SUPPLEMENTARY MATERIAL
Methods: explanation of cognitive tests and psychiatric scales
LONGITUDINAL STUDY OF COGNITIVE AND PSYCHIATRIC FUNCTIONS IN SPINOCEREBELLAR ATAXIAS TYPE 1 AND 2
Journal of Neurology
Fancellu R, Paridi D, Tomasello C, Panzeri M, Castaldo A, Genitrini S, Soliveri P, Girotti F
Corresponding Author: Roberto Fancellu
Unit of Neurology, VillaScassiHospital – ASL3, Corso Scassi 1 – 16149 Genova – Italy
Tel.: +390108492382; +393395288892; Fax: +390108492584
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Cognitive tests
MiniMentalState Examination (MMSE):test for global cognitive conditions, assessing orientation in time and space, attention, calculation, short- and long-term verbal memory, language and praxic functions. The score ranges from 0 (very severe cognitive deterioration) to 30 (normal cognitive functions)[36, 37]. A score below the cut-off of 24 usually indicates the presence of dementia.
Digit Span: short-term verbal memory test. The subject listens to and tries to correctly repeat growing random sequences of digits; in case of mistake, a second sequence of the same length is proposed. The score is represented by the longest digit sequence correctly repeated by the examinee [38].
Corsi Span: short-term visuo-spatial memory test. The examiner touches growing random sequences of cubes placed on a board and the subject has to touch in the same order the same cubes touched by the examiner; two different sequences of the same length has to be correctly touched by the subject before to pass to a longer sequence. The score is the longest sequence of cubes correctly touched by the subject [39].
Verbal Memory (immediate and delayed recall): short- and long-term episodic verbal memory test. The subject listens to a short tale and tries to recall the story immediately and after ten minutes occupied by an interference non-verbal trial. The score is represented by the average of the primary and secondary elements of the story recalled by the examinee both in immediate and in delayed trial [40]. This test is the Italian translationof Babcock Story Recall Test,validated and standardized in Italian population.
Attentional Matrices: selective attention and information processing speed test. The subject has to find and check the correct target number(s) in three consecutive matrices with increasing difficulty in a fixed time (one number in the first matrix, two numbers in the second one and three numbers in the third matrix). The sum of the correct target numbers checked in the three trials represents the test score [39].
Modified Wisconsin Card Sorting Test (mWCST) according to Nelson version: executive function test. The subject has to divide a deck of cards in four groups, following one of three possible criteria (shape, color, or number of symbols). The examinee decides the criterion to order the sorting cards and has to change it by following the request of the examiner. Then, the sequence of the criteria has to be repeated in the same order a second time. The scores are the number of correctly concluded series, the total errors and the perseveration errors [41, 42].
Raven’s Progressive (Colored) Matrices (RPM): logical and deductive intelligence and visuo-perceptive ability test. The test is composed by 36 colored tables, divided in 3 series; every table is characterized by a figure with a missing part, and by six possible solutions. The subject has to find the correct answer completing the figure. The test score is the sum of correct answers [43, 44].
Phonemic Fluency: control function test. The subject is required to say as many words as possible beginning with a letter given by the examiner (F-P-L), in the space of a minute for each letter. The score is the sum of the correct words [40].
Semantic Fluency: test for word-searching ability with semantic access. The subject has to say as many words as possible belonging to a defined category (animal-fruit-car brand), in the space of a minute for each category. The score is the sum of the correct words [40].
Benton Test: visual and spatial ability test. The subject is required to evaluate the different orientation of couple of lines. The score is the sum of the correct answers [45, 46].
Psychiatric tests
Hamilton Depression Scale (HDS): this 21-item scale evaluates, on the basis of a score ranging from 0 to 4 for each item, the occurrence and the entity of depressive symptoms in different areas. Scores higher than 14 are suggestive for depression[47].
Hamilton Anxiety Scale (HAS):in this scaleseparate scores, ranging from 0 to 4 in each item, are assigned according to the presence and the level of anxiety symptoms. The entity of anxiety was considered mild with scores lower than 18, moderate between 18 and 24, and severe with scores higher than 24 [48].
Scale for Assessment of Negative Symptoms (SANS):this scale assesses, on a six-point scale (0=not at all, to 5=severe), five symptom complexes in order to obtain clinical ratings of negative symptoms: affective flattening or blunting; alogia (impoverished thinking); avolition/apathy; anhedonia/asociality; disturbance of attention [49, 50].
Scale for Assessment of Positive Symptoms (SAPS): during the clinical interview, the examiner explores, with a score ranging from 0=none to 5=severe, the occurrence of the following positive symptoms: hallucinations, delusions, bizarre behavior, positive formal thought disorder[50].
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