Cerebellar Cognitive Affective Syndrome in Machado Joseph Disease: Core Clinical Features

Cerebellar Cognitive Affective Syndrome in Machado Joseph Disease: Core Clinical Features

Author Braga-Neto, Pedro Autor UNIFESP Google Scholar
Pedroso, Jose Luiz Autor UNIFESP Google Scholar
Alessi, Helena Autor UNIFESP Google Scholar
Dutra, Livia Almeida Autor UNIFESP Google Scholar
Felicio, Andre Carvalho Autor UNIFESP Google Scholar
Minett, Thais Autor UNIFESP Google Scholar
Weisman, Patricia Autor UNIFESP Google Scholar
Santos-Galduroz, Ruth F. Google Scholar
Bertolucci, Paulo Henrique F. Autor UNIFESP Google Scholar
Gabbai, Alberto Alain Autor UNIFESP Google Scholar
Barsottini, Orlando Graziani Povoas Autor UNIFESP Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Hosp Israelita Albert Einstein
Univ Cambridge
Universidade Federal do ABC (UFABC)
Abstract The cerebellum is no longer considered a purely motor control device, and convincing evidence has demonstrated its relationship to cognitive and emotional neural circuits. the aims of the present study were to establish the core cognitive features in our patient population and to determine the presence of Cerebellar Cognitive Affective Syndrome (CCAS) in this group. We recruited 38 patients with spinocerebellar ataxia type 3 (SCA3) or Machado-Joseph disease (MJD)-SCA3/MJD and 31 controls. Data on disease status were recorded (disease duration, age, age at onset, ataxia severity, and CAG repeat length). the severity of cerebellar symptoms was measured using the International Cooperative Ataxia Rating Scale and the Scale for the Assessment and Rating of Ataxia. the neuropsychological assessment consisted of the Mini-Mental State Examination, Clock Drawing Test, Wechsler Adult Intelligence Scale, Rey-Osterrieth Complex Figure, Wisconsin Card Sorting Test, Stroop Color-Word Test, Trail-Making Test, Verbal Paired Associates, and verbal fluency tests. All subjects were also submitted to the Hamilton Anxiety Scale and Beck Depression Inventory. After controlling for multiple comparisons, spatial span, picture completion, symbol search, Stroop Color-Word Test, phonemic verbal fluency, and Trail-Making Tests A and B were significantly more impaired in patients with SCA3/MJD than in controls. Executive and visuospatial functions are impaired in patients with SCA3/MJD, consistent with the symptoms reported in the CCAS. We speculate on a possible role in visual cortical processing degeneration and executive dysfunction in our patients as a model to explain their main cognitive deficit.
Keywords Spinocerebellar ataxia type 3
Machado-Joseph disease
Cognitive deficits
Cerebellar Cognitive Affective Syndrome
Language English
Sponsor Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
Date 2012-06-01
Published in Cerebellum. New York: Springer, v. 11, n. 2, p. 549-556, 2012.
ISSN 1473-4222 (Sherpa/Romeo, impact factor)
Publisher Springer
Extent 549-556
Origin http://dx.doi.org/10.1007/s12311-011-0318-6
Access rights Closed access
Type Article
Web of Science ID WOS:000304466100032
URI http://repositorio.unifesp.br/handle/11600/34949

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