We are analyzing https://link.springer.com/article/10.1007/s00401-005-1079-4.

Title:
Histopathological changes underlying frontotemporal lobar degeneration with clinicopathological correlation | Acta Neuropathologica
Description:
We have investigated the pathological correlates of dementia in the brains from a consecutive series of 70 patients dying with a clinical diagnosis of frontotemporal lobar degeneration (FTLD). Clinical misdiagnosis rate was low with only 3 patients (4%) failing to show pathological changes consistent with this diagnosis; 1 patient had Alzheimer’s disease and 2 had cerebrovascular disease (CVD). In the remaining 67 patients, the most common underlying histological cause was ubiquitin pathology with 24 (36%) cases so affected. In these, ubiquitin-positive inclusions were present in the cerebral cortex as small, rounded or crescent-shaped structures within the cytoplasm of neurones of layer II, together with coiled or curvilinear bodies within neurites, and in the hippocampus as small, solid and more spherical-shaped inclusion bodies within the cytoplasm of dentate gyrus granule cells. In one patient, “cat’s eye” or “lentiform” intranuclear ubiquitin inclusions were also present. The second most common histological type was dementia lacking distinctive histology (DLDH), in which neither tau nor ubiquitin inclusions were present, with 16 cases (24%) being affected. Pick-type histology was seen in 14 cases (21%) and tau histological changes associated with frontotemporal dementia (FTD) linked to chromosome 17 (FTDP-17) were present in 11 cases (16%). One case (1%) showed an unusual tau pathology that could not be allocated to any of the other tau groups. Only 1 case (1%) had neuronal intermediate filament inclusion dementia. No cases with ubiquitinated, valosin-containing protein-immunoreactive intranuclear inclusion bodies of the type seen in inclusion body myopathy with Paget’s disease of bone and frontotemporal dementia were seen. Clinicopathological correlation showed that any of these histological subtypes can be associated with FTD. However, for FTD with motor neurone disease (FTD+MND), semantic dementia or primary progressive aphasia (PA), the histological profile was either ubiquitin type or DLDH type; Pick-type histology was seen in only 1 case of PA. None of these latter three clinical subtypes was associated with a mutation in tau gene and FTDP-17 type of tau pathology. All cases of progressive apraxia were associated with Pick-type histology. Present data therefore indicate that, although ubiquitin pathology is the most common histological form associated with FTLD, this pathology is not tightly linked with, nor is pathologically diagnostic for, any particular clinical form of the disease, including FTD+MND.
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google, scholar, pubmed, article, dementia, frontotemporal, disease, inclusions, motor, neuropathol, degeneration, tau, lobar, ubiquitin, acta, inclusion, neurol, mann, neary, cases, neurone, lateral, van, snowden, clinical, pathology, type, amyotrophic, sclerosis, neuron, david, present, bodies, neuronal, genet, pickeringbrown, patients, histological, ubiquitinpositive, histology, chromosome, mutation, access, protein, neurosci, lett, neurobiol, familial, dma, manchester,

Topics {✒️}

month download article/chapter fronto-temporal lobar degeneration spherical-shaped inclusion bodies ubiquitin-immunoreactive intraneuronal inclusions neocortical ubiquitin-immunoreactive inclusions cortical ubiquitin-positive inclusions ubiquitin-positive intraneuronal inclusions pickering-brown sm ubiquitin-binding protein p62 frontotemporal lobar degeneration stuart pickering-brown full article pdf related subjects frontal lobe degeneration ubiquitin-related proteins amyotrophic lateral sclerosis privacy choices/manage cookies motor neurone degeneration ubiquitin inclusion bodies tau-negative inclusions neurofilament inclusion bodies inclusion body myopathy filamentous inclusion body ubiquitin-positive neuronal ubiquitin-positive inclusions ubiquitin positive inclusions frontal lobe dementia ubiquitin-binding proteins amyotroph lateral scler motor neurone disease motor neuron disease fronto-temporal dementia article shi pick-type histology motor neuron type crescent-shaped structures anterior horn cells rosso sm unusual tau pathology ubiquitin-immunoreactive neurites european economic area disparate microscopic patterns el escorial revisited cruz-sanchez ff pericak-vance ma broad clinicopathological spectrum significant fine mapping phosphotyrosine-independent ligand gomez-esteban jc rodriguez-martinez ab

Questions {❓}

Josephs KA, Holton JL, Rossor MN, Braendgaard H, Osawa T, Fox NC, Petersen RC, Pearl GS, Ganguly M, Rosa P, Laursen H, Parisi JE, Waldemar G, Quinn NP, Dickson DW, Revesz T (2003) Neurofilament inclusion body disease: a new proteinopathy?

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      name:Clinical Neuroscience Research Group, University of Manchester, Greater Manchester Neurosciences Centre, Hope Hospital, Salford, UK
      name:Department of Care of the Elderly, Dongzhimen Hospital, Beijing University of Chinese Medicine, Beijing, PR China
      name:Clinical Neuroscience Research Group, University of Manchester, Greater Manchester Neurosciences Centre, Hope Hospital, Salford, UK
      name:Clinical Neuroscience Research Group, University of Manchester, Greater Manchester Neurosciences Centre, Hope Hospital, Salford, UK
      name:Clinical Neuroscience Research Group, University of Manchester, Greater Manchester Neurosciences Centre, Hope Hospital, Salford, UK
      name:Clinical Neuroscience Research Group, University of Manchester, Greater Manchester Neurosciences Centre, Hope Hospital, Salford, UK
      name:Clinical Neuroscience Research Group, University of Manchester, Greater Manchester Neurosciences Centre, Hope Hospital, Salford, UK
      name:Clinical Neuroscience Research Group, University of Manchester, Greater Manchester Neurosciences Centre, Hope Hospital, Salford, UK
      name:Clinical Neuroscience Research Group, University of Manchester, Greater Manchester Neurosciences Centre, Hope Hospital, Salford, UK
      name:Clinical Neuroscience Research Group, University of Manchester, Greater Manchester Neurosciences Centre, Hope Hospital, Salford, UK
      name:Department of Medical Genetics, University of Manchester, St Mary’s Hospital, Manchester, UK
      name:Clinical Neuroscience Research Group, University of Manchester, Greater Manchester Neurosciences Centre, Hope Hospital, Salford, UK
      name:Department of Care of the Elderly, Dongzhimen Hospital, Beijing University of Chinese Medicine, Beijing, PR China
      name:Clinical Neuroscience Research Group, University of Manchester, Greater Manchester Neurosciences Centre, Hope Hospital, Salford, UK
      name:Division of Laboratory and Regenerative Medicine, University of Manchester, Manchester, UK
      name:Clinical Neuroscience Research Group, University of Manchester, Greater Manchester Neurosciences Centre, Hope Hospital, Salford, UK
      name:Clinical Neuroscience Research Group, University of Manchester, Greater Manchester Neurosciences Centre, Hope Hospital, Salford, UK
      name:Clinical Neuroscience Research Group, University of Manchester, Greater Manchester Neurosciences Centre, Hope Hospital, Salford, UK
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