Reply: Reference values for the Cerebellar Cognitive Affective Syndrome Scale: age and education matter

Abstract Background The cerebellar cognitive affective syndrome scale (CCAS-S) was recently developed to detect specific neuropsychological deficits in patients with cerebellar diseases in an expedited manner. Objectives To evaluate the discriminative ability of the CCAS-S in an etiologically homogeneous cohort of spinocerebellar ataxia type 3 (SCA3) patients and to examine relationships between cognitive deficits and motor symptom severity. Methods The CCAS-S was administered to twenty mildly to moderately affected SCA3 patients and eighteen healthy controls matched for age, sex, and educational level. Disease severity was measured by the Scale for the Assessment and Rating of Ataxia (SARA), Inventory of Non-Ataxia Signs (INAS), 8 m walk test, nine-hole peg test (9HPT), and Patient Health Questionnaire-9 (PHQ-9). Results SCA3 patients had a lower total CCAS-S score (p < 0.001) and higher number of failed tests (p = 0.006) than healthy controls. Patients displayed impairments in semantic fluency, phonemic fluency, category switching, cube drawing, and affect regulation. Total CCAS-S score showed high discriminative ability (area under the curve [AUC]: 0.96) and was associated with disease duration, SARA score, walking speed, and dominant hand 9HPT performance. No correlations were observed with INAS count, repeat length, and PHQ-9 score. Discriminative capacity of the number of failed tests was moderate (AUC: 0.76). Conclusion Essentially all SCA3 patients exhibited some form of cognitive impairment. The CCAS-S differentiates SCA3 patients from healthy controls, detects neuropsychological deficits early in the disease course, and correlates with relevant ataxia severity measures.

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Cerebellar Cognitive Affective Syndrome (CCAS)/Schmahmann Syndrome Scale

PsycTESTS Dataset ◽

10.1037/t67701-000 ◽

2017 ◽

Cited By ~ 1

Author(s):

Franziska Hoche ◽

Xavier Guell ◽

Mark G. Vangel ◽

Janet C. Sherman ◽

Jeremy D. Schmahmann

Keyword(s):

Cerebellar Cognitive Affective Syndrome ◽

Syndrome Scale

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EURRECAs Approach for Estimating Micronutrient Requirements

International Journal for Vitamin and Nutrition Research ◽

10.1024/0300-9831/a000071 ◽

2011 ◽

Vol 81 (4) ◽

pp. 256-263 ◽

Cited By ~ 22

Author(s):

Christophe Matthys ◽

Pieter van ‘t Veer ◽

Lisette de Groot ◽

Lee Hooper ◽

Adriënne E.J.M. Cavelaars ◽

...

Keyword(s):

Reference Values ◽

Policy Decision ◽

Individual Variability ◽

The Body ◽

Decision Makers ◽

Nutrient Requirements ◽

Factorial Approach ◽

Dietary Reference Values ◽

Health Policy Decision ◽

Quantitative Integration

In Europe, micronutrient dietary reference values have been established by (inter)national committees of experts and are used by public health policy decision-makers to monitor and assess the adequacy of diets within population groups. The approaches used to derive dietary reference values (including average requirements) vary considerably across countries, and so far no evidence-based reason has been identified for this variation. Nutrient requirements are traditionally based on the minimum amount of a nutrient needed by an individual to avoid deficiency, and is defined by the bodys physiological needs. Alternatively the requirement can be defined as the intake at which health is optimal, including the prevention of chronic diet-related diseases. Both approaches are confronted with many challenges (e. g., bioavailability, inter and intra-individual variability). EURRECA has derived a transparent approach for the quantitative integration of evidence on Intake-Status-Health associations and/or Factorial approach (including bioavailability) estimates. To facilitate the derivation of dietary reference values, EURopean micronutrient RECommendations Aligned (EURRECA) is developing a process flow chart to guide nutrient requirement-setting bodies through the process of setting dietary reference values, which aims to facilitate the scientific alignment of deriving these values.

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