AIMS: 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. CONCLUSION: Subcortical DWI high signals in NIID strongly correlate with pathological spongiotic changes of NIID. Subcortical spongiotic changes may be a characteristic finding of NIID. .
AIMS: 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. CONCLUSION: 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: Rhoia Neidenbach; Koichiro Niwa; Oeztekin Oto; Erwin Oechslin; Jamil Aboulhosn; David Celermajer; Joerg Schelling; Lars Pieper; Linda Sanftenberg; Renate Oberhoffer; Fokko de Haan; Michael Weyand; Stephan Achenbach; Christian Schlensak; Dirk Lossnitzer; Nicole Nagdyman; Yskert von Kodolitsch; Hans-Carlo Kallfelz; David Pittrow; Ulrike M M Bauer; Peter Ewert; Thomas Meinertz; Harald Kaemmerer Journal: Cardiovasc Diagn Ther Date: 2018-12
Authors: Suradech Suthiphosuwan; Sanskriti Sasikumar; David G Munoz; David K Chan; Walter J Montanera; Aditya Bharatha Journal: Neurol Clin Pract Date: 2019-12