Abstract Basophilic inclusion body disease and neuronal intermediate filament inclusion disease (NIFID) are rare diseases included among frontotemporal lobar degenerations with FUS-positive inclusions (FTLD-FUS).We report clinical and pathologic features of 2 new patients and reevaluate neuropathologic characteristics of 2 previouslydescribed cases,including an early-onset case ofA Case of Pancreatic Undifferentiated Carcinoma Mimicking IntroductionCase ReportDiscussionReferencesUndifferentiated/anaplastic carcinoma (UAC) of the pancreas is a rare neoplasm that accounts for 2-7% of pancreatic neoplasms .The tumor histologically shows round giant cells,pleomorphic cells,spindle cells,or even rhabdoid cells  and commonly contains small foci of atypical glandular elements; this glandular component is identical to that of ductal adenocarcinoma (DAC),thus indicating pancreatic UAC to be a subtype of ordinary DAC of the pancreas.If pancreatic UAC lacks the associated DAC componSee more on pancreas.imedpubPhenotypic Variability Within the Inclusion Body Spectrum IntroductionMaterials and MethodsResultsDiscussionAcknowledgementRecent consensus recommendations concerning frontotemporal lobar degeneration (FTLD) have grouped 3 disorders among the FTLD-FUS subgroup,all characterized by frontotemporal and variable subcortical neuronal degeneration,gliosis,superficial spongiosis,and the presence of FUS protein aggregates on histopathologic examination (1).These diseases comprise basophilic inclusion body disease (BIBD),neuronal intermediate filament inclusion disease (NIFID),and atypical FTLD with ubiSee more on academic.oupTopography of FUS pathology distinguishes late-onset BIBD May 09,2013·Armstrong RA,Gearing M,Bigio EH,Cruz-Sanchez FF,Duyckaerts C,Mackenzie IR,Perry RH,Skullerud K,Yokoo H,Cairns NJ The spectrum and severity of FUS-immunoreactive inclusions in the frontal and temporal lobes of ten cases of neuronal intermediate filament inclusion disease.Acta Neuropathol 2011,121 219228.10.1007/s00401-010-0753-3An autopsied case of sporadic adult-onset amyotrophic An autopsied case of sporadic adult-onset amyotrophic lateral sclerosis with FUS-positive basophilic inclusions Takeshi Matsuoka,Naoki Fujii,Akira Kondo,Akiko Iwaki,Toshihiro Hokonohara,Hiroyuki Honda ,Kensuke Sasaki,Satoshi O.Suzuki,Toru Iwaki
Download Citation An autopsy case of neuronal intermediate filament inclusion disease (NIFID) Background / Purpose Neuronal intermediate filament inclusion disease (NIFID) is a rare An autopsy case report of neuronal intermediate filament Jul 26,2021·We report an autopsy case of neuronal intermediate filament inclusion disease (NIFID),in which pyramidal motor dysfunction preceded cognitive disturbance for 3 years from the onset.Author Kimiko Inoue,Harutoshi Fujimura,Kayo Ueda,Tsuyoshi Matsumura,Kyoko Itoh,Saburo SakodaPublish Year 2015Images of An Autopsy Case of neuronal intermediate filament imagesAn autopsy case report of neuronal intermediate filament Jul 26,2021·We describe an autopsy case of neuronal intermediate filament inclusion disease (NIFID),a subtype of frontotemporal lobar degeneration (FTLD) with the appearance of fused-in-sarcoma (FUS) inclusions
Dec 13,2007·Recently,a new disease entity of frontotemporal lobar degeneration (FTLD) called neuronal intermediate filament inclusion disease (NIFID),neurofilament inclusion body disease (NIBD),or neurofilament inclusion disease (NFID) was proposed [5,6,16].A pathological hallmark of NIFID is the occurrence of neurofilament-positive intraneuronal inclusions.Cells Free Full-Text Functional Characterization of Neurofilaments (NFs),a major cytoskeletal component of motor neurons,play a key role in the differentiation,establishment and maintenance of their morphology and mechanical strength.The de novo assembly of these neuronal intermediate filaments requires the presence of the neurofilament light subunit (NEFL),whose expression is reduced in motor neurons in amyotrophic lateral sclerosisCited by 23Publish Year 2011Author Takeshi Matsuoka,Naoki Fujii,Akira Kondo,Akiko Iwaki,Toshihiro Hokonohara,Hiroyuki Honda,KensuAlveolar rhabdomyosarcoma.Demonstration of the muscle However,immunohistochemically,the results of every case were positive for desmin-the muscle type of the intermediate filament protein.The results suggest that demonstration of desmin may be a helpful adjunct tool in the diagnosis of poorly differentiated alveolar rhabdomyosarcomas.
An autopsy case of neuronal intermediate filament inclusion disease with regard to immunophenotypic and topographical analysis of the neuronal inclusions.Inoue K,Fujimura H,Ueda K,Matsumura T,Itoh K,Sakoda S Neuropathology 2015 Dec;35(6):545-52.Distinct pathological subtypes of FTLD-FUS SpringerLinkOct 30,2010·Neuronal intermediate filament inclusion disease (NIFID) is an uncommon neurological disorder with pathology characterized by NCI that are immunoreactive for all of the class IV neuronal intermediate filaments (IF),which include light,medium and heavy weight neurofilament (NF) subunits and -internexin [7,9].Dysfunctions of neuronal and glial intermediate filaments Jul 01,2009·Intermediate filaments (IFs) are abundant structures found in most eukaryotic cells,including those in the nervous system.In the CNS,the primary components of neuronal IFs are -internexin and the neurofilament triplet proteins.In the peripheral nervous system,a fifth neuronal IF protein known as peripherin is also present.
The main intermediate filament in astrocytes is glial fibrillary acidic protein (GFAP),57 but immature astrocytes express both nestin and vimentin.58 Interestingly,mice devoid of nestin,vimentin,or GFAP develop normally and do not show signs of CNS aberrations.5,57,59 The changes in IF expression during neuronal development coincides Making the Diagnosis of Frontotemporal LobarMar 01,2013·In the last 10 to 15 years,awareness of the frontotemporal dementia (FTD) has increased.In fact,in demented individuals younger than age 65,FTD and AD have the same prevalence.10 Frontotemporal dementia can be separated into 2 broad categories behavioral variant FTD and primary progressive aphasia.11,12 Further consideration of the clinical details of FTD is beyond the scope ofMiR-105 and miR-9 regulate the mRNA stability of neuronal Mar 01,2019·Intermediate filament aggregation within motor neurons is a hallmark of ALS pathogenesis.Changes to intermediate filament stoichiometry due to altered mRNA steady-state levels of NEFL,PRPH and INA is thought to drive protein aggregation,yet the exact cause of these changes is unknown.MicroRNAs (miRNAs)master regulators of gene expressionare largely dysregulated within ALS motor neurons
Jul 01,2001·We describe a case of desmoplastic infantile ganglioglioma (DIG) arising in the ventral diencephalon of a 3-1/2-month-old boy.On biopsy,the tumorNeurofilaments Organization and Function in NeuronsNeurofilaments in Neuronal Function Introduction to Neurofilaments A neuron is composed of the neuronal cell body (perikaryon) and several processes (dendrites and axon),of which the axon is usually the longest one.The axon is comparable to an electrical wire that the neuronal cell body extends to other neurons or mus-cles to transmit its signal.RESEARCH Open Access Topography of FUS pathology sinophilic and are immunoreactive for class IV neuronal intermediate filaments and FUS [1,2,5,9,11,16,19].Recent clinicopathologic series of various FTLD-FUS cases reported distinct pathologic features of aFTLD-U,BIBD and NIFID thereby supporting the notion that these three diseases represent related but distinct pathologic en-tities [5,9,11].
Nov 13,2020·When tissue damage and neuronal loss occur in the central Astrocytic intermediate filaments S.et al.An autopsy case of autosomal-recessive juvenile parkinsonism with aRelated searches for an autopsy case of neuronal intermediintermediate filaments structure and functionintermediate filaments definitionintermediate filaments are found in what structureintermediate filaments quizletintermediate filaments structureSome results are removed in response to a notice of local law requirement.For more information,please see here.12345NextStructural study of Purkinje cell axonal torpedoes in Nov 24,2008·Thus,defects in transport of neurofilaments clearly lead to abnormal accumulations of axonal intermediate filaments and neuronal degenerative changes [14,29].The pathological accumulation of phosphorylated neurofilament epitopes in occasional Purkinje cell bodies and dendrites in ET cases further suggests that impaired axoplasmic transport
Jul 29,2009·162 Fliegner,K.H.et al.(1994) Expression of the gene for the neuronal intermediate filament protein alpha-internexin coincides with the onset of neuronal differentiation in the developing rat nervous system.Journal of Comparative Neurology 342,161-173The role of neurofilament aggregation in neurodegeneration May 16,2019·Many neurodegenerative disorders,including Parkinsons,Alzheimers,and amyotrophic lateral sclerosis,are well known to involve the accumulation of disease-specific proteins.Less well known are the accumulations of another set of proteins,neuronal intermediate filaments (NFs),which have been observed in these diseases for decades.NFs belong to the family of cytoskeletal intermediate autopsyautopsy(PDF) A neurotoxic peripherin splice variant in a mouse This suggested that the epitope recognized by anti genic mice,shows that the disease mechanism of SOD1G37R Per 61 in mouse may exist as an equivalent peripherin iso- includes expression of a neurotoxic splice variant of periph- form in human.Using this antibody,we labeled pathologi- erin.
Jan 01,2006·The intermediate filaments nestin,alpha-internexin,and peripherin,together with the pan-neuronal marker PGP9.5,could be detected by immunohistochemistry in all samples of gut from the 14th WOG and older fetuses,whereas only PGP9.5 also showed a strong staining in the earliest stages investigated (9th WOG).autopsyautopsyFrontotemporal and motor neurone degeneration with May 27,2003·Kimiko Inoue,Harutoshi Fujimura,Kayo Ueda,Tsuyoshi Matsumura,Kyoko Itoh and Saburo Sakoda,An autopsy case of neuronal intermediate filament inclusion disease with regard to immunophenotypic and topographical analysis of the neuronal inclusions,Neuropathology,35,6,(545-552),(2015).autopsyautopsyNeuronal intermediate filaments and ALS A new look at an Nov 01,2006·One of the pathological hallmarks of ALS is the presence of axonal spheroids and perikaryal accumulations/aggregations comprised of the neuronal intermediate filament proteins,neurofilaments and peripherin.
OBJECTIVES To describe CSF-defined neuronal intermediate filament (NIF) autoimmunity.METHODS NIF-IgG CSF-positive patients (41,0.03% of 118599 tested,1996-2019) were included (serum was neither sensitive nor specific).Criteria-based patient NIF-IgG staining of brain and myenteric NIFs was detected by indirect immunofluorescence assay (IFA); NIF-specificity was confirmed by cellpubmed.ncbi.nlm.nih.govAn autopsy case report of neuronal intermediate filament We describe an autopsy case of neuronal intermediate filament inclusion disease (NIFID),a subtype of frontotemporal lobar degeneration (FTLD) with the appearance of fused-in-sarcoma (FUS) inclusions (FTLD-FUS).A 57-year-old man developed dysarthria and dysphagia.One year and five months later,heAn autopsy case of neuronal intermediate filament We report an autopsy case of neuronal intermediate filament inclusion disease (NIFID),in which pyramidal motor dysfunction preceded cognitive disturbance for 3 years from the onset.A 41-year-old Japanese man presented progressive spastic tetraparesis followed by cognitive impairment.