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CC BY 4.0 · Arq Neuropsiquiatr 2023; 81(11): 1018-1019
DOI: 10.1055/s-0043-1772605
Images in Neurology

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

Hiperintensidade em T2 em globo pálido interno na doença de Machado-Joseph
Alex T. Meira 
1   Universidade Federal da Paraíba, Departamento de Medicina Interna, Serviço de Neurologia, João Pessoa PB, Brazil.
,
Jorge E. D. Araruna 
1   Universidade Federal da Paraíba, Departamento de Medicina Interna, Serviço de Neurologia, João Pessoa PB, Brazil.
,
Lais Vieira Araujo 
1   Universidade Federal da Paraíba, Departamento de Medicina Interna, Serviço de Neurologia, João Pessoa PB, Brazil.
,
Raíssa N. L. F. Leite 
2   Nova Diagnóstico por Imagem, João Pessoa PB, Brazil.
,
Gustavo L. Franklin 
3   Pontifícia Universidade Católica, Departamento de Medicina Interna, Serviço de Neurologia, Curitiba PR, Brazil.
,
Adriana M. T. Nepomuceno 
4   Hospital Universitário Lauro Wanderley, João Pessoa PB, Brazil.
,
Hélio A. G. Teive 
5   Universidade Federal do Paraná, Hospital de Clínicas, Departamento de Medicina Interna, Serviço de Neurologia, Curitiba PR, Brazil.
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A 69-year-old male patient with Machado-Joseph disease (MJD) presented with a mild cerebellar ataxia, global areflexia, and nystagmus. Magnetic resonance imaging showed cerebellar atrophy; brainstem atrophy, mainly pontine, and a linear abnormal bilateral hyperintense along the medial aspect of the globus pallidus internus on T2-weighted sequence and fluid-attenuated inversion recovery (FLAIR) ([Figure 1]). This radiographic finding implies degeneration of the lenticular fasciculus.[1] The hyperintensity may be associated with degeneration of the subthalamic fascicles or the nigrostriatal dopaminergic fibers.[1] This finding is not pathognomonic of MJD, although it has been described in subjects with other types of spinocerebellar ataxias and in healthy elderly people.[2]

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Figure 1 T2 and FLAIR hyperintensity in a patient with Machado-Joseph disease (SCA3). Cerebellar atrophy is shown in sagittal T1 (A), and axial T2 (B and C). Hyperintensity in the medial aspect of the internal globus pallidus is shown in the FLAIR (D) and T2 (E), but in T1 (F) there is no sign of abnormality.

Authors' Contributions

ATM: conceptualization, investigation, methodology, project administration, resources, supervision, writing – original draft, writing – review & editing; JEDA: conceptualization, writing – original draft, writing – review & editing; LVA: conceptualization, writing – original draft, writing – review & editing; RNLFL, GLF, AMTN: conceptualization, writing – review & editing; HAGT: conceptualization, project administration, supervision, writing – review & editing.




Publication History

Received: 16 May 2023

Accepted: 02 July 2023

Article published online:
13 October 2023

© 2023. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)

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  • References

  • 1 Yamada S, Nishimiya J, Nakajima T, Taketazu F. Linear high intensity area along the medial margin of the internal segment of the globus pallidus in Machado-Joseph disease patients. J Neurol Neurosurg Psychiatry 2005; 76 (04) 573-575 DOI: 10.1136/jnnp.2004.040279.
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  • 2 Shirai W, Ito S, Hattori T. Linear T2 hyperintensity along the medial margin of the globus pallidus in patients with Machado-Joseph disease and Parkinson disease, and in healthy subjects. AJNR Am J Neuroradiol 2007; 28 (10) 1993-1995 DOI: 10.3174/ajnr.A0705.
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