Isolated paravermal T2 high-intensity lesions in dentatorubral–pallidoluysian atrophy

•DRPLA is caused by the expansion of CAG repeats in atrophin 1.•Paravermal lesions are a characteristic MRI finding in late adult-onset DRPLA.•MRI shows cerebellar atrophy after many years along with paravermal lesions.•Paravermal lesions may be the only abnormality in MRI for early DRPLA diagnosis....

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Bibliographic Details
Published in:Journal of the neurological sciences 2023-08, Vol.451, p.120717-120717, Article 120717
Main Authors: Nakagawa, Yuki, Sugiyama, Atsuhiko, Hirano, Shigeki, Ishige, Takayuki, Kuwabara, Satoshi
Format: Article
Language:English
Subjects:
Atrophy - pathology
Brain - pathology
Dentatorubral–pallidoluysian atrophy
Globus Pallidus
Humans
Magnetic resonance imaging
Myoclonic Epilepsies, Progressive - diagnostic imaging
Paravermal lesions
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Summary:•DRPLA is caused by the expansion of CAG repeats in atrophin 1.•Paravermal lesions are a characteristic MRI finding in late adult-onset DRPLA.•MRI shows cerebellar atrophy after many years along with paravermal lesions.•Paravermal lesions may be the only abnormality in MRI for early DRPLA diagnosis.
ISSN:0022-510X
1878-5883
DOI:10.1016/j.jns.2023.120717