Internal consistency of a Brazilian version of the unified Huntington's disease rating scale

Tumas, Vítor; Camargos, Sarah Teixeira; Jalali, Paulo Samandar; Galesso, Adriano de Paula; Marques, Wilson

doi:10.1590/s0004-282x2004000600009

Cited by 13 publications

(17 citation statements)

References 9 publications

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“…There are no available data on test–retest reliability or inter‐rater reliability. Good convergent validity with other components of the UHDRS has been shown, as well as with motor disability, cognitive, and behavioral deficits . The UHDRS‐FAS has been shown to be sensitive to change over time in several studies …”

Section: Rating Scalesmentioning

confidence: 90%

“…The clinimetric data available show that the UHDRS‐IS has moderate inter‐rater reliability but in a modified version that compares caregiver report with patient self‐report (ICC = 0.71, 95% CI: 0.48, 0.85) . Good correlation with other components of the UHDRS, as well as motor disability, cognitive and behavioral deficits has been shown in various studies . Data from clinical trials suggest sensitivity of the UHDRS‐IS to change over time and across disease stages …”

Section: Rating Scalesmentioning

confidence: 99%

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Rating Scales and Performance‐based Measures for Assessment of Functional Ability in Huntington's Disease: Critique and Recommendations

Mestre

Busse

Davis

et al. 2018

Movement Disord Clin Pract

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Limitation of functional ability is a major feature of Huntington's disease (HD). The International Parkinson and Movement Disorder Society (MDS) commissioned the appraisal of the use and clinimetric properties of clinical measures of functional ability that have been applied in HD studies and trials to date, to make recommendations regarding their use based on standardized criteria. After a systematic literature search, we included a total of 29 clinical measures grouped into two categories: (1) performance‐based measures (e.g., balance, walking, and reaching/grasping), and (2) rating scales. Three performance‐based measures are rated as “recommended”: the Tinetti Mobility Test for screening of fall risk and for severity assessment of mobility in patients with manifest HD (up to stage III); the Berg Balance Scale for severity of balance impairment; and the Six‐Minute Walk Test for assessment of walking endurance (severity) in HD subjects with preserved ambulation. No rating scale targeting functional ability reached a “recommended” status either for screening or severity measurement. The main challenges identified in this review include applying widely accepted conceptual frameworks to the identified measures, the lack of validation of clinical measures to detect change over time, and absence of validated measures for upper limb function. Furthermore, measures of capacity or ability to perform activities of daily living had ceiling effects in people with early and pre‐manifest HD. We recommend that the MDS prioritize the development of new scales that capture small, but meaningful changes in function over time for outcome assessment in clinical trials, particularly in earlier stages of HD.

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Section: Rating Scalesmentioning

confidence: 90%

Section: Rating Scalesmentioning

confidence: 99%

Rating Scales and Performance‐based Measures for Assessment of Functional Ability in Huntington's Disease: Critique and Recommendations

Mestre

Busse

Davis

et al. 2018

Movement Disord Clin Pract

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“…The motor and cognitive evaluation was performed using the Brazilian version of the Unified Huntington's Disease Rating Scale (UHDRS) 8,9 , which addresses four domains of clinical and functional performance: motor function, behavioral abnormalities, cognitive function and functional capacity. The cognitive evaluation included the MMSE, the FAS-COWA, the Digit Symbol Modalities Test (DSMT) 10 , the Stroop Test 11 and the Hooper Visual Organization Test (HVOT) 12 .…”

Section: Methodsmentioning

confidence: 99%

Language impairment in Huntington's disease

Azambuja¹,

Radanovic²,

Haddad³

et al. 2012

Arq. Neuro-Psiquiatr.

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Language alterations in Huntington's disease (HD) are reported, but their nature and correlation with other cognitive impairments are still under investigation. This study aimed to characterize the language disturbances in HD and to correlate them to motor and cognitive aspects of the disease. We studied 23 HD patients and 23 controls, matched for age and schooling, using the Boston Diagnostic Aphasia Examination, Boston Naming Test, the Token Test, Animal fluency, Action fluency, FAS-COWA, the Symbol Digit Modalities Test, the Stroop Test and the Hooper Visual Organization Test (HVOT). HD patients performed poorer in verbal fluency (p<0.0001), oral comprehension (p<0.0001), repetition (p<0.0001), oral agility (p<0.0001), reading comprehension (p=0.034) and narrative writing (p<0.0001). There was a moderate correlation between the Expressive Component and Language Competency Indexes and the HVOT (r=0.519, p=0.011 and r=0.450, p=0.031, respectively). Language alterations in HD seem to reflect a derangement in both frontostriatal and frontotemporal regions.

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“…They were routinely evaluated with a validated Brazilian version of the UHDRS including the VFT, SDMT, SIT [30], the MMSE and the FAB. For comparison, 53 healthy controls matched by age, sex and education were also selected.…”

Section: Patients Selectionmentioning

confidence: 99%

Use of the frontal assessment battery in evaluating executive dysfunction in patients with Huntington’s disease

et al. 2009

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The frontal assessment battery (FAB) is a bedside cognitive scale designed to measure executive functions. Huntington's disease (HD) is a neurodegenerative disorder characterized by motor, behavioral, and cognitive dysfunction. The aim of this study was to check the validity of the FAB for the evaluation of cognitive impairment in patients with HD. Forty-one patients diagnosed with HD and 53 healthy controls matched by education, sex and age were evaluated with a validated Brazilian version of the UHDRS, the VFT, the SDMT, the SIT, the MMSE, and the FAB. The diagnosis of HD was made by DNA analysis. FAB scores were lower in patients than in the controls (p < 0.001) and had significant correlations with the VFT (r = 0.79; p < 0.05), the SDMT (r = 0.80; p < 0.05), the SIT (r = 0.72; p < 0.05), the MMSE (r = 0.83; p < 0.05), the FCS (r = 0.79; p < 0.05) and the motor section of the UHDRS (r = -0.80; p < 0.05). The FAB differentiated between HD patients in the initial and later stages of the disease. The one-year longitudinal evaluation revealed a global trend toward a worsening in the second score of the FAB. The results demonstrate that the FAB presents good internal consistency and also convergent and discriminative validity; therefore it is a useful scale to assess executive functions and to evaluate cognitive impairment in patients with HD.

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Internal consistency of a Brazilian version of the unified Huntington's disease rating scale

Cited by 13 publications

References 9 publications

Rating Scales and Performance‐based Measures for Assessment of Functional Ability in Huntington's Disease: Critique and Recommendations

Rating Scales and Performance‐based Measures for Assessment of Functional Ability in Huntington's Disease: Critique and Recommendations

Language impairment in Huntington's disease

Use of the frontal assessment battery in evaluating executive dysfunction in patients with Huntington’s disease

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