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Protocol - Ataxia Rating Scale

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Description:

The Scale for the Assessment and Rating of Ataxia (SARA) is a clinical scale used to assess cerebellar ataxia. The scale includes 8 items that are related to gait, stance, sitting, speech, finger-chase test, nose-finger test, fast alternating movements and heel-shin test. A trained physician observes the participant perform these physical function tests and gives each one a value from the scale.

Protocol:

1) Gait

Proband is asked (1) to walk at a safe distance parallel to a wall including a half-turn (turn around to face the opposite direction of gait) and (2) to walk in tandem

(heels to toes) without support.

0 = Normal, no difficulties in walking, turning and walking tandem (up to one misstep allowed)

1 = Slight difficulties, only visible when walking 10 consecutive steps in tandem

2 = Clearly abnormal, tandem walking >10 steps not possible

3 = Considerable staggering, difficulties in half-turn, but without support

4 = Marked staggering, intermittent support of the wall required

5 = Severe staggering, permanent support of one stick or light support by one arm required

6 = Walking > 10 m only with strong support (two special sticks or stroller or accompanying person)

7 = Walking < 10 m only with strong support (two special sticks or stroller or accompanying person)

8 = Unable to walk, even supported

SCORE: _____________

2) Stance

Proband is asked to stand (1) in natural position, (2) with feet together in parallel (big toes touching each other) and (3) in tandem (both feet on one line, no space between

heel and toe). Proband does not wear shoes, eyes are open. For each condition, three trials are allowed. Best trial is rated.

0 = Normal, able to stand in tandem for > 10 s

1 = Able to stand with feet together without sway, but not in tandem for > 10s

2 = Able to stand with feet together for > 10 s, but only with sway

3 = Able to stand for > 10 s without support in natural position but not with feet together

4 = Able to stand for >10 s in natural position only with intermittent support

5 = Able to stand >10 s in natural position only with constant support of one arm

6 = Unable to stand for >10 s even with constant support of one arm

SCORE: _____________

3) Sitting

Proband is asked to sit on an examination bed without support of feet, eyes open and arms outstretched to the front.

0 = Normal, no difficulties sitting >10 sec

1 = Slight difficulties, intermittent sway

2 = Constant sway, but able to sit > 10 s without support

3 = Able to sit for > 10 s only with intermittent support

4 = Unable to sit for >10 s without continuous support

SCORE: _____________

4) Speech disturbance

Speech is assessed during normal conversation.

0 = Normal

1 = Suggestion of speech disturbance

2 = Impaired speech, but easy to understand

3 = Occasional words difficult to understand

4 = Many words difficult to understand

5 = Only single words understandable

6 = Speech unintelligible / anarthria

SCORE: _____________

5) Finger chase

Rated separately for each side

Proband sits comfortably. If necessary, support of feet and trunk is allowed. Examiner sits in front of proband and performs 5 consecutive sudden and fast pointing

movements in unpredictable directions in a frontal plane, at about 50 % of proband´s reach. Movements have an amplitude of 30 cm and a frequency of 1 movement

every 2 s. Proband is asked to follow the movements with his index finger, as fast and precisely as possible. Average performance of last 3 movements is rated.

0 = No dysmetria

1 = Dysmetria, under/overshooting target <5 cm

2 = Dysmetria, under/overshooting target < 15 cm

3 = Dysmetria, under/overshooting target > 15 cm

4 = Unable to perform 5 pointing movements

SCORE ____Right:___ Left:______

Mean of both sides (R+L)/2:______________

6) Nose-finger test

Rated separately for each side

Proband sits comfortably. If necessary, support of feet and trunk is allowed. Proband is asked to point repeatedly with his index finger from his nose to examiner’s finger which is in front of the proband at about 90 % of proband’s reach. Movements are performed at moderate speed. Average performance of movements is rated according to the amplitude of the kinetic tremor.

0 = No tremor

1 = Tremor with an amplitude < 2 cm

2 = Tremor with an amplitude < 5 cm

3 = Tremor with an amplitude > 5 cm

4 = Unable to perform 5 pointing movements

SCORE____Right:___ Left:______

Mean of both sides (R+L)/2:______________

7) Fast alternating hand movements

Rated separately for each side

Proband sits comfortably. If necessary, support of feet and trunk is allowed. Proband is asked to perform 10 cycles of repetitive alternation of pro- and supinations of

the hand on his/her thigh as fast and as precise as possible. Movement is demonstrated by examiner at a speed of approx. 10 cycles within 7 s. Exact times for

movement execution have to be taken.

0 = Normal, no irregularities (performs <10s)

1 = Slightly irregular (performs <10s)

2 = Clearly irregular, single movements difficult to distinguish or relevant interruptions, but performs <10s

3 = Very irregular, single movements difficult to distinguish or relevant interruptions,

performs >10s

4 = Unable to complete 10 cycles

SCORE ____Right:___ Left:______

Mean of both sides (R+L)/2:______________

8) Heel-shin slide

Rated separately for each side

Proband lies on examination bed, without sight of his legs. Proband is asked to lift one leg, point with the heel to the opposite knee, slide down along the shin to the

ankle, and lay the leg back on the examination bed. The task is performed 3 times. Slide-down movements should be performed within 1 s. If proband slides down without contact to shin in all three trials, rate 4.

0 = Normal

1 = Slightly abnormal, contact to shin maintained

2 = Clearly abnormal, goes off shin up to 3 times during 3 cycles

3 = Severely abnormal, goes off shin 4 or more times during 3 cycles

4 = Unable to perform the task

SCORE ____Right:___ Left:______

Mean of both sides (R+L)/2:______________

Mean values from each one of the eight items are summed to obtain the total score.

The total scores range from 0 (no ataxia) to 40 (severe ataxia).

(If scale administered to children, the results should be interpreted based on age.)

Protocol Name from Source:

This section will be completed when reviewed by an Expert Review Panel.

Availability:

Publicly available

Personnel and Training Required

Trained clinician must administer the protocol

Equipment Needs

Examination bed

Requirements
Requirement CategoryRequired
Major equipment No
Specialized training Yes
Specialized requirements for biospecimen collection No
Average time of greater than 15 minutes in an unaffected individual No
Mode of Administration

Physical Measurement

Life Stage:

Child, Adolescent, Adult

Participants:

Ages 4 or older but normally administered to adults and older adults

Specific Instructions:

The Scale for the Assessment and Rating of Ataxia (SARA) must be administered by a trained clinician.

Selection Rationale

The Scale for the Assessment and Rating of Ataxia (SARA) has good metric properties and inter-rater reliability, is easy to use and is a promising outcome measure for future clinical trials. This scale has been shown to be a reliable and valid scale for measuring ataxia. Its scores correlate closely with other scales of ataxia, as well as activities of daily living.

Language

English

Standards
StandardNameIDSource
Common Data Elements (CDE) Ataxia Rating Assessment Scale 4797993 CDE Browser
Derived Variables

None

Process and Review

This section will be completed when reviewed by an Expert Review Panel.

Source

Schmitz-Hübsch, T., du Montcel, S. T., Baliko, L., Berciano, J., Boesch, S., Depondt, C., Fancellu, R. (2006). Scale for the assessment and rating of ataxia: Development of a new clinical scale. Neurology, 66(11), 1717-1720.

General References

Brandsma, R., Spits, A. H., Kuiper, M. J., Lunsing, R. J., Burger, H., Kremer, H. P., Sival, D. A. (2014). Ataxia rating scales are age-dependent in healthy children. Developmental Medicine & Child Neurology, 56(6), 556-563.

Jacobi, H., Reetz, K., du Montcel, S. T., Bauer, P., Mariotti, C., Nanetti, L., Klockgether T. (2013). Biological and clinical characteristics of individuals at risk for spinocerebellar ataxia types 1, 2, 3, and 6 in the longitudinal RISCA study: analysis of baseline data. The Lancet Neurology, 12(7), 650-658.

Kim, B.-R., Lim, J.-H., Lee, S. A., Park, S., Koh, S.-E., Lee, I. et al. (2011). Usefulness of the Scale for the Assessment and Rating of Ataxia (SARA) in ataxic stroke patients. Annals of Rehabilitation Medicine, 35(6), 772-780.

Saute, J. A., Donis, K. C., Serrano-Munuera, C., Genis, D., Ramirez, L. T., Mazzetti, P., Jardim, I. B.; Iberoamerican Multidisciplinary Network for the Study of Movement Disorders (RIBERMOV) Study Group. (2012). Ataxia rating scales-psychometric profiles, natural history and their application in clinical trials. Cerebellum, 11(2), 488-504.

Schmitz-Hübsch, T., Fimmers, R., Rakowicz, M., Rola, R., Zdzienicka, E., Fancellu, R., Klockgether, T. (2010). Responsiveness of different rating instruments in spinocerebellar ataxia patients. Neurology, 74(8), 678-684

Weyer, A., Abele, M., Schmitz-Hübsch, T., Schoch, B., Frings, M., Klockgether, T., (2007). Reliability and validity of the Scale for the Assessment and Rating of Ataxia: A study in 64 ataxia patients. Movement Disorders, 22(11), 1633-1637.

Protocol ID:

220101

Variables:
Export Variables
Variable NameVariable IDVariable DescriptionVersiondbGaP Mapping
PX220101_Ataxia_Fast_Alternating_HandMovement_Mean PX220101070300 Mean of both sides (R+L)/2:______________ 4 N/A
PX220101_Ataxia_Fast_Alternating_Hand_Movement_Left PX220101070200 Rated separately for each side roband sits comfortably. If necessary, support of feet and trunk is allowed. Proband is asked to perform 10 cycles of repetitive alternation of pro- and supinations of possible. Movement is demonstrated by examiner at a speed of approx. 10 cycles within 7 s. Exact times for movement execution have to be taken. Score Left: ___________ 4 N/A
PX220101_Ataxia_Fast_Alternating_Hand_Movement_Right PX220101070100 Rated separately for each side roband sits comfortably. If necessary, support of feet and trunk is allowed. Proband is asked to perform 10 cycles of repetitive alternation of pro- and supinations of possible. Movement is demonstrated by examiner at a speed of approx. 10 cycles within 7 s. Exact times for movement execution have to be taken. Score Right: ___________ 4 N/A
PX220101_Ataxia_Finger_Chase_Left_Side PX220101050200 Rated separately for each side Proband sits comfortably. If necessary, support of feet and trunk is allowed. Examiner sits in front of proband and performs 5 consecutive sudden and fast pointing movements in unpredictable directions in a frontal plane, at about 50 % of proband's reach. Movements have an amplitude of 30 cm and a frequency of 1 movement every 2 s. Proband is asked to follow the movements with his index finger, as fast and precisely as possible. Average performance of last 3 movements is rated. Score Left: ____________ 4 N/A
PX220101_Ataxia_Finger_Chase_Mean_Both_Sides PX220101050300 Mean of both sides (R+L)/2:______________ 4 N/A
PX220101_Ataxia_Finger_Chase_Right_Side PX220101050100 Rated separately for each side Proband sits comfortably. If necessary, support of feet and trunk is allowed. Examiner sits in front of proband and performs 5 consecutive sudden and fast pointing movements in unpredictable directions in a frontal plane, at about 50 % of proband's reach. Movements have an amplitude of 30 cm and a frequency of 1 movement every 2 s. Proband is asked to follow the movements with his index finger, as fast and precisely as possible. Average performance of last 3 movements is rated. Score Right ___________ 4 N/A
PX220101_Ataxia_Heel_Shin_Slide_Left PX220101080200 Rated separately for each side roband lies on examination bed, without sight of his legs. Proband is asked to lift one leg, point with the heel to the opposite knee, slide down along the shin to the ankle, and lay the leg back on the examination bed. The task is performed 3 times. Slide-down movements should be performed within 1 s. If proband slides down without contact to shin in all three trials, rate 4. Score Left:_____________ 4 N/A
PX220101_Ataxia_Heel_Shin_Slide_Mean PX220101080300 Mean of both sides (R+L)/2:______________ 4 N/A
PX220101_Ataxia_Heel_Shin_Slide_Right PX220101080100 Rated separately for each side roband lies on examination bed, without sight of his legs. Proband is asked to lift one leg, point with the heel to the opposite knee, slide down along the shin to the ankle, and lay the leg back on the examination bed. The task is performed 3 times. Slide-down movements should be performed within 1 s. If proband slides down without contact to shin in all three trials, rate 4. Score Right:_____________ 4 N/A
PX220101_Ataxia_Nose_Finger_Left_Side PX220101060200 Rated separately for each side Proband sits comfortably. If necessary, support of feet and trunk is allowed. Proband is asked to point repeatedly with his index finger from his nose to examiner's finger which is in front of the proband at about 90 % of proband's reach. Movements are performed at moderate speed. Average performance of movements is rated according to the amplitude of the kinetic tremor. Score Left: _______________ 4 N/A
PX220101_Ataxia_Nose_Finger_Mean_Both_Sides PX220101060300 Mean of both sides (R+L)/2:______________ 4 N/A
PX220101_Ataxia_Nose_Finger_Right_Side PX220101060100 Rated separately for each side Proband sits comfortably. If necessary, support of feet and trunk is allowed. Proband is asked to point repeatedly with his index finger from his nose to examiner's finger which is in front of the proband at about 90 % of proband's reach. Movements are performed at moderate speed. Average performance of movements is rated according to the amplitude of the kinetic tremor. Score Right: ______________ 4 N/A
PX220101_Ataxia_Proband_Gait PX220101010000 Proband is asked (1) to walk at a safe distance parallel to a wall including a half-turn (turn around to face the opposite direction of gait) and (2) to walk in tandem (heels to toes) without support. Score _______- 4 N/A
PX220101_Ataxia_Proband_Sitting PX220101030000 Proband is asked to sit on an examination bed without support of feet, eyes open and arms outstretched to the front. Score _____________ 4 N/A
PX220101_Ataxia_Proband_Stance PX220101020000 Proband is asked to stand (1) in natural position, (2) with feet together in parallel (big toes touching each other) and (3) in tandem (both feet on one line, no space between heel and toe). Proband does not wear shoes, eyes are open. For each condition, three trials are allowed. Best trial is rated. Score __________ 4 N/A
PX220101_Ataxia_Speech_Disturbance PX220101040000 Speech is assessed during normal conversation. 4 N/A
PX220101_Ataxia_Total_Score PX220101090000 Mean values from each one of the eight items are summed to obtain the total score. The total scores range from 0 (no ataxia) to 40 (severe ataxia). 4 N/A
Research Domain Information
Measure Name:

Ataxia Rating Scale

Release Date:

April 30, 2015

Definition

The Ataxia Rating Scale is used to assess the severity of cerebellar ataxia.

Purpose

Ataxia is a symptom of several rare neurologic conditions. Disorders that affect the cerebellum cause most genetic forms of ataxia. Ataxia is exhibited by uncoordinated body movements, an unsteady gait, and poor balance.

Keywords

Ataxia, cerebellar ataxia, neurological, gait, scale, Scale for the Assessment and Rating of Ataxia, SARA