Basophilic inclusions and neuronal intermediate filament inclusions in amyotrophic lateral sclerosis and frontotemporal lobar degeneration

  title={Basophilic inclusions and neuronal intermediate filament inclusions in amyotrophic lateral sclerosis and frontotemporal lobar degeneration},
  author={Hidefumi Ito},
Basophilic inclusions (BIs) and neuronal intermediate filament inclusions (NIFIs) are key structures of basophilic inclusion body disease and neuronal intermediate filament inclusion disease (NIFID), respectively. BIs are sharply‐defined, oval or crescent neuronal intracytoplasmic inclusions that appear pale blue‐gray in color with HE staining and purple in color with Nissl but are stained poorly with silver impregnation techniques. Immunohistochemically BIs are negative for tau, trans… 
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An autopsy case report of neuronal intermediate filament inclusion disease presenting with predominantly upper motor neuron features
An autopsy case of neuronal intermediate filament inclusion disease (NIFID), a subtype of frontotemporal lobar degeneration with the appearance of fused‐in‐sarcoma (FUS) inclusions (FTLD‐FUS), supports the notion of the difference between amyotrophic lateral sclerosis and NIFID.
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Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease
Findings suggest that FUS may play an important role in the pathogenesis of NIFID, and double-label immunofluorescence confirmed that many cells had only FUS- positive inclusions and that all cells with IF-positive inclusions also contained pathological FUS.
FUS pathology in basophilic inclusion body disease
The consistent involvement of motorneurons in BIBD indicates that the association of FTLD and MND/ALS can occur on a FUS or TDP-43 pathological substrate, and together comprise a new biochemical category of neurodegenerative disease (FUS proteinopathies).
FUS Immunogold Labeling TEM Analysis of the Neuronal Cytoplasmic Inclusions of Neuronal Intermediate Filament Inclusion Disease: A Frontotemporal Lobar Degeneration with FUS Proteinopathy
It is shown that FUS becomes relatively insoluble in NIFID and there is no apparent posttranslational modifications, there are no pathogenic abnormalities in the FUS gene in NifID, and immunoelectron microscopy demonstrates the fine structural localization of FUS in N IFID which has not previously been described.
Phenotypic Variability Within the Inclusion Body Spectrum of Basophilic Inclusion Body Disease and Neuronal Intermediate Filament Inclusion Disease in Frontotemporal Lobar Degenerations With FUS-Positive Inclusions
Findings indicate that phenotypic variability in NIFID, including clinical manifestations and particular neuropathologic findings, may be related to the age at onset and individual differences in the evolution of lesions.
Basophilic inclusion body disease and neuronal intermediate filament inclusion disease: a comparative clinicopathological study
The clinical features and distribution of neuronal loss are similar in BIBD and NIFIDs, and an unknown protein besides α-internexin and neurofilament may play a pivotal pathogenetic role in at least some NIFID cases.
Screening for neurofilament inclusion disease using α-internexin immunohistochemistry
The frequency of NFID is investigated using α-internexin immunohistochemistry in 92 cases that had been diagnosed neuropathologically as FTLD, ALS, PLS, or MSA but in which routine screening with immunohISTochemistry for NF had not been previously done.
Juvenile ALS with basophilic inclusions is a FUS proteinopathy with FUS mutations
Juvenile ALS with basophilic inclusions is a FUS proteinopathy and should be classified as ALS-FUS, and a novel deletion mutation and two mutations associated with this distinct phenotype are identified.
Frontotemporal and motor neurone degeneration with neurofilament inclusion bodies: additional evidence for overlap between FTD and ALS
The case of a patient who had clinical frontal lobe dementia without apparent motor neurone disease (MND), with pathologic findings not typical of any single currently classified frontotemporal degeneration (FTD), may have additional implications for a link between FTD and ALS.