Cognitive and olfactory deficits in Machado-Joseph disease: A dopamine transporter study

Cognitive and olfactory deficits in Machado-Joseph disease: A dopamine transporter study

Author Braga-Neto, Pedro Autor UNIFESP Google Scholar
Felicio, Andre C. Google Scholar
Hoexter, Marcelo Q. Autor UNIFESP Google Scholar
Pedroso, Jose Luiz Google Scholar
Dutra, Livia Almeida Google Scholar
Alessi, Helena Google Scholar
Minett, Thais Autor UNIFESP Google Scholar
Santos-Galduroz, Ruth F. Google Scholar
Rocha, Antonio Jose da Google Scholar
Garcia, Lucas A. L. Google Scholar
Bertolucci, Paulo Henrique F. Google Scholar
Bressan, Rodrigo A. Autor UNIFESP Google Scholar
Barsottini, Orlando Graziani Povoas Autor UNIFESP Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Univ Cambridge
Universidade Federal do ABC (UFABC)
Univ Estadual Paulista
Santa Casa Misericordia São Paulo
Beneficencia Portuguesa São Paulo
Abstract Cognitive and olfactory impairments have been demonstrated in patients with Machado-Joseph disease (MJD), and a possible relationship with dopaminergic dysfunction is implicated. However, there is still controversy regarding the pattern of striatal dopaminergic dysfunction in patients with MJD. in this study, we investigated whether these patients had different Dopamine Transporter (DAT) densities as compared to healthy subjects, and correlated these data with cognitive performance and sense of smell. Twenty-two MJD patients and 20 control subjects were enrolled. the neuropsychological assessment comprised the Spatial Span, Symbol Search, Picture Completion, Stroop Color Word Test, Trail Making Test and Phonemic Verbal Fluency test. the 16-item Sniffin' Sticks was used to evaluate odor identification. DAT imaging was performed using the SPECT radioligand [Tc-99m]-TRODAT-1, alongside with Magnetic Resonance imaging. Patients with MJD showed significantly lower DAT density in the caudate (1.34 +/- 0.27 versus 2.02 +/- 0.50, p < 0.001), posterior putamen (0.81 +/- 0.32 versus 1.32 +/- 0.34, p < 0.001) and anterior putamen (1.10 +/- 0.31 versus 1.85 +/- 0.45, p < 0.001) compared with healthy controls. the putamen/caudate ratio was also significantly lower in patients compared with controls (0.73 +/- 0.038 versus 0.85 +/- 0.032, p = 0.027). Even though we had only two patients with parkinsonisrn, we detected striatal dopaminergic deficits in those patients. No significant correlations were detected between DAT density and cognitive performance or Sniffin' Sticks scores. the data suggests that striatal dopamine deficit is not involved in cognitive or sense of smell deficits. This finding raises the possibility of extra-striatal dopamine and other neurotransmitter system involvement or of cerebellum neurodegeneration exerting a direct influence on cognitive and sensorial information processing in MJD. (C) 2012 Elsevier B.V. All rights reserved.
Keywords Spinocerebellar ataxia type 3
Machado-Joseph disease
Cognitive deficits
Dopamine transporter
Language English
Sponsor Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
Date 2012-08-01
Published in Parkinsonism & Related Disorders. Oxford: Elsevier B.V., v. 18, n. 7, p. 854-858, 2012.
ISSN 1353-8020 (Sherpa/Romeo, impact factor)
Publisher Elsevier B.V.
Extent 854-858
Access rights Closed access
Type Article
Web of Science ID WOS:000308970200010

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