title: Pathological background of subcortical hyperintensities on diffusion-weighted images in a case of neuronal intranuclear inclusion disease.
abstract: OBJECTIVE
  Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disorder with eosinophilic intranuclear inclusion bodies. The variable symptoms of NIID increase the difficulty in an antemortem diagnosis. NIID shows leukoencephalopathy on brain magnetic resonance imaging MRI, but the significance of the radiological findings have not been clarified.
  
  
  METHODS
  We examined an autopsied case of NIID with subcortical linear hyperintensities on diffusion weighted imaging (DWI) and leukoencephalopathy on fluid attenuation inversion recovery (FLAIR) imaging. Semiquantitative analysis was performed by merging coronal sections of DWI and identical hematoxylin-eosin (H & E) stained brain specimens. The severity of spongiotic changes, the common pathological findings of NIID, were quantified and compared with MRI lesions classified by DWI signals.
  
  
  RESULTS
  The white matter showed diffuse myelin pallor, and multiple focal spongiotic changes were present in the subcortical white matter proximal to the U-fibers. Spongiotic changes were restricted in the lesions with subcortical linear DWI high signals.
  
  
  CONCLUSIONS
  Subcortical DWI high signals in NIID strongly correlate with pathological spongiotic changes of NIID. Subcortical spongiotic changes may be a characteristic finding of NIID. .
authors.forename: Satoshi
authors.initials: S
authors.lastname: Yokoi
authors.forename: Keizo
authors.initials: K
authors.lastname: Yasui
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Hasegawa
authors.forename: Kazuki
authors.initials: K
authors.lastname: Niwa
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Noguchi
authors.forename: Toyonori
authors.initials: T
authors.lastname: Tsuzuki
authors.forename: Maya
authors.initials: M
authors.lastname: Mimuro
authors.forename: Jun
authors.initials: J
authors.lastname: Sone
authors.forename: Hirohisa
authors.initials: H
authors.lastname: Watanabe
authors.forename: Masahisa
authors.initials: M
authors.lastname: Katsuno
authors.forename: Mari
authors.initials: M
authors.lastname: Yoshida
authors.forename: Gen
authors.initials: G
authors.lastname: Sobue
mesh_terms: D000368:Aged; D001927:Brain Diseases / Q000473:pathology*; D038524:Diffusion Magnetic Resonance Imaging; D005260:Female; D006801:Humans; D045586:Intranuclear Inclusion Bodies / Q000473:pathology; D019636:Neurodegenerative Diseases / Q000473:pathology*