Scale for the assessment and rating of ataxia

Schmitz‐Hübsch, Tanja; Montcel, Sophie Tezenas du; Baliko, L.; Berciano, José; Boesch, Sylvia; Depondt, Chantal; Giunti, Paola; Globas, Christoph; Infante, Jon; Kang, Jun-Suk; Kremer, Berry; Mariotti, Caterina; Melegh, Béla; Pandolfo, Massimo; Rakowicz, Maria; Ribaı̈, Pascale; Rola, Rafał; Schöls, Lüdger; Szymanski, Sandra; Warrenburg, Bart P. van de; Dürr, Alexandra; Klockgether, Thomas; Fancellu, Roberto

doi:10.1212/01.wnl.0000219042.60538.92

Cited by 1,538 publications

(1,335 citation statements)

References 10 publications

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“…SARA has 1 underlying construct that explains 80% of the variation with 8 separate criteria, all scored on ordinal scales (gait, stance, sitting, speech disturbance, finger chase, nose‐finger test, fast alternating hand movements, and heel‐shin slide). SARA has high inter‐rater reliability ICC (0.98) and a test–retest reliability ICC of 0.90 39. The equine modified ataxia scale in this study performed comparably to subscales of the ICARS, but poorly in comparison with the overall excellent agreement and reliability of SARA 39.…”

Section: Discussionmentioning

confidence: 57%

“…SARA has high inter‐rater reliability ICC (0.98) and a test–retest reliability ICC of 0.90 39. The equine modified ataxia scale in this study performed comparably to subscales of the ICARS, but poorly in comparison with the overall excellent agreement and reliability of SARA 39. However, only neurologists or senior neurology residents assessed the patients in the SARA work and it was conducted on greater numbers of subjects, meaning that a direct comparison with this study might be misleading.…”

Section: Discussionmentioning

confidence: 99%

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Rater Agreement on Gait Assessment during Neurologic Examination of Horses

Olsen

Dunkel

Barker

et al. 2014

Veterinary Internal Medicne

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BackgroundReproducible and accurate recognition of presence and severity of ataxia in horses with neurologic disease is important when establishing a diagnosis, assessing response to treatment, and making recommendations that might influence rider safety or a decision for euthanasia.ObjectivesTo determine the reproducibility and validity of the gait assessment component in the neurologic examination of horses.AnimalsTwenty‐five horses referred to the Royal Veterinary College Equine Referral Hospital for neurological assessment (n = 15), purchased (without a history of gait abnormalities) for an unrelated study (n = 5), or donated because of perceived ataxia (n = 5).MethodsUtilizing a prospective study design; a group of board‐certified medicine (n = 2) and surgery (n = 2) clinicians and residents (n = 2) assessed components of the equine neurologic examination (live and video recorded) and assigned individual and overall neurologic gait deficit grades (0–4). Inter‐rater agreement and assessment‐reassessment reliability were quantified using intraclass correlation coefficients (ICC).ResultsThe ICCs of the selected components of the neurologic examination ranged from 0 to 0.69. “Backing up” and “recognition of mistakes over obstacle” were the only components with an ICC > 0.6. Assessment‐reassessment agreement was poor to fair. The agreement on gait grading was good overall (ICC = 0.74), but poor for grades ≤ 1 (ICC = 0.08) and fair for ataxia grades ≥ 2 (ICC = 0.43). Clinicians with prior knowledge of a possible gait abnormality were more likely to assign a grade higher than the median grade.Conclusion and Clinical ImportanceClinicians should be aware of poor agreement even between skilled observers of equine gait abnormalities, especially when the clinical signs are subtle.

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Section: Discussionmentioning

confidence: 57%

Section: Discussionmentioning

confidence: 99%

Rater Agreement on Gait Assessment during Neurologic Examination of Horses

Olsen

Dunkel

Barker

et al. 2014

Veterinary Internal Medicne

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“…Of 17 patients, four could not tolerate one of either baclofen or chlorzoxazone due to side effects (Table 1). The Scale for the Assessment and Rating of Ataxia (SARA) is a validated clinical measure of ataxia, with higher scores indicating more prominent ataxia 58. SARA scores were recorded for all patients prior to beginning treatment and were assessed during subsequent visits.…”

Section: Resultsmentioning

confidence: 99%

Targeting potassium channels to treat cerebellar ataxia

Bushart

Chopra

Singh

et al. 2018

Ann Clin Transl Neurol

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ObjectivePurkinje neuron dysfunction is associated with cerebellar ataxia. In a mouse model of spinocerebellar ataxia type 1 (SCA1), reduced potassium channel function contributes to altered membrane excitability resulting in impaired Purkinje neuron spiking. We sought to determine the relationship between altered membrane excitability and motor dysfunction in SCA1 mice.MethodsPatch‐clamp recordings in acute cerebellar slices and motor phenotype testing were used to identify pharmacologic agents which improve Purkinje neuron physiology and motor performance in SCA1 mice. Additionally, we retrospectively reviewed records of patients with SCA1 and other autosomal‐dominant SCAs with prominent Purkinje neuron involvement to determine whether currently approved potassium channel activators were tolerated.ResultsActivating calcium‐activated and subthreshold‐activated potassium channels improved Purkinje neuron spiking impairment in SCA1 mice (P < 0.05). Additionally, dendritic hyperexcitability was improved by activating subthreshold‐activated potassium channels but not calcium‐activated potassium channels (P < 0.01). Improving spiking and dendritic hyperexcitability through a combination of chlorzoxazone and baclofen produced sustained improvements in motor dysfunction in SCA1 mice (P < 0.01). Retrospective review of SCA patient records suggests that co‐treatment with chlorzoxazone and baclofen is tolerated.InterpretationTargeting both altered spiking and dendritic membrane excitability is associated with sustained improvements in motor performance in SCA1 mice, while targeting altered spiking alone produces only short‐term improvements in motor dysfunction. Potassium channel activators currently in clinical use are well tolerated and may provide benefit in SCA patients. Future clinical trials with potassium channel activators are warranted in cerebellar ataxia.

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“…The mean progression rate is 1.49 (±0.07 SE) per year at the Scale for Assessment and Rating of Ataxia (SARA),40 with earlier age at onset being associated with faster progression 41…”

Section: Clinical Featuresmentioning

confidence: 99%

The Multiple Faces of Spinocerebellar Ataxia type 2

Antenora

Rinaldi

Roca

et al. 2017

Ann Clin Transl Neurol

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Spinocerebellar ataxia type 2 (SCA2) is among the most common forms of autosomal dominant ataxias, accounting for 15% of the total families. Occurrence is higher in specific populations such as the Cuban and Southern Italian. The disease is caused by a CAG expansion in ATXN2 gene, leading to abnormal accumulation of the mutant protein, ataxin‐2, in intracellular inclusions. The clinical picture is mainly dominated by cerebellar ataxia, although a number of other neurological signs have been described, ranging from parkinsonism to motor neuron involvement, making the diagnosis frequently challenging for neurologists, particularly when information about the family history is not available. Although the functions of ataxin‐2 have not been completely elucidated, the protein is involved in mRNA processing and control of translation. Recently, it has also been shown that the size of the CAG repeat in normal alleles represents a risk factor for ALS, suggesting that ataxin‐2 plays a fundamental role in maintenance of neuronal homeostasis.

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Scale for the assessment and rating of ataxia

Abstract: The Scale for the Assessment and Rating of Ataxia is a reliable and valid measure of ataxia, making it an appropriate primary outcome measure for clinical trials.

Cited by 1,538 publications

References 10 publications

Rater Agreement on Gait Assessment during Neurologic Examination of Horses

Rater Agreement on Gait Assessment during Neurologic Examination of Horses

Targeting potassium channels to treat cerebellar ataxia

The Multiple Faces of Spinocerebellar Ataxia type 2

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