Neuropathology and Applied Neurobiology

Article	Citations
Intracerebral endotheliitis and microbleeds are neuropathological features of COVID‐19	92
Invited Review: The spectrum of neuropathology in COVID‐19	92
Dysfunction of the blood–brain barrier in Alzheimer's disease: Evidence from human studies	67
Peripheral inflammation exacerbates α‐synuclein toxicity and neuropathology in Parkinson's models	57
Neuropathological findings in two patients with fatal COVID‐19	54
Classification of diseases with accumulation of Tau protein	46
Deep learning‐based model for diagnosing Alzheimer's disease and tauopathies	43
Systematic review of human post‐mortem immunohistochemical studies and bioinformatics analyses unveil the complexity of astrocyte reaction in Alzheimer's disease	40
Cell senescence in neuropathology: A focus on neurodegeneration and tumours	39
Loss of the metabolism and sleep regulating neuronal populations expressing orexin and oxytocin in the hypothalamus in amyotrophic lateral sclerosis	35
Distinct brain‐derived TDP‐43 strains from FTLD‐TDP subtypes induce diverse morphological TDP‐43 aggregates and spreading patterns in vitro and in vivo	29
A case series of Diffuse Glioneuronal Tumours with Oligodendroglioma‐like features and Nuclear Clusters (DGONC)	29
Impaired brain insulin signalling in Parkinson's disease	28
Utrophin modulator drugs as potential therapies for Duchenne and Becker muscular dystrophies	26
Identification of intraneuronal amyloid beta oligomers in locus coeruleus neurons of Alzheimer’s patients and their potential impact on inhibitory neurotransmitter receptors and neuronal excitability	25
A systems‐level analysis highlights microglial activation as a modifying factor in common epilepsies	24
Machine learning‐based decision tree classifier for the diagnosis of progressive supranuclear palsy and corticobasal degeneration	23
STochastic Optical Reconstruction Microscopy (STORM) reveals the nanoscale organization of pathological aggregates in human brain	22
Correlations in post‐mortem imaging‐histopathology studies of sporadic human cerebral small vessel disease: A systematic review	21
Hippocampal subfield pathologic burden in Lewy body diseases vs. Alzheimer’s disease	20
NOTCH3 variant position is associated with NOTCH3 aggregation load in CADASIL vasculature	20
Neurodegenerative movement disorders: An epigenetics perspective and promise for the future	20
Robust methylation‐based classification of brain tumours using nanopore sequencing	20
Balloon cells promote immune system activation in focal cortical dysplasia type 2b	19
Novel clinicopathological characteristics differentiate dementia with Lewy bodies from Parkinson’s disease dementia	19

Necrosome‐positive granulovacuolar degeneration is associated with TDP‐43 pathological lesions in the hippocampus of ALS/FTLD cases	18
Heterozygous CAPN3 missense variants causing autosomal‐dominant calpainopathy in seven unrelated families	18
Olfactory bulb SARS‐CoV‐2 infection is not paralleled by the presence of virus in other central nervous system areas	18
Intranuclear inclusions in skin biopsies are not limited to neuronal intranuclear inclusion disease but can also be seen in oculopharyngodistal myopathy	17
Molecular pathophysiology of human MICU1 deficiency	17
The S1PR2‐CCL2‐BDNF‐TrkB pathway mediates neuroinflammation and motor incoordination in hyperammonaemia	17
Neutrophil extracellular traps (NETs) infiltrate haematoma and surrounding brain tissue after intracerebral haemorrhage: A post‐mortem study	17
The autophagy‐enhancing drug carbamazepine improves neuropathology and motor impairment in mouse models of Machado–Joseph disease	17
Spinal manifestations of CLN1 disease start during the early postnatal period	17
Expression pattern of perilipins in human brain during aging and in Alzheimer's disease	17
Signs of early cellular dysfunction in multiple system atrophy	17
Concurrent tau pathologies in frontotemporal lobar degeneration with TDP‐43 pathology	16
Sex differences in the neuropathological hallmarks of Alzheimer’s disease: focus on cognitively intact elderly individuals	16
Non‐coding regulatory elements: Potential roles in disease and the case of epilepsy	16
Impaired myelin production due to an intrinsic failure of oligodendrocytes in mTORpathies	16
Proteomic characterisation of polyglucosan bodies in skeletal muscle in RBCK1 deficiency	15
MiR‐142‐3p regulates synaptopathy‐driven disease progression in multiple sclerosis	15
TRAPPC11‐related muscular dystrophy with hypoglycosylation of alpha‐dystroglycan in skeletal muscle and brain	15
Faster disease progression in Parkinson’s disease with type 2 diabetes is not associated with increased α‐synuclein, tau, amyloid‐β or vascular pathology	15
CD47 promotes the proliferation and migration of adamantinomatous craniopharyngioma cells by activating the MAPK/ERK pathway, and CD47 blockade facilitates microglia‐mediated phagocytosis	15
A rare case of paediatric astroblastoma with concomitant MN1‐GTSE1 and EWSR1‐PATZ1 gene fusions altering management	14
HnRNP K mislocalisation in neurons of the dentate nucleus is a novel neuropathological feature of neurodegenerative disease and ageing	14
G protein‐coupled receptor kinases are associated with Alzheimer's disease pathology	14
Significance of cytosolic cathepsin D in Alzheimer's disease pathology: Protective cellular effects of PLGA nanoparticles against β‐amyloid‐toxicity	14
Tumour‐associated CD204+ microglia/macrophages accumulate in perivascular and perinecrotic niches and correlate with an interleukin‐6‐enriched inflammatory profile in glioblastoma	14
RAPGEF2 mediates oligomeric Aβ‐induced synaptic loss and cognitive dysfunction in the 3xTg‐AD mouse model of Alzheimer’s disease	14
Arteriolar neuropathology in cerebral microvascular disease	14
Lipid alterations in human frontal cortex in ALS‐FTLD‐TDP43 proteinopathy spectrum are partly related to peroxisome impairment	13
The prognostic significance of clinicopathological features in meningiomas: Microscopic brain invasion can predict patient outcome in otherwise benign meningiomas	13
The blood–CSF–brain route of neurological disease: The indirect pathway into the brain	13
Irisin treatment lowers levels of phosphorylated tau in the hippocampus of pre‐symptomatic female but not male htau mice	13
Concomitant LATE‐NC in Alzheimer's disease is not associated with increased tau or amyloid‐β pathological burden	13
BRAIN UK: Accessing NHS tissue archives for neuroscience research	13
The molecular landscape and associated clinical experience in infant medulloblastoma: prognostic significance of second‐generation subtypes	13
DNA methylation profiling improves routine diagnosis of paediatric central nervous system tumours: A prospective population‐based study	13
Neuron types in the primate striatum: Stereological analysis of projection neurons and interneurons in control and parkinsonian monkeys	12
Accurate calling of KIAA1549‐BRAF fusions from DNA of human brain tumours using methylation array‐based copy number and gene panel sequencing data	12
Characterisation of early ultrastructural changes in the cerebral white matter of CADASIL small vessel disease using high‐pressure freezing/freeze‐substitution	12
Old age genetically confirmed frontotemporal lobar degeneration with TDP‐43 has limbic predominant TDP‐43 deposition	12
L‐DOPA regulates α‐synuclein accumulation in experimental parkinsonism	12
Heterozygous APOE Christchurch in familial Alzheimer’s disease without mutations in other Mendelian genes	11
Potent T cell‐mediated anti‐inflammatory role of the selective CB2 agonist lenabasum in multiple sclerosis	11
Transcriptional signatures of synaptic vesicle genes define myotonic dystrophy type I neurodegeneration	11
Targeted next‐generation sequencing of adult gliomas for retrospective prognostic evaluation and up‐front diagnostics	11
Emerging roles for the YAP/TAZ transcriptional regulators in brain tumour pathology and targeting options	11
MOBP and HIP1 in multiple system atrophy: New α‐synuclein partners in glial cytoplasmic inclusions implicated in the disease pathogenesis	11
Anaplastic ganglioglioma—A diagnosis comprising several distinct tumour types	11
Protein homeostasis in LGMDR9 (LGMD2I) – The role of ubiquitin–proteasome and autophagy–lysosomal system	11
Telomeric alterations in the default mode network during the progression of Alzheimer’s disease: Selective vulnerability of the precuneus	10
Alpha adaptins show isoform‐specific association with neurofibrillary tangles in Alzheimer's disease	10

AKT1E17K‐mutated meningioma cell lines respond to treatment with the AKT inhibitor AZD5363	10
Diagnostic accuracy of 1p/19q codeletion tests in oligodendroglioma: A comprehensive meta‐analysis based on a Cochrane systematic review	10
Invited Review – A 5‐year update on epigenome‐wide association studies of DNA modifications in Alzheimer’s disease: progress, practicalities and promise	10
Extracellular matrix remodelling is associated with muscle force increase in overloaded mouse plantaris muscle	10
The severity of behavioural symptoms in FTD is linked to the loss of GABRQ‐expressing VENs and pyramidal neurons	10
Systemic delivery of human GlyR IgG antibody induces GlyR internalization into motor neurons of brainstem and spinal cord with motor dysfunction in mice	10
Seizure activity and brain damage in a model of focal non‐convulsive status epilepticus	10
Post‐treatment hypermutation in a recurrent diffuse glioma with H3.3 p.G34 Mutation	9
Combinatorial model of amyloid β and tau reveals synergy between amyloid deposits and tangle formation	9
Cauda equina paragangliomas express HOXB13	9
Pathogenic variants of Valosin‐containing protein induce lysosomal damage and transcriptional activation of autophagy regulators in neuronal cells	9
Cutaneous sensory and autonomic denervation in progressive supranuclear palsy	9
Neuropathology of genetically defined malformations of cortical development—A systematic literature review	9
Capillary pathology with prominent basement membrane reduplication is the hallmark histopathological feature of scleromyositis	9
ALK‐rearranged histiocytosis: Report of two cases with involvement of the central nervous system	9
The desmin mutation R349P increases contractility and fragility of stem cell‐generated muscle micro‐tissues	9
Splicing factor proline and glutamine rich intron retention, reduced expression and aggregate formation are pathological features of amyotrophic lateral sclerosis	9
Delineating selective vulnerability of inhibitory interneurons in Alpers' syndrome	9
Automated immunofluorescence analysis for sensitive and precise dystrophin quantification in muscle biopsies	9
Genetic knockdown of Klk8 has sex‐specific multi‐targeted therapeutic effects on Alzheimer’s pathology in mice	9
Low‐grade epilepsy‐associated neuroepithelial tumours with a prominent oligodendroglioma‐like component: The diagnostic challenges	8
CircRNA SRRM4 affects glucose metabolism by regulating PKM alternative splicing via SRSF3 deubiquitination in epilepsy	8
Gonadotroph tumours with a low SF‐1 labelling index are more likely to recur and are associated with enrichment of the PI3K‐AKT pathway	8
Expression, prognostic significance and therapeutic implications of PD‐L1 in gliomas	8
Modulation of mitochondrial and inflammatory homeostasis through RIP140 is neuroprotective in an adrenoleukodystrophy mouse model	8
Implementation of TERT promoter mutations improve prognostication of the WHO classification in meningioma	8
Prodromal sensory neuropathy in Pink1−/−SNCAA53T double mutant Parkinson mice	8
Identifying cellular signalling molecules in developmental disorders of the brain: Evidence from focal cortical dysplasia and tuberous sclerosis	8
Cortical morphometric vulnerability to generalised epilepsy reflects chromosome‐ and cell type‐specific transcriptomic signatures	8
Axonal response of mitochondria to demyelination and complex IV activity within demyelinated axons in experimental models of multiple sclerosis	8
Ataxic phenotype and neurodegeneration are triggered by the impairment of chaperone‐mediated autophagy in cerebellar neurons	8
Brain TDP‐43 pathology in corticobasal degeneration: Topographical correlation with neuronal loss	8
Pathological substrate of memory impairment in multiple system atrophy	8
Advanced molecular pathology for rare tumours: A national feasibility study and model for centralised medulloblastoma diagnostics	8
The contribution of brain banks to knowledge discovery in amyotrophic lateral sclerosis: A systematic review	7
Epigenetic age acceleration is associated with oligodendrocyte proportions in MSA and control brain tissue	7
Blinded review of hippocampal neuropathology in sudden unexplained death in childhood reveals inconsistent observations and similarities to explained paediatric deaths	7
Caspase‐6‐cleaved tau is relevant in Alzheimer's disease and marginal in four‐repeat tauopathies: Diagnostic and therapeutic implications	7
Extracellular tau oligomers affect extracellular glutamate handling by astrocytes through downregulation of GLT‐1 expression and impairment of NKA1A2 function	7
Microglial contribution to synaptic uptake in the prefrontal cortex in schizophrenia	7
Morphological differences between dementia with Lewy bodies and Parkinson's disease‐dementia	7
Activating ATF6 in spinal muscular atrophy promotes SMN expression and motor neuron survival through the IRE1α‐XBP1 pathway	7
Optimised tissue clearing minimises distortion and destruction during tissue delipidation	7
Respiratory chain dysfunction in perifascicular muscle fibres in patients with dermatomyositis is associated with mitochondrial DNA depletion	6
Early changes in visuospatial episodic memory can help distinguish primary age‐related tauopathy from Alzheimer’s disease	6
Rosette‐forming glioneuronal tumours are midline, FGFR1‐mutated tumours	6
Met166‐Glu168 residues in human PrP β2‐α2 loop account for evolutionary resistance to prion infection	6
Primary age‐related tauopathy in a Finnish population‐based study of the oldest old (Vantaa 85+)	6
Early loss of locus coeruleus innervation promotes cognitive and neuropathological changes before amyloid plaque deposition in a transgenic rat model of Alzheimer's disease	6
Recurrent posterior fossa group A (PFA) ependymoma in a young child with constitutional mismatch repair deficiency (CMMRD)	6
A high‐throughput single‐cell RNA expression profiling method identifies human pericyte markers	6
Inflammation, fibrosis and skeletal muscle regeneration in LGMDR9 are orchestrated by macrophages	6
A simple novel approach for detecting blood–brain barrier permeability using GPCR internalization	6
Dysregulation of miR‐543 in Parkinson's disease: Impact on the neuroprotective gene SIRT1	6
Cytoplasmic HDAC4 recovers synaptic function in the 3×Tg mouse model of Alzheimer's disease	6
A TOR1AIP1 variant segregating with an early onset limb girdle myasthenia—Support for the role of LAP1 in NMJ function and disease	6
Paediatric astroblastoma‐like neuroepithelial tumour of the spinal cord with a MAMLD1‐BEND2 rearrangement	6
TGF‐β activates pericytes via induction of the epithelial‐to‐mesenchymal transition protein SLUG in glioblastoma	6
CuII(atsm) significantly decreases microglial reactivity in patients with sporadic amyotrophic lateral sclerosis	6
Spatial molecular profiling of a central nervous system low‐grade diffusely infiltrative tumour with INI1 deficiency featuring a high‐grade atypical teratoid/rhabdoid tumour component	6
An autopsy case of pure nigropathy with TUBA4A nonsense mutation	6
Treatment with the copper compound CuATSM has no significant effect on motor neuronal pathology in patients with ALS	6
IDH clonal heterogeneity segregates a subgroup of non‐1p/19q codeleted gliomas with unfavourable clinical outcome	5
Identification of a novel heterozygous DYSF variant in a large family with a dominantly‐inherited dysferlinopathy	5
The interaction of insoluble Amyloid‐β with soluble Amyloid‐β dimers decreases Amyloid‐β plaque numbers	5
Low‐grade diffusely infiltrative tumour (LGDIT), SMARCB1‐mutant: A clinical and histopathological distinct entity showing epigenetic similarity with ATRT‐MYC	5
Plectin‐related scapuloperoneal myopathy with treatment‐responsive myasthenic syndrome	5
Novel naturally occurring autoantibodies attenuate α‐synuclein pathology in a mouse model of Parkinson's disease	5
Nanoscale reorganisation of synaptic proteins in Alzheimer's disease	5
Intrathecal amyloid‐beta oligomer administration increases tau phosphorylation in the medial temporal lobe in the African green monkey: A nonhuman primate model of Alzheimer's disease	5
Myeloid cell subpopulations compensate each other for Ccr2‐deficiency in glioblastoma	5
Past antihypertensive medication use is associated with lower levels of small vessel disease and lower Aβ plaque stage in the brains of older individuals	5
Identification of the calpain‐generated toxic fragment of ataxin‐3 protein provides new avenues for therapy of Machado–Joseph disease| Spinocerebellar ataxia type 3	5
MicroRNA‐34a activation in tuberous sclerosis complex during early brain development may lead to impaired corticogenesis	5
m6A mRNA methylation in human brain is disrupted in Lewy body disorders	5
The molecular characteristics of low‐grade and high‐grade areas in desmoplastic infantile astrocytoma/ganglioglioma	5
GAB1 overexpression identifies hedgehog‐activated anterior skull base meningiomas	5
Lack of difference between amyloid‐beta burden at gyral crests and sulcal depths in diverse neurodegenerative diseases	5
The clinico‐pathological characterisation of focal cortical dysplasia type IIb genetically defined by MTOR mosaicism	5
Atypical astroglial pTDP‐43 pathology in astroglial predominant tauopathy	4

The SPTLC1 p.S331 mutation bridges sensory neuropathy and motor neuron disease and has implications for treatment	4
Abundant copathologies of polyglucosan bodies, frontotemporal lobar degeneration with TDP‐43 inclusions and ageing‐related tau astrogliopathy in a family with a GBE1 mutation	4
Long‐standing multiple system atrophy‐Parkinsonism with limbic and FTLD‐type α‐synuclein pathology	4
Interfering with lysophosphatidic acid receptor edg2/lpa1 signalling slows down disease progression in SOD1‐G93A transgenic mice	4
Novel autophagic vacuolar myopathies: Phenotype and genotype features	4
MBNL‐dependent impaired development within the neuromuscular system in myotonic dystrophy type 1	4
What to do with unusual TDP‐43 proteinopathy cases?	4
Absence of tissue transglutaminase reduces amyloid‐beta pathology in APP23 mice	4
Neuronal infection is a major pathogenetic mechanism and cause of fatalities in human acute Nipah virus encephalitis	4
Novel therapeutic targets in epilepsy: oxidative stress and iron metabolism	4
WNT‐activated, MYC‐amplified medulloblastoma displaying intratumoural heterogeneity	4
Linking epigenetic signature and metabolic phenotype in IDH mutant and IDH wildtype diffuse glioma	4
Inflammatory bowel disease induces pathological α‐synuclein aggregation in the human gut and brain	4
Muscle biopsy practices in the evaluation of neuromuscular disease: A systematic literature review	4
TDP‐43 proteinopathy occurs independently of autophagic substrate accumulation and underlies nuclear defects in Niemann‐Pick C disease	4
Distinguishing post‐translational modifications in dominantly inherited frontotemporal dementias: FTLD‐TDP Type A (GRN) vs Type B (C9orf72)	4
Intelligence quotient–genotype association in dystrophinopathies: A systematic review and meta‐analysis	3
Time to focus on circulating nucleic acids for diagnosis and monitoring of gliomas: A systematic review of their role as biomarkers	3
A novel TRMT5 mutation causes a complex inherited neuropathy syndrome: The role of nerve pathology in defining a demyelinating neuropathy	3
Image‐based deep learning reveals the responses of human motor neurons to stress and VCP‐related ALS	3
Schwann cells and myelin in human peripheral nerve: Major protein components vary with age, axon size and pathology	3
N471D WASH complex subunit strumpellin knock‐in mice display mild motor and cardiac abnormalities and BPTF and KLHL11 dysregulation in brain tissue	3
The analysis of epidermal nerve fibre spatial distribution improves the diagnostic yield of skin biopsy	3
TAPPing into the potential of inducible tau/APP transgenic mice	3
Molecular landscape of IDH‐wild‐type, H3‐wild‐type glioblastomas of adolescents and young adults	3
RNA sequencing of peripheral blood in amyotrophic lateral sclerosis reveals distinct molecular subtypes: Considerations for biomarker discovery	3
Hippocampal subfield pathologic Burden in Lewy body diseases versus Alzheimer’s disease	3
First report of medulloblastoma in a patient with MUTYH‐associated polyposis	3
Insights into the pathological basis of dementia from population‐based neuropathology studies	3
The genomic landscape of dysembryoplastic neuroepithelial tumours and a comprehensive analysis of recurrent cases	3
CD8 T‐cell‐mediated cerebellitis directed against Purkinje cell antigen after ipilimumab for small cell lung cancer	3
Evidence of neural crest cell origin of a DICER1 mutant CNS sarcoma in a child with DICER1 syndrome and NRAS‐mutant neurocutaneous melanosis	3
Unveiling the identities of null cell tumours: Epigenomics corroborate subtle histological cues in pituitary neuroendocrine tumour/adenoma classification	3
Comprehensive analysis of intratumoural heterogeneity of somatic copy number alterations in diffuse glioma reveals clonality‐dependent prognostic patterns	2
Identifying diagnostic and prognostic factors in cerebral amyloid angiopathy‐related inflammation: A systematic analysis of published and seven new cases	2
Successful plasmapheresis and immunoglobulin treatment for severe lipid storage myopathy: Doing the right thing for the wrong reason	2
Molecular characterisation of sporadic endolymphatic sac tumours and comparison to von Hippel–Lindau disease‐related tumours	2
Different patterns of hippocampal subfield pathology in Lewy body disease and Alzheimer's disease	2
Myostatin in idiopathic inflammatory myopathies: Serum assessment and disease activity	2
Progressive hemispheric atrophy in HIV: A Rasmussen's‐like variant of CD8 encephalitis?	2
Alzheimer's disease pathology concomitant with memory impairment in late‐onset multiple system atrophy	2
Effects of mutant huntingtin in oxytocin neurons on non‐motor features of Huntington's disease	2
Paths to hippocampal damage in neuromyelitis optica spectrum disorders	2
Target formation in muscle fibres indicates reinnervation – A proteomic study in muscle samples from peripheral neuropathies	2
A threshold for mitotic activity and post‐surgical residual volume defines distinct prognostic groups for astrocytoma IDH‐mutant	2
Motor neuron involvement threatens survival in spinocerebellar ataxia type 1	2
Simultaneous Nbs1 and p53 inactivation in neural progenitors triggers high‐grade gliomas	2
Neurotherapeutic implications of sense and respond strategies generated by astrocytes and astrocytic tumours to combat pH mechanical stress	2
Effects of mutant huntingtin inactivation on Huntington disease‐related behaviours in the BACHD mouse model	2
Neuromuscular junction denervation and terminal Schwann cell loss in the hTDP‐43 overexpression mouse model of amyotrophic lateral sclerosis	2
A gene dosage‐dependent effect unveils NBS1 as both a haploinsufficient tumour suppressor and an essential gene for SHH‐medulloblastoma	2
Oxygen treatment reduces neurological deficits and demyelination in two animal models of multiple sclerosis	2
Genetic inhibition of PDK1 robustly reduces plaque deposition and ameliorates gliosis in the 5×FAD mouse model of Alzheimer's disease	2
Tuberous sclerosis complex‐1 (TSC1) contributes to selective neuronal vulnerability in Alzheimer's disease	2