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Neuropathology and Applied Neurobiology

Susan C Shelmerdine, John Ciaran Hutchinson, Safa Al-Sarraj, Nat Cary, Tim Dawson, Daniel Du Plessis, Paul G Ince, Siobhan McLaughlin, Liina Palm, Colin Smith, Neil Stoodley, Rick van Rijn, Owen J Arthurs, Thomas S Jacques
AIMS: To develop an expert consensus statement regarding appropriate clinical and forensic post-mortem neurological imaging. METHODS: An expert panel of clinicians were recruited from registered members of the British Neuropathological Society (BNS) and the International Society of Forensic Radiology and Imaging (ISFRI) with post-mortem expertise. Following a focus group meeting, 16 core statements were incorporated into an online modified Delphi survey and each panellist was asked to score their level of agreement...
March 13, 2018: Neuropathology and Applied Neurobiology
J Bryan Iorgulescu, Sean Ferris, Ashima Agarwal, Sandro Casavilca Zambrano, D Ashley Hill, Robert Schmidt, Arie Perry
Meningioangiomatosis (MA) is a rare entity characterized by the perivascular spread of meningothelial and fibroblastic cells along the Virchow-Robin spaces of small leptomeningeal and intracortical blood vessels [1,2]. Sporadic cases of MA are thought to represent benign hamartomatous proliferations that are associated with refractory seizures and plaque-like cerebral hemispheric masses, primarily involving the temporal and/or frontal lobes. A distinct subset of MA presents in association with neurofibromatosis type 2 (NF2), where the MA is often multifocal, non-epileptogenic, and accompanied by perilesional glial microhamartomas [3,4]...
March 1, 2018: Neuropathology and Applied Neurobiology
Matthew MacGregor Sharp, Diederik Bulters, Sebastian Brandner, Janice Holton, Ajay Verma, David J Werring, Roxana O Carare
Aβ, amyloid beta, CAA, cerebral amyloid angiopathy, CT, computed tomographic scanning, IPAD, intramural periarterial drainage, ISF, interstitial fluid, MRI, magnetic resonance imaging, PVS, perivascular spaces, WMH, white matter hyperintensities.Cerebral white matter hyperintensities (WMH) observed on magnetic resonance imaging (MRI), or low attenuation on computed tomographic scanning (CT), are the most frequent brain imaging finding in patients with small vessel disease or dementia. It has been assumed that WMH are due to arteriosclerosis or blood-brain barrier breakdown, though recently it was demonstrated that WMH have distinct molecular signatures in Alzheimer's disease (AD) where markers of Wallerian degeneration are present, compared to normal ageing [1]...
February 27, 2018: Neuropathology and Applied Neurobiology
Giuseppina Catanzaro, Zein Mersini Besharat, Evelina Miele, Martina Chiacchiarini, Agnese Po, Andrea Carai, Carlo Efisio Marras, Manila Antonelli, Manuela Badiali, Alessandro Raso, Samantha Mascelli, Daniel Schrimpf, Damian Stichel, Marco Tartaglia, David Capper, Andreas von Deimling, Felice Giangaspero, Angela Mastronuzzi, Franco Locatelli, Elisabetta Ferretti
AIMS: Paediatric low-grade gliomas (pLGGs) are a heterogeneous group of brain tumours associated with a high overall survival: however, they are prone to recur and supratentorial lesions are difficult to resect, being associated with high percentage of disease recurrence. Our aim was to shed light on the biology of pLGGs. METHODS: We performed microRNA profiling on 45 fresh-frozen grade I tumour samples of various histological classes, resected from patients aged ≤ 16 years...
February 25, 2018: Neuropathology and Applied Neurobiology
Yazi D Ke, Lars M Ittner
Changes to the neuronal cytoskeleton, and in particular to microtubules, have been implicated in a wide range of neurodegenerative diseases, including Alzheimer's disease (AD) and frontotemporal dementia (FTD). As such, there are growing reports of microtubules-modulating drugs used to treat neurodegeneration in mice. For example, long-term treatment of a mutant tau transgenic mouse model of FTD and AD with low doses of the cancer drug Epothilone D (EpoD), a microtubule stabilizing compound, reduced cognitive deficits in young animals [1] and improved neuropathology in aged animals [2], without overt side effects...
February 13, 2018: Neuropathology and Applied Neurobiology
Jeremy J Pruzin, Peter T Nelson, Erin L Abner, Zoe Arvanitakis
Type 2 diabetes (T2D) and Alzheimer disease (AD) are both highly prevalent diseases worldwide, and each is associated with high-morbidity and high-mortality. Numerous clinical studies have consistently shown that T2D confers a two-fold increased risk for a dementia, including dementia attributable to AD. Yet, the mechanisms underlying this relationship, especially non-vascular mechanisms, remain debated. Cerebral vascular disease (CVD) is likely to be playing a role. But increased AD neuropathologic changes (ADNC), specifically neuritic amyloid plaques (AP) and neurofibrillary tangles (NFT), are also posited mechanisms...
February 8, 2018: Neuropathology and Applied Neurobiology
Hung-Wei Kan, Hao Chiang, Whei-Min Lin, I-Shing Yu, Shu-Wha Lin, Sung-Tsang Hsieh
AIMS: Sensory nerve degeneration and consequent abnormal sensations are the earliest and most prevalent manifestations of familial amyloid polyneuropathy (FAP) due to amyloidogenic transthyretin (TTR). FAP is a relentlessly progressive degenerative disease of the peripheral nervous system. However, there is a lack of mouse models to replicate the early neuropathic manifestations of FAP. METHODS: We established human TTR knock-in mice by replacing one allele of the mouse Ttr locus with human wild-type TTR (hTTRwt ) or human TTR with the A97S mutation (hTTRA97S )...
February 8, 2018: Neuropathology and Applied Neurobiology
Christina E Murray, Andrew King, Claire Troakes, Angela Hodges, Tammaryn Lashley
In late-onset Alzheimer's disease (AD), the ε4 allele of the apolipoprotein E gene (APOE) is the major known genetic risk factor [1]. In 2013 two research groups reported the R47H variant of triggering receptor expressed on myeloid cells 2 (TREM2), is associated with AD by almost as much as APOE ε4 [2,3]. A loss-of-function R47H mutation in TREM2 is also one of the strongest single allele genetic risk factors for AD [2,3], providing a link between microglia dysfunction and AD pathogenesis. TREM2 encodes a single-pass type I membrane protein that forms a receptor-signaling complex with the TYRO protein tyrosine kinase-binding protein (TYROBP) triggering immune responses in certain macrophages and dendritic cells...
February 7, 2018: Neuropathology and Applied Neurobiology
Akihiko Hoshi, Ayako Tsunoda, Teiji Yamamoto, Mari Tada, Akiyoshi Kakita, Yoshikazu Ugawa
AIMS: Glutamate neurotoxicity plays an important role in the pathogenesis of various neurodegenerative disorders. Many studies have demonstrated that glutamate transporter-1 (GLT-1), the dominant astrocytic glutamate transporter, is significantly reduced in the cerebral cortex of patients with Alzheimer's disease (AD), suggesting that glutamate-mediated excitotoxicity might contribute to the pathogenesis of AD. In a previous study, we have demonstrated marked alterations in the expression of the astrocytic water channel protein aquaporin-4 (AQP4) in relation to amyloid β deposition in human AD brains...
February 6, 2018: Neuropathology and Applied Neurobiology
Harry V Vinters, Chris Zarow, Ewa Borys, Jeffrey D Whitman, Spencer Tung, William G Ellis, Ling Zheng, Helena C Chui
The incidence and severity of cerebrovascular disease (CVD) increase with advancing age, as does the risk of developing Alzheimer disease (AD). Not surprisingly, heterogeneous forms of CVD may coexist with AD changes in the 'aging brain'. These include angiopathies (affecting both large and small arteries) that result from 'classical' risk factors (hypertension, smoking, diabetes) and others (cerebral amyloid angiopathy) that are biochemically closely linked to AD. The morphologic consequences of these various vascular diseases are infarcts and/or haemorrhages of varying sizes within the brain, which lead to neurocognitive decline that may mimic AD-though the vascular abnormalities are usually detectable by neuroimaging...
January 30, 2018: Neuropathology and Applied Neurobiology
Jayden A Clark, Catherine A Blizzard, Monique C Breslin, Elise J Yeaman, Ka M Lee, Jyoti A Chuckowree, Tracey C Dickson
Degeneration of the distal neuromuscular circuitry is a hallmark pathology of Amyotrophic Lateral Sclerosis (ALS). The potential for microtubule dysfunction to be a critical pathophysiological mechanism in the destruction of this circuitry is increasingly being appreciated. Stabilisation of microtubules to improve neuronal integrity and pathology has been shown to be a particularly favourable approach in other neurodegenerative diseases. We present evidence here that treatment with the microtubule-targeting compound Epothilone D (EpoD) both positively and negatively affects the spinal neuromuscular circuitry in the SOD1G93A mouse model of ALS METHODS: SOD1G93A mice were treated every 5 days with 2mg/kg EpoD...
January 30, 2018: Neuropathology and Applied Neurobiology
Melissa Leija-Salazar, Charlotte Lucie Piette, Christos Proukakis
Somatic mutations are post-zygotic mutations which may lead to mosaicism, the presence of cells with genetic differences in an organism. Their role in cancer is well established, but detailed investigation in health and other diseases has only been recently possible. This has been empowered by the improvements of sequencing techniques, including single cell sequencing, which can still be error-prone but is rapidly improving. Mosaicism appears relatively common in the human body, including the normal brain, probably arising in early development, but also potentially during ageing...
January 25, 2018: Neuropathology and Applied Neurobiology
Maria Thom
As epilepsy surgery programmes commenced in the 1950s, many eminent neuropathologists embraced the opportunity to study the varied lesions identified in relation to electroencephalography, neuroimaging, psychometry as well as outcome following removal of what was termed the 'alien' brain tissue [1-3]. Beyond their insightful and meticulous histological descriptions, they began to address the questions of what caused these lesions, how they gave rise to epilepsy (the processes of 'epileptogensis') and how they related to other co-morbidities or mortality associated with epilepsy...
January 23, 2018: Neuropathology and Applied Neurobiology
Imad Najm, Harvey B Sarnat, Ingmar Blümcke
The Diagnostic Methods commission of the International League against Epilepsy (ILAE) released a first international consensus classification of Focal Cortical Dysplasia (FCD) in 2011. Since that time, this FCD classification has been widely used in clinical diagnosis and research (more than 740 papers cited in Pubmed between 1/1/2012 and 7/1/2017). Herein, we review the new data that will inform and revise the FCD classification. Many recent papers described molecular-genetic characteristics in FCD Type II including multiple mutations in the mTOR pathway...
January 23, 2018: Neuropathology and Applied Neurobiology
Elise Marsan, Stéphanie Baulac
Over the last decade, there has been increasing evidence that hyperactivation of the mechanistic target of rapamycin (mTOR) pathway is a hallmark of focal cortical dysplasia (FCD), as well as other cortical malformations such as hemimegalencephaly (HME) or in tuberous sclerosis complex (TSC). The mTOR pathway governs protein and lipid synthesis, cell growth and proliferation as well as metabolism and autophagy. The molecular genetic aetiology of mTOR hyperactivation has only been recently clarified. This article will review the current and still evolving genetic advances in the elucidation of the molecular basis of FCD...
January 23, 2018: Neuropathology and Applied Neurobiology
Alessia Franceschini, Rosaria Strammiello, Sabina Capellari, Armin Giese, Piero Parchi
AIMS: To describe the regional profiles of microglial activation in sporadic Creutzfeldt-Jakob disease (sCJD) subtypes and analyse the influence of prion strain, disease duration and codon 129 genotype. METHODS: We studied the amount/severity and distribution of activated microglia, protease-resistant prion protein (PrPSc ) spongiform change, and astrogliosis in 8 regions of 57 brains representative of the entire spectrum of sCJD subtypes. RESULTS: In each individual subtype, the regional extent and distribution of microgliosis significantly correlated with PrPSc deposition and spongiform change, leading to subtype-specific "lesion profiles"...
January 18, 2018: Neuropathology and Applied Neurobiology
Aya Murakami, Masataka Nakamura, Satoshi Kaneko, Wen-Lang Lin, Dennis W Dickson, Hirofumi Kusaka
The human epidermal growth factor receptor family consists of 4 members that belong to the ErbB lineage of proteins (ErbB1-4). Neuregulin-1 (NRG1)/ErbB signalling regulates brain development and function. Abnormalities in this signalling have been implicated in the aetiology or development of neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, and amyotrophic lateral sclerosis. So, we aimed at investigating whether the expression of NRG1 or ErbB proteins are altered in progressive supranuclear palsy (PSP) METHODS: The brains of 10 PSP and 6 control patients were investigated by immunohistochemical analysis RESULTS: Whereas C-terminal ErbB4 immunoreacitivity was partially but distinctly present in the cytoplasm and/or in the nucleus of neurons in control patients, it was rarely observed in the neuronal nuclei in PSP patients...
January 10, 2018: Neuropathology and Applied Neurobiology
Thomas J Stone, Rachel Rowell, Bodiabaduge Ashan Prasanna Jayasekera, Mark O Cunningham, Thomas S Jacques
Brain tumours are the second most common cause of seizures identified in epilepsy surgical series. While any tumour involving the brain has the potential to cause seizures, specific subtypes are more frequently associated with epilepsy. Tumour-related epilepsy has a profound impact on patients with brain tumours and these seizures are often refractory to anti-epileptic treatments, resulting in long-term disability and patient morbidity. Despite the drastic impact epilepsy-associated tumours have on patients, they have not traditionally enjoyed as much attention as more malignant neoplasms...
January 6, 2018: Neuropathology and Applied Neurobiology
X Y Tai, B Bernhardt, M Thom, P Thompson, S Baxendale, M Koepp, N Bernasconi
Cognitive decline is increasingly described as a co-morbidity of temporal lobe epilepsy (TLE). Mechanisms underlying cognitive impairment are not fully understood despite examining clinical factors, such as seizure frequency, and cellular mechanisms of excitotoxicity. We review the neuropsychometry evidence for progressive cognitive decline and examine the pathology and neuroimaging evidence supporting a neurodegenerative process in hippocampal sclerosis (HS)-related TLE. Accelerated cognitive decline is described in groups of adult HS-related TLE patients...
December 30, 2017: Neuropathology and Applied Neurobiology
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March 2018: Neuropathology and Applied Neurobiology
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