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Teaching NeuroImage: Paravermal Lesions in

Neuronal Intranuclear Inclusion Disease
Atsuhiko Sugiyama, MD, PhD, Jun Sone, MD, PhD, and Satoshi Kuwabara, MD, PhD Correspondence
Dr. Sugiyama
®
Neurology 2022;99:484-485. doi:10.1212/WNL.0000000000200984 asugiyama@chiba-u.jp

Figure 1 Brain MRI Without Contrast

Brain MRI showing a bilateral high-intensity signal in the medial part of the cerebellar hemisphere by the vermis
(paravermal lesions, white arrows; A, B), cerebral white matter (C) on fluid-attenuated inversion recovery, a high-
intensity signal along the corticomedullary junction on diffusion-weighted imaging (D) without restrictions to the
apparent diffusion coefficient map (E).

A 77-year-old woman presented with a several-year history of progressive cerebellar ataxia and MORE ONLINE
cognitive impairment. MRI revealed paravermal lesions on fluid-attenuated inversion recovery
Teaching slides
and a high-intensity signal along the corticomedullary junction on diffusion-weighted imaging
links.lww.com/WNL/
(Figure 1). Abnormal expansion of GGC repeats in the NOTCH2NLC gene confirmed the
C181
neuronal intranuclear inclusion disease (NIID) diagnosis. NIID is a clinically heterogeneous
neurodegenerative disorder usually occurring at age 50 years or older in sporadic cases. Par-
avermal lesions are a characteristic MRI finding in NIID.1 Paravermal lesions are not specific to
NIID alone (Supplement, links.lww.com/WNL/C182) but precede other imaging findings and
can be the sole radiologic indication for NIID diagnosis.2

Study Funding
No targeted funding reported.

From the Department of Neurology (A.S., S.K.), Graduate School of Medicine, Chiba University; and Department of Neuropathology (J.S.), Institute for Medical Science of Aging, Aichi
Medical University, Japan.

Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

484 Copyright © 2022 American Academy of Neurology

Copyright © 2022 American Academy of Neurology. Unauthorized reproduction of this article is prohibited.
Disclosure
The authors report no relevant disclosures. Go to Neurology. Appendix (continued)

org/N for full disclosures. Name Location Contribution

Publication History Jun Sone, MD, Aichi Medical Genetic analysis; revised the
PhD University, manuscript for intellectual content
Received by Neurology January 31, 2022. Accepted in final form Japan
June 3, 2022. Submitted and externally peer reviewed. The handling
Satoshi Chiba Revised the manuscript for intellectual
editor was Roy Strowd III, MD, Med, MS.
Kuwabara, MD, University, content; supervised the study and gave
PhD Japan the final approval

Appendix Authors
References
Name Location Contribution 1. Sugiyama A, Sato N, Kimura Y, et al. MR imaging features of the cerebellum in adult-
onset neuronal intranuclear inclusion disease: 8 cases. AJNR Am J Neuroradiol. 2017;
Atsuhiko Chiba Concept and design; drafted the 38(11):2100-2104.
Sugiyama, MD, University, manuscript 2. Okamura S, Takahashi M, Abe K, et al. A case of neuronal intranuclear inclusion
PhD Japan disease with recurrent vomiting and without apparent DWI abnormality for the first
seven years. Heliyon. 2020;6(8):e04675.

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Teaching NeuroImage: Paravermal Lesions in Neuronal Intranuclear Inclusion Disease
Atsuhiko Sugiyama, Jun Sone and Satoshi Kuwabara
Neurology 2022;99;484-485 Published Online before print July 8, 2022
DOI 10.1212/WNL.0000000000200984

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