OOIR: Observatory of International Research

Papers

(The median citation count of Neuropathology and Applied Neurobiology is 2. The table below lists those papers that are above that threshold based on CrossRef citation counts [max. 250 papers]. The publications cover those that have been published in the past four years, i.e., from 2021-08-01 to 2025-08-01.)

Article	Citations
Paediatric astroblastoma‐like neuroepithelial tumour of the spinal cord with a MAMLD1‐BEND2 rearrangement	120
Intelligence quotient–genotype association in dystrophinopathies: A systematic review and meta‐analysis	60
Neuronal infection is a major pathogenetic mechanism and cause of fatalities in human acute Nipah virus encephalitis	59
Novel naturally occurring autoantibodies attenuate α‐synuclein pathology in a mouse model of Parkinson's disease	53
An autopsy case of late‐onset spinocerebellar atrophy type 14	49
Oral Abstracts	44
Editorial	31
Potent T cell‐mediated anti‐inflammatory role of the selective CB2 agonist lenabasum in multiple sclerosis	31
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	26
A novel SREBF1::NACC1 gene fusion in an unclassifiable intracranial tumour	26
Anaplastic ganglioglioma—A diagnosis comprising several distinct tumour types	25
Analysing cerebrospinal fluid with explainable deep learning: From diagnostics to insights	25
Author Index	24
Rapid bacterial identification from formalin‐fixed paraffin‐embedded neuropathology specimens using 16S rDNA nanopore sequencing	24
Neuronal and astrocytic tetraploidy is increased in drug‐resistant epilepsy	23
Erratum	22
The S1PR2‐CCL2‐BDNF‐TrkB pathway mediates neuroinflammation and motor incoordination in hyperammonaemia	22
Myostatin in idiopathic inflammatory myopathies: Serum assessment and disease activity	21
Response letter: Complexities in pericyte markers	21
Synaptic gene expression changes in frontotemporal dementia due to the MAPT 10 + 16 mutation	21
	20
Cover Image, Volume 50, Issue 3	20
Perineuronal nets are phagocytosed by MMP‐9 expressing microglia and astrocytes in the SOD1^G93A ALS mouse model	19
Issue Information	19

Non‐coding regulatory elements: Potential roles in disease and the case of epilepsy	18
Genetic inhibition of PDK1 robustly reduces plaque deposition and ameliorates gliosis in the 5×FAD mouse model of Alzheimer's disease	18
A standardised protocol for blood and cerebrospinal fluid collection and processing for biomarker research in ataxia	16
124th Meeting of the British Neuropathological Society The View, Royal College of Surgeons of England, Lincoln's Inn Fields, London	16
Impaired brain insulin signalling in Parkinson's disease	16
Fibrodysplasia ossificans progressiva: Histopathological implications of aberrant bone morphogenic protein signalling for CNS dysgenesis	15
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Evidence of neural crest cell origin of a DICER1 mutant CNS sarcoma in a child with DICER1 syndrome and NRAS‐mutant neurocutaneous melanosis	15
Concurrent tau pathologies in frontotemporal lobar degeneration with TDP‐43 pathology	15
m⁶A mRNA methylation in human brain is disrupted in Lewy body disorders	14
Robust methylation‐based classification of brain tumours using nanopore sequencing	14
The autophagy‐enhancing drug carbamazepine improves neuropathology and motor impairment in mouse models of Machado–Joseph disease	14
Application of Oral Mucosal Epithelial Cells in Noninvasive Pathological Diagnosis of Neuronal Intranuclear Inclusion Disease	14
Microglia induce an interferon‐stimulated gene expression profile in glioblastoma and increase glioblastoma resistance to temozolomide	14
TDP‐43 pathology and functional deficits in wild‐type and ALS/FTD mutant cyclin F mouse models	14
Epigenetic age acceleration is associated with oligodendrocyte proportions in MSA and control brain tissue	14
Image‐based deep learning reveals the responses of human motor neurons to stress and VCP‐related ALS	14
RNA sequencing of peripheral blood in amyotrophic lateral sclerosis reveals distinct molecular subtypes: Considerations for biomarker discovery	14
Low‐grade epilepsy‐associated neuroepithelial tumours with a prominent oligodendroglioma‐like component: The diagnostic challenges	14
Adult‐onset Alexander disease with unusual inflammatory features and a novel GFAP mutation in two patients	14
Phenotypic and epigenetic heterogeneity in FGFR2‐fused glial and glioneuronal tumours	14
Is islet amyloid polypeptide indeed expressed in the human brain?	13
Systematic review of human post‐mortem immunohistochemical studies and bioinformatics analyses unveil the complexity of astrocyte reaction in Alzheimer's disease	13
Neurodegenerative movement disorders: An epigenetics perspective and promise for the future	13
MiR‐142‐3p regulates synaptopathy‐driven disease progression in multiple sclerosis	13
	13
Hippocampal dentate granule cells in temporal lobe epilepsy: A morphometry and transcriptomic study	12
The genomic landscape of dysembryoplastic neuroepithelial tumours and a comprehensive analysis of recurrent cases	12
Axonal response of mitochondria to demyelination and complex IV activity within demyelinated axons in experimental models of multiple sclerosis	12
126^th Meeting of the British Neuropathological Society The View, Royal College of Surgeons of England, Lincoln's Inn Fields, London	12
The blood–CSF–brain route of neurological disease: The indirect pathway into the brain	12
WNT‐activated, MYC‐amplified medulloblastoma displaying intratumoural heterogeneity	12
Neurotherapeutic implications of sense and respond strategies generated by astrocytes and astrocytic tumours to combat pH mechanical stress	12
Comprehensive analysis of intratumoural heterogeneity of somatic copy number alterations in diffuse glioma reveals clonality‐dependent prognostic patterns	12
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Fibroblast growth factor 9 (FGF9)‐mediated neurodegeneration: Implications for progressive multiple sclerosis?	11
	11
AKT1^E17K‐mutated meningioma cell lines respond to treatment with the AKT inhibitor AZD5363	11
Issue Information	11
Intramuscular Nerve Bundles Reflect TDP‐43 Pathology in the Medulla and Spinal Cord of ALS Patients	11
Nanopore sequencing identifies Borrelia miyamotoi as an unexpected cause of meningitis after B cell depletion	11
Issue Information	11
Pathological Characterisation of Posterior Cortical Atrophy in Comparison With Amnestic Alzheimer's Disease	11
Issue Information	11
Interferon‐gamma contributes to disease progression in the Ndufs4(−/−) model of Leigh syndrome	11
Muscle biopsy practices in the evaluation of neuromuscular disease: A systematic literature review	11
	10
Corrigendum	10
Corrigendum	9
Low‐grade diffusely infiltrative tumour (LGDIT), SMARCB1‐mutant: A clinical and histopathological distinct entity showing epigenetic similarity with ATRT‐MYC	9

HnRNP K mislocalisation in neurons of the dentate nucleus is a novel neuropathological feature of neurodegenerative disease and ageing	9
Amino‐terminally elongated Aβ peptides are generated by the secreted metalloprotease ADAMTS4 and deposit in a subset of Alzheimer's disease brains	9
Quantitative cellular changes in multiple system atrophy brains	9
Unfolded protein response markers Grp78 and eIF2alpha are upregulated with increasing alpha‐synuclein levels in Lewy body disease	8
Adult brain tumour research in 2024: Status, challenges and recommendations	8
Regulation of CNS pathology by Serpina3n/SERPINA3: The knowns and the puzzles	8
Atypical teratoid/rhabdoid tumour‐TYR subtype arising in the setting of germline ring chromosome 22: An uncommon form of tumour predisposition	8
Regional redistribution of CB1 cannabinoid receptors in human foetal brains with Down's syndrome and their functional modifications in Ts65Dn^+/+ mice	8
GFAP expression in the brain during human postnatal development	8
Cytoplasmic HDAC4 recovers synaptic function in the 3×Tg mouse model of Alzheimer's disease	8
The SPTLC1 p.S331 mutation bridges sensory neuropathy and motor neuron disease and has implications for treatment	8
Proteomic profiling of polyglucosan bodies associated with glycogenin‐1 deficiency in skeletal muscle	8
Ageing‐related tau astrogliopathy severely affecting the substantia nigra	7
	7
Issue Information	7
CD8 T‐cell‐mediated cerebellitis directed against Purkinje cell antigen after ipilimumab for small cell lung cancer	7
Letter to the editor	7
A novel homozygous nonsense variant in COL12A1 causes myopathic Ehlers‐Danlos syndrome: A case report and literature review	7
Issue Information	7
Diagnostic accuracy of 1p/19q codeletion tests in oligodendroglioma: A comprehensive meta‐analysis based on a Cochrane systematic review	7
Cerebellar phenotypes in germline PTEN mutation carriers	7
Expression pattern of perilipins in human brain during aging and in Alzheimer's disease	7
Ectopic expression of neuronal adenosine kinase, a biomarker in mesial temporal lobe epilepsy without hippocampal sclerosis	6
Phosphorylation of MAP 1A regulates hyperphosphorylation of Tau in Alzheimer's disease model	6
Multiplex Immunofluorescent Analysis of Alpha‐Synuclein in Nigral Lewy Bodies With Heat‐Induced Antibody Stripping Reveals an Intricate Multilayered Structure	6
Dysfunction of the blood–brain barrier in Alzheimer's disease: Evidence from human studies	6
Artificial intelligence in histopathological image analysis of central nervous system tumours: A systematic review	6
PLAG‐Family Amplified CNS Embryonal Tumour With PLAG1 Immunohistochemical Expression: Expanding the Spectrum of Diagnostic Tools	6
Cover Image, Volume 50, Issue 1	6
Soluble amyloid‐β dimers are resistant to amyloid‐β prion conversion in vivo suggesting antiprion properties	6
LMNB1‐duplication mediated nuclear architecture alteration and demyelination of cerebral white matter in a patient with ADLD	6
Metastatic Bifocal Germinoma With Dramatic Early Steroid Response, Utility of Circulating miR‐371a‐3p and Vinblastine Monotherapy Prior to Definitive Craniospinal Irradiation	6
125th Meeting of the British Neuropathological Society The View, Royal College of Surgeons of England, Lincoln's Inn Fields, London	6
Psychotic symptoms in frontotemporal dementia with TDP‐43 tend to be associated with type B pathology	6
Methyl donor supplementation reduces phospho‐Tau, Fyn and demethylated protein phosphatase 2A levels and mitigates learning and motor deficits in a mouse model of tauopathy	6
Mechanisms of COVID‐19‐associated olfactory dysfunction	6
Identification of a novel heterozygous DYSF variant in a large family with a dominantly‐inherited dysferlinopathy	6
Issue Information	6
TAF15 and Transportin 1 in Intranuclear Inclusions of Neuronal Intranuclear Inclusion Disease	5
Computational Analysis of SOD1‐G93A Mouse Muscle Biomarkers for Comprehensive Assessment of ALS Progression	5
Cortical morphometric vulnerability to generalised epilepsy reflects chromosome‐ and cell type‐specific transcriptomic signatures	5
	5
Loss of IDH mutation or secondary tumour manifestation? Evolution of an IDH‐mutant and 1p/19q‐codeleted oligodendroglioma after 15 years of continuous temozolomide treatment and radiotherapy: A case r	5
Refining Muscle Morphometry Through Machine Learning and Spatial Analysis	5
Analysis of a pituitary tumour with histological features of central neurocytoma points towards the emergence of a new entity recognisable by a specific epigenetic signature	5
Emerging roles for the YAP/TAZ transcriptional regulators in brain tumour pathology and targeting options	5
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Neuroinvasion via Peripheral Nerves in Epidemic Viral Encephalitis Caused by Enterovirus, Orthoflavivirus and SARS‐Coronavirus	4
Issue Information	4
Classification of diseases with accumulation of Tau protein	4
Long‐standing multiple system atrophy‐Parkinsonism with limbic and FTLD‐type α‐synuclein pathology	4
The molecular mechanisms that underlie IGHMBP2‐related diseases	4
Pathological substrate of memory impairment in multiple system atrophy	4
Distinguishing post‐translational modifications in dominantly inherited frontotemporal dementias: FTLD‐TDP Type A (GRN) vs Type B (C9orf72)	4
Simultaneous Nbs1 and p53 inactivation in neural progenitors triggers high‐grade gliomas	4
Muscleblind‐like 2 knockout shifts adducin 1 isoform expression and alters dendritic spine dynamics of cortical neurons during brain development	4
	3
Early loss of locus coeruleus innervation promotes cognitive and neuropathological changes before amyloid plaque deposition in a transgenic rat model of Alzheimer's disease	3
	3
Oxygen treatment reduces neurological deficits and demyelination in two animal models of multiple sclerosis	3
Unveiling the identities of null cell tumours: Epigenomics corroborate subtle histological cues in pituitary neuroendocrine tumour/adenoma classification	3
Change in the molecular properties of CH1641 prions after transmission to wild‐type mice: Evidence for a single strain	3
A systems‐level analysis highlights microglial activation as a modifying factor in common epilepsies	3
Protein Coaggregation in Caribbean Atypical Parkinsonism: The Contribution of Annonacin	3
Skeletal muscle involvement in systemic amyloidosis is often overlooked	3
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Cover Image, Volume 48, Issue 5	3
Paths to hippocampal damage in neuromyelitis optica spectrum disorders	3
Issue Information	3
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Combinatorial model of amyloid β and tau reveals synergy between amyloid deposits and tangle formation	3
An Intracerebrally‐Infected Mouse Model of Enterovirus A71 Demonstrates Restricted Inter‐Neuronal Spread Within the Brain Parenchyma Despite Strong SCARB2 Expression	3
Splicing factor proline and glutamine rich intron retention, reduced expression and aggregate formation are pathological features of amyotrophic lateral sclerosis	3
Respiratory chain dysfunction in perifascicular muscle fibres in patients with dermatomyositis is associated with mitochondrial DNA depletion	3
	3
Testing Meningiomas With Methylation Arrays: Insights and Recommendations From a Large Single‐Centre Study	3
Diffuse infiltrating tumour with the molecular profile of an atypical teratoid rhabdoid tumour (AT/RT SHH‐1B) in an adult patient	2
Genotype–Phenotype Correlation in Progressive External Ophthalmoplegia: Insights From a Retrospective Analysis	2
Tuberous sclerosis complex‐1 (TSC1) contributes to selective neuronal vulnerability in Alzheimer's disease	2

Identical Seeding Characteristics and Cryo‐EM Filament Structures in FTLD‐Synuclein and Typical Multiple System Atrophy	2
Progressive hemispheric atrophy in HIV: A Rasmussen's‐like variant of CD8 encephalitis?	2
Olfactory bulb SARS‐CoV‐2 infection is not paralleled by the presence of virus in other central nervous system areas	2
Investigating key factors underlying neurodegeneration linked to alpha‐synuclein spread	2
Chronic traumatic encephalopathy neuropathologic change in former Australian rugby players	2
Slow disease progression and characteristic TDP‐43 inclusions in a patient with familial amyotrophic lateral sclerosis carrying a TARDBP G357S variant	2
Expression, prognostic significance and therapeutic implications of PD‐L1 in gliomas	2
Post‐mortem 7T MR imaging and neuropathology in middle stage juvenile‐onset Huntington disease: A case report	2
Three‐Dimensional Visualisation of Blood Vessels in Human Gliomas Using Tissue Clearing and Deep Learning	2
Clinicopathological Characteristics and Immune Microenvironment of Posterior Pituitary Tumours	2
A high‐throughput single‐cell RNA expression profiling method identifies human pericyte markers	2
Cu^II(atsm) significantly decreases microglial reactivity in patients with sporadic amyotrophic lateral sclerosis	2
A reassessment of spinal cord pathology in severe infantile spinal muscular atrophy	2
Pathogenic variants of Valosin‐containing protein induce lysosomal damage and transcriptional activation of autophagy regulators in neuronal cells	2
	2
Recurrent posterior fossa group A (PFA) ependymoma in a young child with constitutional mismatch repair deficiency (CMMRD)	2
TAPPing into the potential of inducible tau/APP transgenic mice	2
The contribution of brain banks to knowledge discovery in amyotrophic lateral sclerosis: A systematic review	2
Elevated expression of urokinase plasminogen activator in rodent models and patients with cerebral amyloid angiopathy	2
Nanoscale reorganisation of synaptic proteins in Alzheimer's disease	2