Cerebellar Cognitive Affective Syndrome in Machado Joseph Disease: Core Clinical Features
Adult
Male
Machado-Joseph disease
Emotions
150
Anxiety
Neuropsychological Tests
Cohort Studies
Executive Function
03 medical and health sciences
Cognition
0302 clinical medicine
Spinocerebellar ataxia type 3
Cerebellar Diseases
Humans
Attention
Age of Onset
Aged
Neurologic Examination
Depression
Cognitive deficits
Machado-Joseph Disease
Middle Aged
3. Good health
Cerebellar Cognitive Affective Syndrome
Logistic Models
Memory, Short-Term
Female
DOI:
10.1007/s12311-011-0318-6
Publication Date:
2011-10-05T13:34:13Z
AUTHORS (11)
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.
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