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Pathological background of subcortical hyperintensities on diffusion-weighted images in a case of neuronal intranuclear inclusion disease.
Clin Neuropathol. 2016 Nov/Dec; 35(6):375-380.CN

Abstract

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

Yokoi S
No affiliation info available
Yasui K
No affiliation info available
Hasegawa Y
No affiliation info available
Niwa K
No affiliation info available
Noguchi Y
No affiliation info available
Tsuzuki T
No affiliation info available
Mimuro M
No affiliation info available
Sone J
No affiliation info available
Watanabe H
No affiliation info available
Katsuno M
No affiliation info available
Yoshida M
No affiliation info available
Sobue G
No affiliation info available

MeSH

AgedBrain DiseasesDiffusion Magnetic Resonance ImagingFemaleHumansIntranuclear Inclusion BodiesNeurodegenerative Diseases

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

27719745

Citation

Yokoi, Satoshi, et al. "Pathological Background of Subcortical Hyperintensities On Diffusion-weighted Images in a Case of Neuronal Intranuclear Inclusion Disease." Clinical Neuropathology, vol. 35, no. 6, 2016, pp. 375-380.
Yokoi S, Yasui K, Hasegawa Y, et al. Pathological background of subcortical hyperintensities on diffusion-weighted images in a case of neuronal intranuclear inclusion disease. Clin Neuropathol. 2016;35(6):375-380.
Yokoi, S., Yasui, K., Hasegawa, Y., Niwa, K., Noguchi, Y., Tsuzuki, T., Mimuro, M., Sone, J., Watanabe, H., Katsuno, M., Yoshida, M., & Sobue, G. (2016). Pathological background of subcortical hyperintensities on diffusion-weighted images in a case of neuronal intranuclear inclusion disease. Clinical Neuropathology, 35(6), 375-380.
Yokoi S, et al. Pathological Background of Subcortical Hyperintensities On Diffusion-weighted Images in a Case of Neuronal Intranuclear Inclusion Disease. Clin Neuropathol. 2016 Nov/Dec;35(6):375-380. PubMed PMID: 27719745.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Pathological background of subcortical hyperintensities on diffusion-weighted images in a case of neuronal intranuclear inclusion disease. AU - Yokoi,Satoshi, AU - Yasui,Keizo, AU - Hasegawa,Yasuhiro, AU - Niwa,Kazuki, AU - Noguchi,Yoshinori, AU - Tsuzuki,Toyonori, AU - Mimuro,Maya, AU - Sone,Jun, AU - Watanabe,Hirohisa, AU - Katsuno,Masahisa, AU - Yoshida,Mari, AU - Sobue,Gen, PY - 2016/11/07/accepted PY - 2016/10/11/pubmed PY - 2017/2/9/medline PY - 2016/10/11/entrez SP - 375 EP - 380 JF - Clinical neuropathology JO - Clin Neuropathol VL - 35 IS - 6 N2 - 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. . SN - 0722-5091 UR - https://www.unboundmedicine.com/medline/citation/27719745/Pathological_background_of_subcortical_hyperintensities_on_diffusion_weighted_images_in_a_case_of_neuronal_intranuclear_inclusion_disease_ DB - PRIME DP - Unbound Medicine ER -