CDE Detailed Report

Disease: Friedreich's Ataxia
Sub-Domain: Ataxia and Performance Measures
CRF: Friedreich's Ataxia Rating Scale (FARS)

Displaying 1 - 50 of 90
CDE ID CDE Name Variable Name Definition Short Description Question Text Permissible Values Description Data Type Disease Specific Instructions Disease Specific Reference Population Classification (e.g., Core) Version Number Version Date CRF Name (CRF Module / Guideline) Sub Domain Name Domain Name Size Input Restrictions Min Value Max Value Measurement Type External Id Loinc External Id Snomed External Id caDSR External Id CDISC
C11808 Friedreich's Ataxia Rating Scale (FARS) - instrumental testing 10 second PATA trial 1 count FARSInstrTest10SecPATATry1Ct Count of times participant/subject is able to repeat word "pata" in 10-sec period for trial 1 of the PATA Rate test of the instrumental testing section, as part of the Friedreich's Ataxia Rating Scale (FARS). Count of times participant/subject is able to repeat word "pata" in 10-sec period for trial 1 of the PATA Rate test of the instrumental testing section, as part of the Friedreich's Ataxia Rating Scale (FARS). Trial 1 Numeric Values

Use a tape recorder that can play at slow and fast speeds (1.2 & 2.4 cm/sec). Record at normal (2.4) speed. Use a digital stopwatch. Patient seated comfortably and instructed to repeat the syllable "pata" as quickly and distinctly as possible for 10 seconds until told to stop. Start recorder and record patient's name and date. Preset stopwatch for 10 seconds. Say "go" and as soon as patient starts speaking, start timer. Say "stop" when timer beeps at end of 10 seconds. Count # of "patas" for each 10 seconds, using playback at slower speed.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Free-Form Entry

C11744 Friedreich's Ataxia Rating Scale (FARS) - daily activities falling score FARSDailyActivsFallingScore Falling score for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Falling score for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Falling 0;0.5;1;1.5;2;2.5;3;3.5;4 0 - Normal;;1 - Rare falling (< once a month);;2 - Occasional falls (once a week to once a month);;3 - Falls multiple times a week or requires device to prevent falls;;4 - Unable to stand or walk Numeric Values

Increments of 0.5 may be used if strongly felt that a task falls between 2 scores. Assistive device = score 3.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11776 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral nerve system vibratory sense right score FARSPNSNeuExVibratSenRtScore Score for right-side vibratory sense in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for right-side vibratory sense in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Right 0;1;2 0 - Normal;1 - Impaired at toes;2 - Impaired at toes or fingers Numeric Values

Abnormal

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C17525 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral deep tendon reflex biceps jerk left score FARSPNSNeuExDTenRefBicJLftScr Score for left-side biceps jerk in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for left-side biceps jerk in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). BJ 0;1;2;3;4 0 - absent;1 - hyporeflexia;2 - normal;3 - hyperreflexia;4 - pathologic hyperreflexia Numeric Values Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Supplemental 3.00 2013-07-15 16:08:48.687 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11755 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upper limb coordination finger-finger test left score FARSNeuExULCFingrFingrLftScore The score for the left finger in the finger-to-finger test in the upper limb coordination section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). The score for the left finger in the finger-to-finger test of upper limb coordination, as part of the Friedreich's Ataxia Rating Scale (FARS). Finger to Finger Test 0;1;2;3 0 - Normal;1 - Mild oscillations of finger (< 2 cm);2 - Moderate oscillations of finger (2-6 cm);3 - Severe oscillations of finger (> 6 cm) Numeric Values

The index fingers are placed in front of each other with flexion at the elbow about 25 cm from the sternum. Observe for 10 seconds. Score amplitude of oscillations

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11787 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upright stability stance feet apart trial 2 duration FARSNEUprStbStncFtAprtTry2Dur Duration in seconds of feet-apart stance trial 2 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Duration in seconds of feet-apart stance trial 2 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Trial 2 0;1;2;3;4 0 - 1 minute or longer;1 - <1 minute, >45 sec;2 - <45 sec, >30 sec;3 - <30 sec, >15 sec;4 - <15 sec or needs hands held by assistant/device Numeric Values

If a patient can stand in a particular position for 1 minute or longer in trial 1, trials 2 and 3 are abandoned.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

second
C11766 Friedreich's Ataxia Rating Scale (FARS) - neurological exam lower limb coordination heel shin slide test right score FARSNeuExLLCShinSlidTesRtScore Score for the right heel in the heel-along-shin slide test in the lower limb coordination section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for the right heel in the heel-along-shin slide test in the lower limb coordination section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Heel Along Shin Slide 0;1;2;3;4 0 - Normal (stay on shin);1 - Mild (abnormally slow, tremulous but contact maintained);2 - Moderate (goes off shin a total of 3 or fewer times during 3 cycles);3 - Severe (goes off shin 4 or more times during 3 cycles);4 - Too poorly coordinated to attempt the task Numeric Values

Under visual control, slide heel on the contralateral tibia from the patella to the ankle, up and down, 3 cycles at moderate speed, 2 sec/cycle, one at a time.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11798 Friedreich's Ataxia Rating Scale (FARS) - neurological exam dominant foot stance trial 1 duration FARSNEDomFtStanceTry1Dur Duration in seconds of dominant-foot stance trial 1 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Duration in seconds of dominant-foot stance trial 1 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Trial 1 0;1;2;3;4 0 - 1 minute or longer;1 - <1 minute, >45 sec;2 - <45 sec, >30 sec;3 - <30 sec, >15 sec;4 - <15 sec or needs hands held by assistant/device Numeric Values

For one foot stance, the patient is asked to stand on dominant foot and the other leg is elevated by bringing it forward with knee extended; this gives some advantage to the patient. Stance and gait tests may be done barefoot if patient does not have appropriate footwear, however, it should be done the same way for serial measurement.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

second
C11809 Friedreich's Ataxia Rating Scale (FARS) - instrumental testing 10 second PATA trial 2 count FARSInstrTest10SecPATATry2Ct Count of times participant/subject is able to repeat word "pata" in 10-sec period for trial 2 of the PATA Rate test of the instrumental testing section, as part of the Friedreich's Ataxia Rating Scale (FARS). Count of times participant/subject is able to repeat word "pata" in 10-sec period for trial 2 of the PATA Rate test of the instrumental testing section, as part of the Friedreich's Ataxia Rating Scale (FARS). Trial 2 Numeric Values

Use a tape recorder that can play at slow and fast speeds (1.2 & 2.4 cm/sec). Record at normal (2.4) speed. Use a digital stopwatch. Patient seated comfortably and instructed to repeat the syllable "pata" as quickly and distinctly as possible for 10 seconds until told to stop. Start recorder and record patient's name and date. Preset stopwatch for 10 seconds. Say "go" and as soon as patient starts speaking, start timer. Say "stop" when timer beeps at end of 10 seconds. Count # of "patas" for each 10 seconds, using playback at slower speed.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Free-Form Entry

C11745 Friedreich's Ataxia Rating Scale (FARS) - daily activities walking score FARSDailyActivsWalkingScore Walking score for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Walking score for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Walking 0;0.5;1;1.5;2;2.5;3;3.5;4 0 - Normal;;1 - Mild difficulty, perception of imbalance;;2 - Moderate difficulty, but requires little or no assistance;;3 - Severe disturbance of walking, requires assistance or walking aids;;4 - Cannot walk at all even with assistance (wheelchair bound) Numeric Values

Increments of 0.5 may be used if strongly felt that a task falls between 2 scores. Assistive device = score 3.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11777 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral nerve system vibratory sense left score FARSPNSNeuExVibratSenseLftScr Score for left-side vibratory sense in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for left-side vibratory sense in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Left 0;1;2 0 - Normal;1 - Impaired at toes;2 - Impaired at toes or fingers Numeric Values

Abnormal

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C17526 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral deep tendon reflex biceps jerk right score FARSPNSNeuExDTenRefBicJRtScore Score for right-side biceps jerk in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for right-side biceps jerk in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). BJ 0;1;2;3;4 0 - absent;1 - hyporeflexia;2 - normal;3 - hyperreflexia;4 - pathologic hyperreflexia Numeric Values Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Supplemental 3.00 2013-07-15 16:08:48.687 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11756 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upper limb coordination nose-finger test right score FARSNeuExULCNoseFingrRtScore The score for the right finger in the nose-to-finger test in the upper limb coordination section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). The score for the right finger in the nose-to-finger test of upper limb coordination, as part of the Friedreich's Ataxia Rating Scale (FARS). Nose-Finger Test 0;1;2;3;4 0 - None;1 - Mild (< 2 cm amplitude);2 - Moderate (2-4 cm amplitude or persisting through movement);3 - Severe (> 6 cm & persisting through movement);4 - Too poorly coordinated to perform task Numeric Values

Assess kinetic or intention tremor during and towards the end of movement: examiner holds index finger at 90% reach of patient; test at least 3 nose-finger-nose trials; movement slow > 3 sec.:

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11788 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upright stability stance feet apart trial 3 duration FARSNEUprStbStncFtAprtTry3Dur Duration in seconds of feet-apart stance trial 3 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Duration in seconds of feet-apart stance trial 3 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Trial 3 0;1;2;3;4 0 - 1 minute or longer;1 - <1 minute, >45 sec;2 - <45 sec, >30 sec;3 - <30 sec, >15 sec;4 - <15 sec or needs hands held by assistant/device Numeric Values

If a patient can stand in a particular position for 1 minute or longer in trial 1, trials 2 and 3 are abandoned.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

second
C11767 Friedreich's Ataxia Rating Scale (FARS) - neurological exam lower limb coordination heel shin slide test left score FARSNeuExULCSlideLftScore Score for the left heel in the heel-along-shin slide test in the lower limb coordination section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS) Score for the left heel in the heel-along-shin slide test in the lower limb coordination section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS) Heel Along Shin Slide 0;1;2;3;4 0 - Normal (stay on shin);1 - Mild (abnormally slow, tremulous but contact maintained);2 - Moderate (goes off shin a total of 3 or fewer times during 3 cycles);3 - Severe (goes off shin 4 or more times during 3 cycles);4 - Too poorly coordinated to attempt the task Numeric Values

Under visual control, slide heel on the contralateral tibia from the patella to the ankle, up and down, 3 cycles at moderate speed, 2 sec/cycle, one at a time.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11799 Friedreich's Ataxia Rating Scale (FARS) - neurological exam dominant foot stance trial 2 duration FARSNEDomFtStanceTry2Dur Duration in seconds of dominant-foot stance trial 2 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Duration in seconds of dominant-foot stance trial 2 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Trial 2 0;1;2;3;4 0 - 1 minute or longer;1 - <1 minute, >45 sec;2 - <45 sec, >30 sec;3 - <30 sec, >15 sec;4 - <15 sec or needs hands held by assistant/device Numeric Values

If a patient can stand in a particular position for 1 minute or longer in trial 1, trials 2 and 3 are abandoned.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

second
C11810 Friedreich's Ataxia Rating Scale (FARS) - instrumental testing 10 second PATA mean count value FARSInstrTest10SecPataMeanVal Mean count of times participant/subject is able to repeat word "pata" in 10-sec period over 2 trials of the PATA Rate test of the instrumental testing section, as part of the Friedreich's Ataxia Rating Scale (FARS). Mean count of times participant/subject is able to repeat word "pata" in 10-sec period over 2 trials of the PATA Rate test of the instrumental testing section, as part of the Friedreich's Ataxia Rating Scale (FARS). AVG Numeric Values

Refer to instructions on form.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Free-Form Entry

C11746 Friedreich's Ataxia Rating Scale (FARS) - daily activities sitting position quality score FARSDailyActivsSitQualtyScore Quality-of-sitting-position score for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Quality-of-sitting-position score for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Quality of Sitting Position 0;0.5;1;1.5;2;2.5;3;3.5;4 0 - Normal;;1 - Slight imbalance of the trunk, but needs no back support;;2 - Unable to sit without back support;;3 - Can sit only with extensive support (Geriatric chair, posy, etc.);;4 - Unable to sit Numeric Values

Increments of 0.5 may be used if strongly felt that a task falls between 2 scores.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11778 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral nerve system position sense right score FARSPNSNeuExPositnSenseRtScore Score for right-side position sense in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for right-side position sense in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Position Sense 0;1;2 0 - Normal;1 - Impaired at toes or fingers;2 - Impaired at toes and fingers Numeric Values

Test using minimal random movement of distal interphalangeal joints of index finger and big toe.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C17527 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral deep tendon reflex brachioradialis jerk left score FARSPNSNeuExDTenRefBrJLftScore Score for left-side brachioradialis jerk in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for left-side brachioradialis jerk in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). BrJ 0;1;2;3;4 0 - absent;1 - hyporeflexia;2 - normal;3 - hyperreflexia;4 - pathologic hyperreflexia Numeric Values Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Supplemental 3.00 2013-07-15 16:08:48.687 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11757 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upper limb coordination nose-finger test left score FARSNeuExULCNoseFingrLftScore The score for the left finger in the nose-to-finger test in the upper limb coordination section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). The score for the left finger in the nose-to-finger test of upper limb coordination, as part of the Friedreich's Ataxia Rating Scale (FARS). Nose-Finger Test 0;1;2;3;4 0 - None;1 - Mild (< 2 cm amplitude);2 - Moderate (2-4 cm amplitude or persisting through movement);3 - Severe (> 6 cm & persisting through movement);4 - Too poorly coordinated to perform task Numeric Values

Assess kinetic or intention tremor during and towards the end of movement: examiner holds index finger at 90% reach of patient; test at least 3 nose-finger-nose trials; movement slow > 3 sec.:

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11789 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upright stability stance feet apart mean duration score FARSUprtStabltyStncFtApartScr Score for mean duration of feet-apart stance trials 1–3 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for mean duration of feet-apart stance trials 1–3 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Avg 0;1;2;3;4 0 - 1 minute or longer;1 - <1 minute, >45 sec;2 - <45 sec, >30 sec;3 - <30 sec, >15 sec;4 - <15 sec or needs hands held by assistant/device Numeric Values

If a patient can stand in a particular position for 1 minute or longer in trial 1, trials 2 and 3 are abandoned. Otherwise each of 3 trials is timed and then averaged. Grading scores are then given as noted.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11800 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upright stability dominant foot stance trial 3 duration FARSNEUpStbltyDomFtStanTry3Dur Duration in seconds of dominant-foot stance trial 3 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Duration in seconds of dominant-foot stance trial 3 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Trial 3 0;1;2;3;4 0 - 1 minute or longer;1 - <1 minute, >45 sec;2 - <45 sec, >30 sec;3 - <30 sec, >15 sec;4 - <15 sec or needs hands held by assistant/device Numeric Values

If a patient can stand in a particular position for 1 minute or longer in trial 1, trials 2 and 3 are abandoned.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

second
C11768 Friedreich's Ataxia Rating Scale (FARS) - neurological exam lower limb coordination heel shin tap test position indicator FARSNeuExLLCHeelShTapTesPosInd Indicator of the participant/subject's position while performing heel-to-shin tap test in the lower limb coordination section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS) Indicator of the participant/subject's position while performing heel-to-shin tap test in the lower limb coordination section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS) Supine|Seated Supine;Seated Supine;Seated Alphanumeric

May be seated with contralateral leg extended or supine but perform same way each time.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11811 Friedreich's Ataxia Rating Scale (FARS) - instrumental testing 9 hole peg test right trial 1 duration FARSInstrTest9HPTRtTry1Dur Duration in seconds for participant/subject to complete right-hand trial 1 of the 9-hole peg test in the instrumental testing section, as part of the Friedreich's Ataxia Rating Scale (FARS). Duration in seconds for participant/subject to complete right-hand trial 1 of the 9-hole peg test in the instrumental testing section, as part of the Friedreich's Ataxia Rating Scale (FARS). Trial 1 Numeric Values

Make sure the stopwatch is set to zero. Introduce this section by saying, "Now, we're going to be measuring your arm and hand function." If this is the first visit, as, "Are you right- or left-handed?" Make a note of the dominant hand for subsequent instructions. Place the 9-HPT apparatus on the table directly in front of the patient. Arrange the apparatus so that the side with the pegs is in front of the hand being tested and the side with the empty pegboard is in front of the hand not being tested. Secure with Dycem. Read the following instructions to the patient: "On this test, I want you to pick up the pegs one at a time, using one hand only, and put them into the holes as quickly as you can in any order until all the holes are filled. Then, without pausing, remove the pegs one at a time and return them to the container as quickly as you can. We'll have you do this two (2) times with each hand. We'll start with your [DOMINANT] hand. You can hold the peg board steady with your [NON-DOMINANT] hand. If a peg falls onto the table, you retrieve it and continue with the task. If a peg falls on the floor, keep working on the task and I will retrieve it for you. See how fast you can put all the pegs in and take them out again. Are you ready? Begin." Start timing as soon as the patient touches the first peg, and stop timing when the last peg hits the container. If a peg drops on the floor, the examiner will retrieve it and put it back in the peg box. However, if a peg drops onto the table, the patient is to retrieve it unless it is beyond their arm reach; then you can retrieve it for them. It is possible that a peg may fall beyond the reach of the examiner; therefore we recommend that you keep a few extra pegs in hand so that testing is not interrupted. Do not put extra pegs in the testing apparatus as this may confuse the subject. Record the patient's time under "Dominant hand -- Trial 1." If the subject stops after having put all the pegs into the holes, prompt the subject to move them as well by saying, "And now remove them all." If the subject begins to remove more than one peg at a time, correct him/her by saying, "Pick up one peg at a time." The total time to complete the task is recorded in seconds including one decimal place, rounded as needed. Round up to the next tenth if hundredths place is > = .05, round down if hundredths place is

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Free-Form Entry

second
C11747 Friedreich's Ataxia Rating Scale (FARS) - daily activities bladder function score FARSDailyActivsBladrFuncScore Bladder function score for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Bladder function score for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Bladder Function 0;0.5;1;1.5;2;2.5;3;3.5;4 0 - Normal;;1 - Mild urinary hesitance, urgency or retention (< once a month);;2 - Moderate hesitance, urgency, rare retention/incontinence (> once a month, but < once a week);;3 - Frequent urinary incontinence (> once a week);;4 - Loss of bladder function requiring intermittent catheterization/indwelling catheter Numeric Values

Increments of 0.5 may be used if strongly felt that a task falls between 2 scores. If using drugs for bladder, automatic score of 3.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11779 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral nerve system position sense left score FARSPrphrlNrvPosSenseLftScore Score for left-side position sense in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for left-side position sense in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Position Sense 0;1;2 0 - Normal;1 - Impaired at toes or fingers;2 - Impaired at toes and fingers Numeric Values

Test using minimal random movement of distal interphalangeal joints of index finger and big toe.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C17528 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral deep tendon reflex brachioradialis jerk right score FARSPNSNeuExDTenRefBrJRtScore Score for right-side brachioradialis jerk in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for right-side brachioradialis jerk in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). BrJ 0;1;2;3;4 0 - absent;1 - hyporeflexia;2 - normal;3 - hyperreflexia;4 - pathologic hyperreflexia Numeric Values Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Supplemental 3.00 2013-07-15 16:08:48.687 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11758 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upper limb coordination dysmetria right score FARSNeuExULCDysmetriaRtScore The score for the right finger in the dysmetria (fast nose-finger) test in the upper limb coordination section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). The score for the right finger in the dysmetria (fast nose-finger) test of upper limb coordination, as part of the Friedreich's Ataxia Rating Scale (FARS). Dysmetria (Fast Nose-Finger) Test 0;1;2;3;4 0 - None;1 - Mild (misses 2 or fewer times);2 - Moderate (misses 3-5 times);3 - Severe (misses 6-8 times);4 - Too poorly coordinated to perform task Numeric Values

Assess dysmetria: The patient touches tip of examiner's finger 8 times as rapidly as possible while the examiner moves his finger and stops at different locations at about 90% reach of the patient. Assess dysmetria—i.e., inaccuracy of reaching the target—at examiner's finger.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11790 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upright stability feet together stance trial 1 duration FARSUpStbltyStncFtTgthrTry1Dur Duration in seconds of feet-together stance trial 1 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Duration in seconds of feet-together stance trial 1 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Trial 1 0;1;2;3;4 0 - 1 minute or longer;1 - <1 minute, >45 sec;2 - <45 sec, >30 sec;3 - <30 sec, >15 sec;4 - <15 sec or needs hands held by assistant/device Numeric Values

For feet together the entire inside of the feet should be close together as much as possible. Stance and gait tests may be done barefoot if patient does not have appropriate footwear, however, it should be done the same way for serial measurement. Inside of feet 20 cm apart marked on floor. Use stopwatch; 3 attempts; time in seconds.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

second
C11801 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upright stability dominant foot stance mean duration score FARSNEUpStbltyStancDomFtScore Score for mean duration of dominant-foot stance trials 1–3 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for mean duration of dominant-foot stance trials 1–3 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Avg 0;1;2;3;4 0 - 1 minute or longer;1 - <1 minute, >45 sec;2 - <45 sec, >30 sec;3 - <30 sec, >15 sec;4 - <15 sec or needs hands held by assistant/device Numeric Values

If a patient can stand in a particular position for 1 minute or longer in trial 1, trials 2 and 3 are abandoned. Otherwise each of 3 trials is timed and then averaged. Grading scores are then given as noted.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11769 Friedreich's Ataxia Rating Scale (FARS) - neurological exam lower limb coordination heel shin tap test right score FARSNeuExLLCHeelShTapTeRtScore Score for the right heel in the heel-to-shin tap test in the lower limb coordination section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS) Score for the right heel in the heel-to-shin tap test in the lower limb coordination section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS) Heel-to-Shin Tap 0;1;2;3;4 0 - Normal (stays on target);1 - Mild (misses shin 2 or < times);2 - Moderate (misses shin 3-5 times);3 - Severe (misses shin > 4 times);4 - Too poorly coordinated to perform task. Numeric Values

Patient taps heel on midpoint of contralateral shin 8 times on each side from about 6-10", one at a time.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11812 Friedreich's Ataxia Rating Scale (FARS) - instrumental testing 9 hole peg test right trial 2 duration FARSInstrTest9HPTRtTry2Dur Duration in seconds for participant/subject to complete right-hand trial 2 of the 9-hole peg test in the instrumental testing section, as part of the Friedreich's Ataxia Rating Scale (FARS). Duration in seconds for participant/subject to complete right-hand trial 2 of the 9-hole peg test in the instrumental testing section, as part of the Friedreich's Ataxia Rating Scale (FARS). Trial 2 Numeric Values

Make sure the stopwatch is set to zero. Start timing as soon as the patient touches the first peg, and stop timing when the last peg hits the container. The total time to complete the task is recorded in seconds including one decimal place, rounded as needed. Round up to the next tenth if hundredths place is > = .05, round down if hundredths place is

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Free-Form Entry

second
C11748 Friedreich's Ataxia Rating Scale (FARS) - daily activities total value FARSDailyActivtyTotalVal The sum of the scores recorded for Part 2: Activities of Daily Living section, as part of the Friedreich's Ataxia Rating Scale (FARS). The sum of the scores recorded for Part 2: Activities of Daily Living section, as part of the Friedreich's Ataxia Rating Scale (FARS). Total Activities of Daily Living Score: 0;0.5;1;1.5;2;2.5;3;3.5;4;4.5;5;5.5;6;6.5;7;7.5;8;8.5;9;9.5;10;10.5;11;11.5;12;12.5;13;13.5;14;14.5;15;15.5;16;16.5;17;17.5;18;18.5;19;19.5;20;20.5;21;21.5;22;22.5;23;23.5;24;24.5;25;25.5;26;26.5;27;27.5;28;28.5;29;29.5;30;30.5;31;31.5;32;32.5;33;33.5;34;34.5;35;35.5;36 0;0.5;1;1.5;2;2.5;3;3.5;4;4.5;5;5.5;6;6.5;7;7.5;8;8.5;9;9.5;10;10.5;11;11.5;12;12.5;13;13.5;14;14.5;15;15.5;16;16.5;17;17.5;18;18.5;19;19.5;20;20.5;21;21.5;22;22.5;23;23.5;24;24.5;25;25.5;26;26.5;27;27.5;28;28.5;29;29.5;30;30.5;31;31.5;32;32.5;33;33.5;34;34.5;35;35.5;36 Numeric Values

Enter numeric value for score of 0-36. Refer to instructions on form.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11780 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral deep tendon reflex right score FARSNeurExPrphrlNrvDTRRtScore Score for right-side deep tendon reflex in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for right-side deep tendon reflex in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). DTR 0;1;2 0 - No areflexia;1 - Areflexia in either upper or lower limbs;2 - Generalized areflexia. Numeric Values

Refer to instructions on form.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C17529 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral deep tendon reflex knee jerk left score FARSPNSNeuExDTenRKneeJLtScore Score for left-side knee jerk in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for left-side knee jerk in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). KJ 0;1;2;3;4 0 - absent;1 - hyporeflexia;2 - normal;3 - hyperreflexia;4 - pathologic hyperreflexia Numeric Values Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Supplemental 3.00 2013-07-15 16:08:48.687 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11759 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upper limb coordination dysmetria left score FARSNeuExULCDysmetriaLftScore The score for the left finger in the dysmetria (fast nose-finger) test in the upper limb coordination section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). The score for the left finger in the dysmetria (fast nose-finger) test of upper limb coordination, as part of the Friedreich's Ataxia Rating Scale (FARS). Dysmetria (Fast Nose-Finger) Test 0;1;2;3;4 0 - None;1 - Mild (misses 2 or fewer times);2 - Moderate (misses 3-5 times);3 - Severe (misses 6-8 times);4 - Too poorly coordinated to perform task Numeric Values

Assess dysmetria: The patient touches tip of examiner's finger 8 times as rapidly as possible while the examiner moves his finger and stops at different locations at about 90% reach of the patient. Assess dysmetria—i.e., inaccuracy of reaching the target—at examiner's finger.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11791 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upright stability feet together stance trial 2 duration FARSNEUpStbStncFtTgthrTry2Dur Duration in seconds of feet-together stance trial 2 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Duration in seconds of feet-together stance trial 2 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Trial 2 0;1;2;3;4 0 - 1 minute or longer;1 - <1 minute, >45 sec;2 - <45 sec, >30 sec;3 - <30 sec, >15 sec;4 - <15 sec or needs hands held by assistant/device Numeric Values

If a patient can stand in a particular position for 1 minute or longer in trial 1, trials 2 and 3 are abandoned.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

second
C11802 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upright stability tandem walk score FARSNEUprtStabilityTandmWlkScr Score for tandem walk in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for tandem walk in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Tandem Walk 0;1;2;3 0 - Normal (able to tandem walk >8 sequential steps);1 - Able to tandem walk in < perfect manner/can tandem walk >4 sequential steps, but <8;2 - Can tandem walk, but fewer than 4 steps before losing balance;3 - Too poorly coordinated to attempt task. Numeric Values

Tandem walk and gait are performed in a hallway. Preferably no carpet but at least serial examinations should be on the same surface. Stance and gait tests may be done barefoot if patient does not have appropriate footwear, however, it should be done the same way for serial measurement. Tandem walk 10 steps in straight line; performed in hallway with no furniture within reach (1 m / 3 ft) and no loose carpet.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11738 Friedreich's Ataxia Rating Scale (FARS) - functional staging ataxia score FARSFunctnlStageAtaxScore Functional staging for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Functional staging for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Functional staging for ataxia: Stage 0;0.5;1;1.5;2;2.5;3;3.5;4;4.5;5;5.5;6 Stage 0: Normal;;Stage 1.0: Minimal signs detected by physician during screening. Can run or jump without loss of balance. No disability;;Stage 2.0: Symptoms present, recognized by patient, but still mild. Cannot run or jump without losing balance. The patient is physically capable of leading an independent life, but daily activities may be somewhat restricted. Minimal disability;;Stage 3.0: Symptoms are overt and significant. Requires regular or periodic holding onto wall/furniture or use of a cane for stability and walking. Mild disability. (Note: many patients postpone obtaining a cane by avoiding open spaces and walking with the aid of walls/people etc. These patients are graded as stage 3.0);;Stage 4.0: Walking requires a walker, Canadian crutches or two canes, or other aids such as walking dogs. Can perform several activities of daily living. Moderate disability;;Stage 5.0: Confined but can navigate a wheelchair. Can perform some activities of daily living that do not require standing or walking. Severe disability;;Stage 6.0: Confined to wheelchair or bed with total dependency for all activities of daily living. Total disability Numeric Values

Increments of 0.5 may be used if the status is about the middle between two stages.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Exploratory 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11770 Friedreich's Ataxia Rating Scale (FARS) - neurological exam lower limb coordination heel shin tap test left score FARSNeuExLLCShinTapLftScore Score for the left heel in the heel-to-shin tap test in the lower limb coordination section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS) Score for the left heel in the heel-to-shin tap test in the lower limb coordination section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS) Heel-to-Shin Tap 0;1;2;3;4 0 - Normal (stays on target);1 - Mild (misses shin 2 or < times);2 - Moderate (misses shin 3-5 times);3 - Severe (misses shin > 4 times);4 - Too poorly coordinated to perform task. Numeric Values

Patient taps heel on midpoint of contralateral shin 8 times on each side from about 6-10", one at a time.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11813 Friedreich's Ataxia Rating Scale (FARS) - instrumental testing 9 hole peg test right mean duration value FARSInstrTest9HPTRtMeanDurVal Mean duration in seconds for right-hand trials 1 and 2 of the 9-hole peg test in the instrumental testing section, as part of the Friedreich's Ataxia Rating Scale (FARS). Mean duration in seconds for right-hand trials 1 and 2 of the 9-hole peg test in the instrumental testing section, as part of the Friedreich's Ataxia Rating Scale (FARS). AVG Numeric Values

Refer to instructions on form.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Free-Form Entry

second
C11749 Friedreich's Ataxia Rating Scale (FARS) - neurological exam bulbar facial atrophy fasciculation myoclonus weakness score FARSNEBulbrFacAtrFasMyWeaScore The score for severity of facial atrophy, fasciculation, action myoclonus, and weakness in the bulbar portion of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). The score for severity of facial atrophy, fasciculation, action myoclonus, and weakness in the bulbar portion of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Facial Atrophy, Fasciculation, Action Myoclonus, and Weakness: 0;0.5;1;1.5;2;2.5;3 0 - None;;1 - Fasciculations or action myoclonus, but no atrophy;;2 - Atrophy present but not profound or complete;;3 - Profound atrophy and weakness Numeric Values

Rate each item on the basis of the patient status during examination. To the extent possible, sequential patient examinations should be carried out at the same time of the day. If the patient is taking any medication, the examination should be carried out prior to dosing, or at a fixed time following the dosing based on the maximum expected therapeutic response. Increments of 0.5 may be used if examiner feels an item falls between 2 defined severities.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11781 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral deep tendon reflex left score FARSNeurExPrphrlNrvDTRLftScore Score for left-side deep tendon reflex in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for left-side deep tendon reflex in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). DTR 0;1;2 0 - No areflexia;1 - Areflexia in either upper or lower limbs;2 - Generalized areflexia. Numeric Values

Refer to instructions on form.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C17531 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral nerve system vibratory sense finger left duration FARSPNSNeuExVibSenFngrLftDur Delay in seconds before participant/subject feels vibration in the left-hand fingers, as part of the Friedreich's Ataxia Rating Scale (FARS). Delay in seconds before participant/subject feels vibration in the left-hand fingers, as part of the Friedreich's Ataxia Rating Scale (FARS). Time felt for fingers Numeric Values

Educate patient regarding the sensation. Tested with 128 cps tuning fork set to near full vibration; eyes closed; test over index finger and great toe.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Supplemental 3.00 2013-07-15 16:08:48.687 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Free-Form Entry

second
C11760 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upper limb coordination rapid alternate hand movement right score FARSNeuExULCRapdAltHandRtScore The score for the right hand in the rapid alternating movement of hands test in the upper limb coordination section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). The score for the right hand in the rapid alternating movement of hands test of upper limb coordination, as part of the Friedreich's Ataxia Rating Scale (FARS). Rapid Alternating Movements of Hands 0;1;2;3 0 - Normal;1 - Mild (slightly irregular or slowed);2 - Moderate (irregular and slowed);3 - Too poorly coordinated to perform task. Numeric Values

Forearm pronation/supination 15 cm above thigh; 10 full cycles as fast as possible; assess rate, rhythm, accuracy; practice 10 cycles before rating, if time > 7 sec add 1 to score. Use stopwatch:

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11792 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upright stability feet together stance trial 3 duration FARSNEUpStbStncFtTgStTry3Dur Duration in seconds of feet-together stance trial 3 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Duration in seconds of feet-together stance trial 3 in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Trial 3 0;1;2;3;4 0 - 1 minute or longer;1 - <1 minute, >45 sec;2 - <45 sec, >30 sec;3 - <30 sec, >15 sec;4 - <15 sec or needs hands held by assistant/device Numeric Values

If a patient can stand in a particular position for 1 minute or longer in trial 1, trials 2 and 3 are abandoned.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

second
C11803 Friedreich's Ataxia Rating Scale (FARS) - neurological exam gait device text FARSNeurExUprtStbltyGaitDevTxt Text description of any device used for assistance during gait test in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Text description of any device used for assistance during gait test in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Device, if any Alphanumeric

Note if the gait was achieved with or without device, and serial examinations should be done with the same device as in the first examination.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points 255

Free-Form Entry

C11739 Friedreich's Ataxia Rating Scale (FARS) - daily activities speech score FARSDailyActivsSpeechScore Speech score for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Speech score for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Speech 0;0.5;1;1.5;2;2.5;3;3.5;4 0 - Normal;;1 - Mildly affected. No difficulty being understood;;2 - Moderately affected. Sometimes asked to repeat statements;;3 - Severely affected. Frequently asked to repeat statements;;4 - Unintelligible most of the time Numeric Values

Increments of 0.5 may be used if strongly felt that a task falls between 2 scores.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

C11771 Friedreich's Ataxia Rating Scale (FARS) - neurological exam lower limb coordination total value FARSNeuroExamLowLimbTotalVal The sum of the scores recorded for Part 3: Neurological Examination C. Lower Limb Coordination, as part of the Friedreich's Ataxia Rating Scale (FARS). The sum of the scores recorded for Part 3: Neurological Examination C. Lower Limb Coordination, as part of the Friedreich's Ataxia Rating Scale (FARS). Total Lower Limb Coordination Score 0;1;2;3;4;5;6;7;8 0;1;2;3;4;5;6;7;8 Numeric Values

Enter numeric value for score of 0-16. Refer to instructions on form.

Subramony SH, May W, Lynch D, et al. (2005). Measuring Friedreich ataxia: interrater reliability of a neurologic rating scale. Neurology. 64:1261-2. Delatycki MB. (2009). Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009. 256(Suppl 1):36-41. Adult;Pediatric Core 3.00 2013-07-11 15:03:49.2 Friedreich's Ataxia Rating Scale (FARS) Ataxia and Performance Measures Outcomes and End Points

Single Pre-Defined Value Selected

CSV