropathology in AD and other neurodegenerative disorders.

BACKGROUND AND OBJECTIVES: Evaluating tau biomarkers in patients with available autopsy data is critical for validating their sensitivity and specificity to detect Alzheimer's disease neuropathologic changes (ADNC). We examined the association between tau-PET (using [18F]Flortaucipir), plasma ptau-217, and measures of AD neuropathology in a group of clinically-impaired participants from a tertiary dementia center, including patients with AD and FTLD. METHODS: We analyzed Flortaucipir-PET (80-100 minutes post-injection acquisition, normalized to inferior cerebellar cortex) from 73 patients wit…

The profiling of extracellular vesicle subtypes in Huntington's disease brains identifies Alix as a novel marker of neuropathology.

BACKGROUND: Huntington's disease (HD) is the most frequent autosomal dominant neurodegenerative disorder, which is caused by a CAG repeat expansion in the HTT gene. Despite its well-defined genetic origin, there is currently no cure, and reliable biomarkers for disease progression and pathophysiology remain limited. Mutant huntingtin protein accumulates in endosomal compartments, disrupting endosomal trafficking and potentially affecting the biogenesis, release, and cargo of exosomes-extracellular vesicles (EVs) derived from the endosomal pathway. However, the role of exosomes in HD pathogenes

The Simplified Edinburgh Criteria in Clinical Practice: A CT-Neuropathology Accuracy Study for Diagnosis of Cerebral Amyloid Angiopathy.

BACKGROUND AND OBJECTIVES: Cerebral amyloid angiopathy (CAA) is a major cause of lobar intracerebral hemorrhage (ICH) in older patients and an important contributor to cognitive decline and recurrent hemorrhagic stroke. Diagnosis of CAA is commonly based on MRI findings interpreted according to established criteria. Recently, the simplified Edinburgh criteria were proposed as a CT-based alternative to detect CAA in patients presenting with lobar ICH, potentially enabling faster diagnosis in emergency settings. The aim of this study was to evaluate the diagnostic accuracy of the simplified Edin

Временное развитие аксоновой дегенерации в визуальной системе в экспериментальной аутоиммунной энцефаломиэлите: Отчёты от нейропатологии с высоким разрешением.

Множественный склероз (MS) характеризуется воспалением, обезвоживанием и аксоновой дегенерацией в CNS, что приводит к постепенной неврологической инвалидности, как правило, рассматривается как аутоиммунное расстройство. Визуальное расстройство, частый симптом, является результатом повреждения клеток сетчатки (КЖК) и их аксонов на переднем визуальном пути. Используя экспериментальную модель аутоиммунного энцефаломиелита (EAE) в мышах, мы использовали несколько методов, включая нейропатологию с высоким разрешением с использованием новой иммуногистохимической технологии на сечениях ультратина, дл

Совпадение в аннотации оригинала
Multiple sclerosis (MS) is characterized by inflammation, demyelination, and axonal degeneration in the CNS, leading to progressive neurolog … utoimmune encephalomyelitis (EAE) model in mice, we used several methods, including high-resolution neuropathology with a novel immunohistochemical technique on ultrathin sections, to characterize axonal pathology and demyelination, terminal disruption, perikaryal degeneration, and visual acuity. Electron microscopy demonstrated early axonopathy and myelin split …

Cognitive integrity in Non-Demented Individuals with Alzheimer's Neuropathology is associated with preservation and remodeling of dendritic spines.

INTRODUCTION: Individuals referred to as Non-Demented with Alzheimer's Neuropathology (NDAN) exhibit cognitive resilience despite presenting Alzheimer's disease (AD) histopathological signs. Investigating the mechanisms behind this resilience may unveil crucial insights into AD resistance. METHODS: DiI labeling technique was used to analyze dendritic spine morphology in control (CTRL), AD, and NDAN post mortem frontal cortex, particularly focusing on spine types near and far from amyloid beta (Aβ) plaques. RESULTS: NDAN subjects displayed a higher spine density in regions distant from Aβ pla…

Neuropathology of patients with preclinical or early clinical Alzheimer's disease with pathogenic PSEN1_p. L392V: Comparison of advanced siblings.

INTRODUCTION: Neuropathological investigation of presymptomatic or early symptomatic presenilin-1 (PSEN1) mutation carriers in familial Alzheimer's disease (AD) is extremely scarce. METHODS: We report the autopsy findings of brothers with familial AD. Case 1 is a 45-year-old man without obvious cognitive impairment, who committed suicide. Case 2 is a 57-year-old older brother of Case 1 with advanced AD symptoms, who died of hypothermia during wondering. RESULTS: In both cases, abundant amyloid plaques positive for amyloid β (Aβ) were found throughout the brain. Progression of neuronal loss a

The potential role of brain renin-angiotensin system in the neuropathology of Parkinson disease: Friend, foe or turncoat?

Parkinson disease (PD) is one of the most common neurodegenerative diseases of the brain. Of note, brain renin-angiotensin system (RAS) is intricate in the PD neuropathology through modulation of oxidative stress, mitochondrial dysfunction and neuroinflammation. Therefore, modulation of brain RAS by angiotensin receptor blockers (ARBs) and angiotensin-converting enzyme inhibitors (ACEIs) may be effective in reducing the risk and PD neuropathology. It has been shown that all components including the peptides and enzymes of the RAS are present in the different brain areas. Brain RAS plays a crit…

Gerstmann-Sträussler-Scheinker Disease Presenting as Late-Onset Slowly Progressive Spinocerebellar Ataxia, and Comparative Case Series with Neuropathology.

BACKGROUND: Genetic prion diseases, including Gerstmann-Sträussler-Scheinker disease (GSS), are extremely rare, fatal neurodegenerative disorders, often associated with progressive ataxia and cognitive/neuropsychiatric symptoms. GSS typically presents as a rapidly progressive cerebellar ataxia, associated with cognitive decline. Late-onset cases are rare. OBJECTIVE: To compare a novel GSS phenotype with six other cases and present pathological findings from a single case. METHODS: Case series of seven GSS patients, one proceeding to autopsy. RESULTS: Case 1 developed slowly progressive gait

Optimal blood tau species for the detection of Alzheimer's disease neuropathology: an immunoprecipitation mass spectrometry and autopsy study.

Plasma-to-autopsy studies are essential for validation of blood biomarkers and understanding their relation to Alzheimer's disease (AD) pathology. Few such studies have been done on phosphorylated tau (p-tau) and those that exist have made limited or no comparison of the different p-tau variants. This study is the first to use immunoprecipitation mass spectrometry (IP-MS) to compare the accuracy of eight different plasma tau species in predicting autopsy-confirmed AD. The sample included 123 participants (AD = 69, non-AD = 54) from the Boston University Alzheimer's disease Research Center who