T2-hyperintensity in the internal globus pallidus in Machado-Joseph disease

A 69-year-old male patient with Machado-Joseph disease (MJD) presented with mild cerebellar ataxia, global areflexia, and nystagmus. MRI showed cerebellar and brainstem atrophy, as well as abnormal hyperintensity along the medial aspect of the globus pallidus intemus. This finding suggests degenerat...

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Published inArquivos de neuro-psiquiatria Vol. 81; no. 11; pp. 1018 - 1019
Main Authors Meira, Alex T., Araruna, Jorge E. D., Araujo, Lais Vieira, Leite, Raíssa N. L. F., Franklin, Gustavo L., Nepomuceno, Adriana M. T., Teive, Hélio A. G.
Format Journal Article
LanguageEnglish
Portuguese
Published Sao Paulo Arquivos de Neuro-Psiquiatria 01.11.2023
Thieme Revinter Publicações Ltda
Academia Brasileira de Neurologia (ABNEURO)
Subjects
Atrophy
Brain stem
Cerebellar ataxia
Cerebellum
Globus pallidus
Images in Neurology
Machado-Joseph disease
Neurodegeneration
Spinocerebellar ataxia
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Summary:A 69-year-old male patient with Machado-Joseph disease (MJD) presented with mild cerebellar ataxia, global areflexia, and nystagmus. MRI showed cerebellar and brainstem atrophy, as well as abnormal hyperintensity along the medial aspect of the globus pallidus intemus. This finding suggests degeneration of the lenticular fasciculus and may be associated with degeneration of other neural fibers. While not specific to MJD, similar findings have been observed in other types of spinocerebellar ataxias and in healthy elderly individuals.
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ISSN:0004-282X
1678-4227
DOI:10.1055/s-0043-1772605