Translation and validation into Brazilian version of the Scale of the Assessment and Rating of Ataxia (SARA)

Braga‐Neto, Pedro; Godeiro-Júnior, Clécio; Dutra, Lívia Almeida; Pedroso, José Luiz; Barsottini, Orlando Graziani Póvoas

doi:10.1590/s0004-282x2010000200014

Cited by 75 publications

(45 citation statements)

References 12 publications

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“…The demographic variables studied were: age, disease duration and gender. Patients with SCA3/MJD were evaluated using the following clinical scales: International Cooperative Ataxia Rating Scale (ICARS) and Brazilian version of the Scale for the Assessment and Rating of Ataxia (SARA) [11] were used determine the severity of ataxia symptoms; Unified Parkinson’s Disease Rating Scale part III (UPDRS III) was performed to determine parkinsonism, since it is often described in patients with SCA3/MJD and the Hamilton Anxiety Scale and Beck Depression Inventory to evaluate psychiatric symptoms (anxiety and depression, respectively). The number of CAG(n) expansions was also recorded.…”

Section: Methodsmentioning

confidence: 99%

Is Neuropathy Involved with Restless Legs Syndrome in Machado-Joseph Disease?

Pedroso

Bezerra²,

Braga‐Neto³

et al. 2011

Eur Neurol

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Objective: To evaluate a group of spinocerebellar ataxia type 3 (SCA3) or Machado-Joseph disease (MJD) (SCA3/MJD) patients and assess whether there is an association between neuropathy and serum ferritin levels and restless legs syndrome (RLS). Methods: Twenty-six SCA3/MJD patients underwent electromyography studies to check for neuropathy. Their serum ferritin levels were measured as well. These findings were evaluated based on the presence or not of RLS and its severity. Results: The proportion of neuropathy in the RLS group was not significantly higher compared to the non-RLS group (23 vs. 15%, Fisher’s exact test, p = 1.000). Furthermore, no association was found between RLS and ferritin levels. Conclusion: We found no correlation between neuropathy or ferritin levels and RLS in SCA3/MJD patients.

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Section: Methodsmentioning

confidence: 99%

Is Neuropathy Involved with Restless Legs Syndrome in Machado-Joseph Disease?

Pedroso

Bezerra²,

Braga‐Neto³

et al. 2011

Eur Neurol

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“…Patients underwent detailed neurological examination and the SARA scale was used to quantify disease severity [17]. Age of onset, disease duration, and length of expansion (CAG) were determined for each subject and used for further analyses.…”

Section: Clinical Evaluationmentioning

confidence: 99%

Spinal Cord Damage in Machado-Joseph Disease

et al. 2014

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Machado-Joseph disease (SCA3) is the most frequent spinocerebellar ataxia worldwide and characterized by remarkable phenotypic heterogeneity. MRI-based studies in SCA3 focused in the cerebellum and connections, but little is known about cord damage in the disease and its clinical relevance. To evaluate the spinal cord damage in SCA3 through quantitative analysis of MRI scans. A group of 48 patients with SCA3 and 48 age and gender-matched healthy controls underwent MRI on a 3T scanner. We used T1-weighted 3D images to estimate the cervical spinal cord area (CA) and eccentricity (CE) at three C2/C3 levels based on a semi-automatic image segmentation protocol. The scale for assessment and rating of ataxia (SARA) was employed to quantify disease severity. The two groups-SCA3 and controls-were significantly different regarding CA (49.5 ± 7.3 vs 67.2 ± 6.3 mm(2), p < 0.001) and CE values (0.79 ± 0.06 vs 0.75 ± 0.05, p = 0.005). In addition, CA presented a significant correlation with SARA scores in the patient group (p = 0.010). CE was not associated with SARA scores (p = 0.857). In the multiple variable regression, we found that disease duration was the only variable associated with CA (coefficient = -0.629, p = 0.025). SCA3 is characterized by cervical cord atrophy and antero-posterior flattening. In addition, the spinal cord areas did correlate with disease severity. This suggests that quantitative analyses of the spinal cord MRI might be a useful biomarker in SCA3.

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“…The demographic variables studied included: gender, age at disease onset, age at disease evaluation, and disease duration. We also used the following clinical scales to assess the SCA3/MJD group: International Cooperative Ataxia Rating Scale, Brazilian-translated and -validated Scale for the Assessment and Rating of Ataxia [14,15], Unified Parkinson's Disease Rating Scale part III, Hamilton Anxiety Scale (HAM-A), and Beck Depression Inventory (BDI). The number of CAG(n) expansions was also recorded.…”

Section: Subjects and Clinical Protocolmentioning

confidence: 99%

Sleep Disorders in Machado–Joseph Disease: Frequency, Discriminative Thresholds, Predictive Values, and Correlation with Ataxia-Related Motor and Non-Motor Features

et al. 2011

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Sleep disorders are common complaints in patients with neurodegenerative diseases such as spinocerebellar ataxia type 3 (SCA3) or Machado-Joseph disease (MJD)--SCA3/MJD. We evaluated the frequency of sleep disorders in SCA3/MJD patients against controls matched by age and gender, and correlated data with demographic and clinical variables. The main sleep disorders evaluated were rapid eye movement (REM) sleep behavior disorder (RBD), restless leg syndrome (RLS), and excessive daytime sleepiness (EDS). We recruited 40 patients with clinical and molecular-proven SCA3/MJD and 38 controls. We used the following clinical scales to evaluate our primary outcome measures: RBD Screening Questionnaire, International RLS Rating Scale, and Epworth Sleepiness Scale. To evaluate ataxia-related motor and non-motor features, we applied the International Cooperative Ataxia Rating Scale, the Scale for the Assessment and Rating of Ataxia, and the Unified Parkinson's Disease Rating Scale part III. Psychiatric manifestations were tested with the Hamilton Anxiety Scale, and Beck Depression Inventory. The frequency of RBD and RLS were significantly higher in the SCA3/MJD group than in the control group (p < 0.001). There was no difference between both groups with regard to EDS. The accuracy of RDBSQ to discriminate between cases and controls was considered the best area under the ROC curve (0.86). Within-SCA3/MJD group analysis showed that anxiety and depression were significantly correlated with RDB, but not with RLS. Additionally, depression was considered the best predictive clinical feature for RDB and EDS.

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Translation and validation into Brazilian version of the Scale of the Assessment and Rating of Ataxia (SARA)

Cited by 75 publications

References 12 publications

Is Neuropathy Involved with Restless Legs Syndrome in Machado-Joseph Disease?

Is Neuropathy Involved with Restless Legs Syndrome in Machado-Joseph Disease?

Spinal Cord Damage in Machado-Joseph Disease

Sleep Disorders in Machado–Joseph Disease: Frequency, Discriminative Thresholds, Predictive Values, and Correlation with Ataxia-Related Motor and Non-Motor Features

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