Dorota Dziewulska
Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a form of cerebellar ataxia related to mutations in the SACS gene on chromosome 13q12.12 encoding sacsin protein. Characteristic clinical features are ataxia, spasticity, distal muscle wasting, neuropathy, dysarthria, nystagmus, and finger or feet deformities. The presented case concerns a 32-year-old man with clinical diagnosis of ARSACS. Magnetic resonance imaging (MRI) scans of the brain revealed cerebellar atrophy typical of the disease while neuroimaging of the C1-C3 and C6-Th12 segments showed only the thin thoracic spinal cord...
2020: Folia Neuropathologica
Jing Wang, Haibo Tong, Xiangyang Wang, Xinxing Wang, Yang Wang
Stroke is partial or complete brain dysfunction combined with acute cerebral circulatory disorders, and it affects millions of individuals around the world each year. A total of 70-80% of patients experience ischaemic stroke caused by disturbances in cerebral circulation, leading to cerebral ischaemia, neuronal apoptosis, and necrosis. Tanshinone IIA is a natural compound extracted from Salvia miltiorrhiza and has been proven to assist in recovery from cerebral ischaemia reperfusion injury. GLUT1 is ubiquitously expressed in all types of tissues in the human body and has important physiological functions due to its glucose uptake ability...
2020: Folia Neuropathologica
Yan Song, Qibing Huang, Zeli Zhang, Feng Li, Zhenkuan Xu
INTRODUCTION: Nuclear factor-kB (NF-kB) is a critical regulator of inflammatory responses following intracerebral haemorrhage (ICH). According to our previous study, inhibiting the p65 subunit at an early stage after ICH can reduce cell death, while inhibiting c-Rel at a late stage can lead to the opposite result. The aim of this study is to clarify whether patient prognosis can be improved by inhibiting p65 at the early stage and promoting c-Rel at the late stage. MATERIAL AND METHODS: Rats were divided into a sham group, ICH group, early NF-kB-inhibiting group using ammonium pyrrolidinedithiocarbamate (PDTC; group A, p65 subunit was dominant and inhibited at the early stage), late NF-kB-activating group using phorbol myristate acetate (PMA; group B, c-Rel was dominant and promoted at the late stage), and early NF-kB-inhibiting and late-activating group (group C, p65 subunit was inhibited at the early stage and c-Rel was promoted at the late stage)...
2020: Folia Neuropathologica
Md Sarikul Khan, Hiroaki Nabeka, Farzana Islam, Tetsuya Shimokawa, Shouichiro Saito, Tetsuya Tachibana, Seiji Matsuda
Spina bifida aperta (SBA), one of the most common congenital malformations, causes various neurological disorders. Pain is a common complaint of patients with SBA. However, little is known about the neuropathology of SBA-related pain. Because loss of g-aminobutyric acid GABAergic neurons in the spinal cord dorsal horn is associated with pain, we hypothesised the existence of crosstalk between SBA-related pain and alterations in GABAergic transmission in the spinal cord. Therefore, we investigated the kinetics of GABAergic transmission in the spinal cord dorsal horn in a chicken model of SBA...
2020: Folia Neuropathologica
Teresa Wierzba-Bobrowicz, Eliza Lewandowska, Paulina Felczak, Tomasz Stępień, Albert Acewicz, Sylwia Tarka, Beata Błażejewska-Hyżorek, Agnieszka Bednarska, Ewa Matyja, Wiesława Grajkowska
Herpes simplex encephalomyelitis (HSE) is a rare disease with a high mortality rate. Correct diagnosis is established on the basis of the combination of the clinical and investigative features. Unfortunately, precise diagnosis remains difficult due to several clinical similarities and false negative or inconclusive results of diagnostic tests. Here, we present two cases of HSE together with the morphological and ultrastructural picture. The first case was a 45-year-old man with acute symptoms of encephalitis, and the other one was a 28-year-old woman presenting subacute encephalomyelitis...
2020: Folia Neuropathologica
Swagatika Samal, Ashis Patnaik, Fakir Sahu, Suvendu Purkait
INTRODUCTION: Meningiomas comprises of a wide variety of histological entities with heterogeneous biological behaviour and prognosis. The plethora of genetic data are yet to produce relevant biomarkers for routine use. In contrast, epigenetic alterations are less elucidated. MATERIAL AND METHODS: The expression of the key molecules involved in the two principal epigenetic systems, i.e. DNA methylation (DNA methyltransferases [DNMT-1, -3A and -3B]) and histone modification (Enhancer of Zeste homolog-2 [EZH2] and trimethyl histone-3 [H3K27me3]) were assessed in 149 cases of meningiomas (grade I - 102, grade II - 47) by immunohistochemistry...
2020: Folia Neuropathologica
Renata Gruszka, Krzysztof Zakrzewski, Paweł P Liberski, Magdalena Zakrzewska
Nowadays molecular investigations have a significant impact on the understanding of primary brain tumour biology,as well as on their classification and progress in the treatment modalities. Among novel type of biomarkers with potential therapeutic value, microRNAs (miRNAs) are considered in some cases. miRNAs are small molecules regu-lating gene expression, including genes encoding key proteins involved in signalling pathways responsible for growth and cell survival during tumour formation. Incorrectly hyperactivated pathways implicated in brain tumour development are inter alia the PI3K/AKT/mTOR and RAS/MAPK/ERK cascades associated with worse prognosis and decreased patient survival...
2020: Folia Neuropathologica
Daniel Hernandez Baltazar, Rasajna Nadella, Abril Barrientos Bonilla, Yazmin Flores Martínez, Abraham Olguín, Paola Heman Bozadas, María Rovirosa Hernández, Iliana Cibrián Llanderal
Lipopolysaccharide (LPS) is a potent immunogen when administered locally and/or systemically. The peripheral immunization with LPS could contribute to the progression of neurological diseases because a strong link between neuroinflammation and dopaminergic degeneration has been found. The switch between the survival and neuronal death in substantia nigra could be related to M1 (neurotoxic) and M2 (neuroprotective) microglia phenotypes. In this review, we present the current findings about microglia roles, biomarkers, and natural or synthetic immune modulators determined in the LPS-based murine model...
2020: Folia Neuropathologica
Richard Armstrong
A hypothesis is proposed to explain the pathogenesis of neurodegenerative disease and the diversity of its phenotypes. The hypothesis is based on seven main propositions: 1) neurodegenerative disease is associated with multiple risk factors, 2) age is the most important of the risk factors, 3) aging differentially affects neuroanatomical pathways, 4) degeneration of these pathways results in the formation of pathogenic proteins, 5) pathogenic proteins spread along anatomical pathways, 6) the phenotypes of familial and sporadic forms of disease are similar and 7) neurodegenerative disease is characterised by heterogeneity, overlapping phenotypes, and multiple pathology...
2020: Folia Neuropathologica
Jolanta Dorszewska, Marta Kowalska, Tomasz Grzegorski, Dorota Dziewulska, Katarzyna Karmelita-Katulska, Anna-Maria Barciszewska, Michał Prendecki, Wojciech Gorczyński, Wojciech Kozubski
INTRODUCTION: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary, progressive ischemic disease of small vessels of the brain characterized by migraine with aura (MA), recurrent subcortical ischemic episodes, cognitive decline and psychiatric disorders. CADASIL is caused by mutations in the NOTCH3 gene. We identified the NOTCH3 Y189C mutation as a genetic cause of CADASIL in a Polish family and provided its first clinical manifestation...
2020: Folia Neuropathologica
Yanxiang Zhang, Qi Chen, Yu Nai, Chunni Cao
INTRODUCTION: The polarization state of microglia affects the progress of neuropathic pain. MiR-155 modulates polarization of microglia, while its role in neuropathic pain has not been well studied. MATERIAL AND METHODS: We separately used lipopolysaccharide (LPS) and interleukin 4 (IL-4) for constructing an M1/M2 polarization model in BV-2 cells. The levels of CD86, iNOS, CD206, Arg and miR-155 were measured by western blot or qRT-PCR, as needed. Subsequently, BV-2 cells were transfected with miR-155 mimics or inhibitor to explore the effects of miR-155 on polarization states...
2020: Folia Neuropathologica
Degang Han, Zheng Zhou, Jingpeng Liu, Tingting Wang, Jinbo Yin
Microglia, as the first line of defence of the central nervous system (CNS), has a major role in inflammatory response. It was reported that isoflurane has a neuroprotective role in the pathological process of CNS by interfering with inflammatory response. While the mechanism and function of isoflurane in microglia-mediated inflammation are still not clearly articulated. In our study, the inflammation model was established by the activation of lipopolysaccharide (LPS) in BV2 cells in vitro. The results demonstrated that isoflurane inhibited the release of nitric oxide (NO) and enhanced the survival of BV2 cells, meanwhile, isoflurane reduced the levels of inflammatory factors and downregulated the expressions of inflammation-related genes and proteins in LPS-mediated BV2 cells...
2020: Folia Neuropathologica
Yiqiang Cao, Yu Chen, Fei Wang, Yonggang Wang, Jiang Long
Trauma is the main cause of death for people aged 1-45, and among them, traumatic brain injury (TBI) is the major condition, which causes over 50,000 deaths each year and costs over 80 billion per year. Tetrahydroxystilbene glucoside (TSG) is the active ingredient of polygonum multiflorum, a traditional Chinese herbal medicine, which presented multiple pharmacological effects, including antioxidative, anti-inflammatory, reducing blood fat and neuroprotection effects. However, the effect of TSG in promoting the recovery of the nerve system after TBI is not fully understood...
2020: Folia Neuropathologica
Gokhan Gorgisen, Zafer Yaren
Glioblastoma multiforme (GBM) is the most common and malignant type of central nervous system tumours in adults. Strict regulation of glucose homeostasis has a significant role in GBM pathogenesis. Insulin receptor substrate 1 (IRS1) protein is the most important adaptor molecule involved in the regulation of glucose metabolism. It interacts with many cancer-related receptors and its overexpression is strongly associated with cell proliferation and survival. Our study was aimed to understand the role of IRS1 proteins in GBM cell viability...
2020: Folia Neuropathologica
Wei Li, Huisheng Chen, Zhijia Xu, Yan Lv, Ziai Zhao, Jinghua Zhang, Fang Qu
This investigation evaluated the neuroprotective effect of melodinhenine B in a cerebral ischemia/reperfusion (I/R)-induced neuronal injury rat model. The effect of melodinhenine B was determined by evaluating the neurological deficit score, cerebral infarcted area, and blood-brain barrier (BBB) permeability. Moreover, the level of inflammatory cytokines and expression of nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κβ), interleukin-1β (IL-1β), NLRP3, zonula occludens-1 (ZO-1), and occluding proteins were estimated by Western blotting...
2020: Folia Neuropathologica
Junna He, Xiangjian Zhang
Cerebral stroke is a major cause of brain injury due to the production of hypoxic conditions, and new therapeutic interventions are required for its management. Here, we evaluated the protective effect of miR-668 inhibitor against ischemia/reperfusion (I/R)-induced stroke. Cerebral stroke was induced by cerebral artery occlusion in rats, followed by treatment with miR-668 inhibitor for 10 minutes before reperfusion. The neuroprotective effect of miR-668 inhibitor was determined by estimating the neurological deficit score, cerebral infarct area and blood-brain barrier (BBB) permeability...
2020: Folia Neuropathologica
Kun Han, Chengyan Sun, Ning Jia, Rui Li, Ze Zhang, Yi Zhong
INTRODUCTION: Alzheimer's disease (AD) is still the fifth leading cause of death and most common dementia worldwide. To date, there is no efficient strategy that can slow down the progression of AD owing to delayed diagnosis and limited therapies. MiR-143-3p is up-regulated in serum of AD patients, yet the exact role it plays in AD pathology is still poorly understood. The aim of this study was to investigate the effect of miR-143-3p on neuronal survival. MATERIAL AND METHODS: We induced neuronal differentiation in SH-SY5Y cells using all-trans-retinoic acid (RA), and Aβ1-42 was used to establish the in vitro AD cell model...
2020: Folia Neuropathologica
Richard A Armstrong
Alzheimer's disease neuropathologic change (ADNC) in the form of β-amyloid (Aβ) deposits occurs not only in Alzheimer's disease (AD) and Down's syndrome (DS) but also as a 'co-pathology' in several disorders including dementia with Lewy bodies (DLB), corticobasal degeneration (CBD), and chronic traumatic encephalopathy (CTE). To determine whether cortical laminar degeneration, as measured by Aβ deposition, is similar in different disorders, changes in density of the diffuse, primitive, and classic morphological subtypes of Aβ deposit were studied across all cortical layers in the frontal and temporal cortex in AD, DS, DLB, CBD, and CTE using quantitative analysis and polynomial curve fitting...
2020: Folia Neuropathologica
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2019: Folia Neuropathologica
Jiri Soukup, Petra Kasparova, Milan Vajda, Ales Ryska
Meningiomas are the most common primary extra-axial tumours of the central nervous system, however their metastatic spread beyond central nervous system is rare. Here we present the case of a 54-year-old male with anaplastic meningioma who, 1.5 years after initial diagnosis, developed a tumorous expansion in his left adrenal gland, showing octreotide uptake on scintigraphy. Clinical diagnosis of pheochromocytoma was made and left adrenalectomy performed. The tumour weighed 480 grams and measured up to 140 mm in diameter...
2019: Folia Neuropathologica
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