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Protocol - Ataxia Rating Scale

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Protocol Name from Source:

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

Availability:

Publicly available

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.)

Personnel and Training Required

Trained clinician must administer the protocol

Equipment Needs

Examination bed

Requirements

Requirement CategoryRequired
Average time of greater than 15 minutes in an unaffected individualNo
Major equipmentNo
Specialized requirements for biospecimen collectionNo
Specialized trainingYes

Mode of Administration

Self-administered

Life Stage:

Child, Adolescent, Adult

Specific Instructions:

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

Research Domain Information

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.

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 Scale4797993CDE Browser

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_Proband_GaitPX220101010000Proband 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 _______-4N/A
PX220101_Ataxia_Proband_StancePX220101020000Proband 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 __________4N/A
PX220101_Ataxia_Proband_SittingPX220101030000Proband is asked to sit on an examination bed without support of feet, eyes open and arms outstretched to the front. Score _____________4N/A
PX220101_Ataxia_Speech_DisturbancePX220101040000Speech is assessed during normal conversation.4N/A
PX220101_Ataxia_Finger_Chase_Right_SidePX220101050100Rated 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 ___________4N/A
PX220101_Ataxia_Finger_Chase_Left_SidePX220101050200Rated 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: ____________4N/A
PX220101_Ataxia_Finger_Chase_Mean_Both_SidesPX220101050300Mean of both sides (R+L)/2:______________4N/A
PX220101_Ataxia_Nose_Finger_Right_SidePX220101060100Rated 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: ______________4N/A
PX220101_Ataxia_Nose_Finger_Left_SidePX220101060200Rated 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: _______________4N/A
PX220101_Ataxia_Nose_Finger_Mean_Both_SidesPX220101060300Mean of both sides (R+L)/2:______________4N/A
PX220101_Ataxia_Fast_Alternating_Hand_Movement_RightPX220101070100Rated 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: ___________4N/A
PX220101_Ataxia_Fast_Alternating_Hand_Movement_LeftPX220101070200Rated 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: ___________4N/A
PX220101_Ataxia_Fast_Alternating_HandMovement_MeanPX220101070300Mean of both sides (R+L)/2:______________4N/A
PX220101_Ataxia_Heel_Shin_Slide_RightPX220101080100Rated 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:_____________4N/A
PX220101_Ataxia_Heel_Shin_Slide_LeftPX220101080200Rated 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:_____________4N/A
PX220101_Ataxia_Heel_Shin_Slide_MeanPX220101080300Mean of both sides (R+L)/2:______________4N/A
PX220101_Ataxia_Total_ScorePX220101090000Mean 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).4N/A