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 Additional Notes (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
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

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
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

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

C11814 Friedreich's Ataxia Rating Scale (FARS) - instrumental testing 9 hole peg test left trial 1 duration FARSInstrTest9HPTLftTry1Dur Duration in seconds for participant/subject to complete left-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 left-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
C11750 Friedreich's Ataxia Rating Scale (FARS) - neurological exam bulbar tongue atrophy fasciculation myoclonus weakness score FARSNEBulbrTonAtroFasMyWeScore The score for severity of tongue 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 tongue atrophy, fasciculation, action myoclonus, and weakness in the bulbar portion of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Tongue 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

C11782 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral nervous system total value FARSNeuroExPrphrlNrvSysTotlVal The sum of the scores recorded for Part 3: Neurological Examination D. Peripheral Nervous System, as part of the Friedreich's Ataxia Rating Scale (FARS). The sum of the scores recorded for Part 3: Neurological Examination D. Peripheral Nervous System, as part of the Friedreich's Ataxia Rating Scale (FARS). Total Peripheral Nervous System Score 0;1;2;3;4;5;6;7;8;9;10;11;12;13;14;15;16;17;18;19;20;21;22;23;24;25;26 0;1;2;3;4;5;6;7;8;9;10;11;12;13;14;15;16;17;18;19;20;21;22;23;24;25;26 Numeric Values

Enter numeric value for score of 0-26. 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

C11793 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upright stability feet together stance mean duration score FARSNEUprtStbStncFtTMeDurScore Score for mean duration of feet-together 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-together 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

C17532 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral nerve system vibratory sense finger right duration FARSPrphNrvSysVibSenFngrRtDur Delay in seconds before participant/subject feels vibration in the right-hand fingers, as part of the Friedreich's Ataxia Rating Scale (FARS). Delay in seconds before participant/subject feels vibration in the right-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

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C11761 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upper limb coordination rapid alternate hand movement left score FARSNeuExULCAltHandLftScore The score for the left 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 left 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

C11804 Friedreich's Ataxia Rating Scale (FARS) - neurological exam gait duration FARSNeuroExmUprtStabltyGaitDur Time in seconds for completion of the gait test in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Time in seconds for completion of the gait test in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Time in seconds Numeric Values

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

Free-Form Entry

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C11740 Friedreich's Ataxia Rating Scale (FARS) - daily activities swallowing score FARSDailyActivsSwallowScore Swallowing score for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Swallowing score for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Swallowing 0;0.5;1;1.5;2;2.5;3;3.5;4 0 - Normal;;1 - Rare choking (< once a month);;2 - Frequent choking (< once a week, > once a month);;3 - Requires modified food, chokes multiple times a week, or patient avoids certain foods;;4 - Requires NG tube or gastrostomy feedings 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

C11772 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral nerve system muscle atrophy right score FARSPNSNeuExMuscAtrphRtScore Score for right-side muscle atrophy in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for right-side muscle atrophy in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Muscle Atrophy 0;1;2 0 - None;1 - Present - mild/moderate;2 - Severe/total wasting Numeric Values

Score most severe atrophy in either upper or lower limb.

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

C11815 Friedreich's Ataxia Rating Scale (FARS) - instrumental testing 9 hole peg test left trial 2 duration FARSInstrTest9HPTLftTry2Dur Duration in seconds for participant/subject to complete left-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 left-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

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C11751 Friedreich's Ataxia Rating Scale (FARS) - neurological exam bulbar cough score FARSNeuroBulbrCoughScore The score for forcefulness of cough in the bulbar portion of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). The score for forcefulness of cough in the bulbar portion of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Cough 0;0.5;1;1.5;2 0 - Normal;;1 - Depressed;;2 - Totally or nearly absent 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. Patient asked to cough forcefully 3 times.

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

C11783 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upright stability footwear indicator FARSNeurExUprtStabltyFootwrInd Indicator whether participant/subject is barefoot or wearing footwear for standing and walking assessments for Part 3: Neurological Examination E. Upright Stability, as part of the Friedreich's Ataxia Rating Scale (FARS). Indicator whether participant/subject is barefoot or wearing footwear for standing and walking assessments for Part 3: Neurological Examination E. Upright Stability, as part of the Friedreich's Ataxia Rating Scale (FARS). BarefootFootwear Barefoot;Footwear Barefoot;Footwear Alphanumeric

For standing and walking assessment instruct patient to wear best walking shoes and record below if barefoot, footwear used. 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

C11794 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upright stability tandem stance trial 1 duration FARSNEUpStTanStanceTr1Dur Duration in seconds of tandem 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 tandem 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 tandem stance, the dominant foot is in the back and the heel of the other foot is lined with the toes of the dominant foot but not in front of the toes (because this makes it even more difficult). 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

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C17533 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral nerve system vibratory sense toe left duration FARSPNSNeuExVibSenToeLtDur Delay in seconds before participant/subject feels vibration in the left-side toes, as part of the Friedreich's Ataxia Rating Scale (FARS). Delay in seconds before participant/subject feels vibration in the left-side toes, as part of the Friedreich's Ataxia Rating Scale (FARS). Time felt for toes 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

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C11762 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upper limb coordination finger taps right score FARSNeuExULCFingerTapsRtScore The score for the right hand in the finger taps 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 finger taps test of upper limb coordination, as part of the Friedreich's Ataxia Rating Scale (FARS). Finger Taps 0;1;2;3;4 Normal;Mild (misses 1-3 times);Moderate (misses 4-9 times);Severe (misses 10-15 times);Cannot perform the task. Numeric Values

Index-fingertip to thumb-crease; 15 reps as fast as possible; practice 15 reps once before rating; if time > 6 sec, add 1 to rating. 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

C11805 Friedreich's Ataxia Rating Scale (FARS) - neurological exam gait score FARSNeuroExmUprtStabltyGaitScr Score for the gait test in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for the gait test in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Gait 0;1;2;3;4;5 0 - Normal;1 - Mild ataxia/veering/difficulty in turning, no cane/other support needed to be safe;2 - Walks with definite ataxia, may need intermittent support or examiner needs to walk with patient for safety's sake;3 - Moderate ataxia/veering/difficulty in turning, walking requires cane/holding onto examiner with one hand to be safe;4 - Severe ataxia/veering, walker or both hands of examiner needed;5 - Cannot walk even with assistance (wheelchair bound) 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. For gait place markers 25 feet apart. Patient walks the distance, turns around and comes back and the activitiy is timed. Serial examinations should be done with the same device as in the first examination. Use stopwatch; walk 8 m/25 ft at normal pace, turn around using single step pivot and return to start; 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

C11741 Friedreich's Ataxia Rating Scale (FARS) - daily activities cutting handling utensils score FARSDalyActsCutUtensilScore Cutting-food-and-handling-utensils score for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Cutting-food-and-handling-utensils score for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Cutting Food and Handling Utensils 0;0.5;1;1.5;2;2.5;3;3.5;4 0 - Normal;;1 - Somewhat slow and clumsy, but no help needed;;2 - Clumsy and slow but can cut most foods with some help needed, or needs assistance when in a hurry;;3 - Food must be cut by someone, but can still feed self slowly;;4 - Needs to be fed 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

C11773 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral nerve system muscle atrophy left score FARSPNSNeuExMuscAtrphLfScore Score for left-side muscle atrophy in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for left-side muscle atrophy in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Muscle Atrophy 0;1;2 0 - None;1 - Present - mild/moderate;2 - Severe/total wasting Numeric Values

Score most severe atrophy in either upper or lower limb.

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

C11816 Friedreich's Ataxia Rating Scale (FARS) - instrumental testing 9 hole peg test left mean duration value FARSInstrTest9HPTLftMeanDurVal Mean duration in seconds for left-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 left-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

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C11752 Friedreich's Ataxia Rating Scale (FARS) - neurological exam bulbar spontaneous speech score FARSNeuroBulbrSpontSpeechScore The score for understandability of spontaneous speech in the bulbar portion of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). The score for understandability of spontaneous speech in the bulbar portion of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Spontaneous Speech 0;0.5;1;1.5;2;2.5;3 0 - Normal;;1 - Mild (all or most words understandable);;2 - Moderate (most words not understandable);;3 - Severe (no or almost no useful speech) 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. Ask the patient to read or repeat the sentences "The President lives in the White House" or "The traffic is heavy today".

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

C11784 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upright stability AFO use indicator FARSNeuExUprtStabltyAFOUseInd Indicator whether participant/subject is using an AFO for standing and walking assessments for Part 3: Neurological Examination E. Upright Stability, as part of the Friedreich's Ataxia Rating Scale (FARS). Indicator whether participant/subject is using an AFO for standing and walking assessments for Part 3: Neurological Examination E. Upright Stability, as part of the Friedreich's Ataxia Rating Scale (FARS). Indicate if AFOs are used No;Yes No;Yes Alphanumeric

Record below if AFOs used.

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

C11795 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upright stability tandem stance trial 2 duration FARSNEUpStbltyTaStanceTr2Dur Duration in seconds of tandem 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 tandem 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
C17534 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral nerve system vibratory sense toe right duration FARSPNSNeuExVibSenToeRtDur Delay in seconds before participant/subject feels vibration in the right-side toes, as part of the Friedreich's Ataxia Rating Scale (FARS). Delay in seconds before participant/subject feels vibration in the right-side toes, as part of the Friedreich's Ataxia Rating Scale (FARS). Time felt for toes 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
C11763 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upper limb coordination finger taps left score FARSNeuExULCFingerTapsLftScore The score for the left hand in the finger taps 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 hand in the finger taps test of upper limb coordination, as part of the Friedreich's Ataxia Rating Scale (FARS). Finger Taps 0;1;2;3;4 Normal;Mild (misses 1-3 times);Moderate (misses 4-9 times);Severe (misses 10-15 times);Cannot perform the task. Numeric Values

Index-fingertip to thumb-crease; 15 reps as fast as possible; practice 15 reps once before rating; if time > 6 sec, add 1 to rating. 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

C11806 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upright stability total value FARSNeuroExUprtStabltyTotlVal The sum of the scores recorded for Part 3: Neurological Examination E. Upright Stability, as part of the Friedreich's Ataxia Rating Scale (FARS). The sum of the scores recorded for Part 3: Neurological Examination E. Upright Stability, as part of the Friedreich's Ataxia Rating Scale (FARS). Total Upright Stability Score 0;1;2;3;4;5;6;7;8;9;10;11;12;13;14;15;16;17;18;19;20;21;22;23;24;25;26;27;28 0;1;2;3;4;5;6;7;8;9;10;11;12;13;14;15;16;17;18;19;20;21;22;23;24;25;26;27;28 Numeric Values

Enter numeric value for score of 0-28. 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

C11742 Friedreich's Ataxia Rating Scale (FARS) - daily activities dressing score FARSDailyActivsDressingScore Dressing score for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Dressing score for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Dressing 0;0.5;1;1.5;2;2.5;3;3.5;4 0 - Normal;;1 - Somewhat slow, but no help needed;;2 - Occasional assistance with buttoning, getting arms in sleeves, etc., or has to modify activity in some way (e.g., having to sit to get dressed, use velcro for shoes, stop wearing ties, etc.);;3 - Considerable help required, but can do some things alone;;4 - Helpless 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

C11774 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral nerve system muscle weakness right score FARSPNSNeuExMuscWknsRtScore Score for right-side muscle weakness in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for right-side muscle weakness in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Muscle Weakness 0;1;2;3;4;5 0 - Normal (5/5);1 - Mild (movement against resistance but not full power 4/5);2 - Moderate (movement against gravity but not with added resistance 3/5);3 - Severe (movement of joint but not against gravity 2/5);4 - Near paralysis (muscular activity without movement 1/5);5 - Total paralysis (0/5) Numeric Values

Test deltoids, interossei, iliopsoas and tibialis anterior. Score most severe weakness in either upper or lower limb.

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

C17523 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral deep tendon reflex ankle jerk left score FARSPNSNeuExDTenRAnkJLftScore Score for left-side ankle 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 ankle jerk in the peripheral nervous system section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). AJ 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

C11753 Friedreich's Ataxia Rating Scale (FARS) - neurological exam bulbar total value FARSNeuroBulbrTotalVal The sum of the scores recorded for Part 3: Neurological Examination A. Bulbar, as part of the Friedreich's Ataxia Rating Scale (FARS). The sum of the scores recorded for Part 3: Neurological Examination A. Bulbar, as part of the Friedreich's Ataxia Rating Scale (FARS). Total Bulbar 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 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 Numeric Values

Enter number value of 0-11. 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

C11785 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upright stability sitting posture score FARSNeuExUprtStbltySitPostrScr Score for sitting posture in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Score for sitting posture in the upright stability section of the neurological exam, as part of the Friedreich's Ataxia Rating Scale (FARS). Sitting Posture 0;1;2;3;4 0 - Normal;1 - Mild oscillations of head/trunk without touching chair back or side;2 - Moderate oscillations of head/trunk, needs contact with chair back or side for stability;3 - Severe oscillations of head/trunk, needs contact with chair back or side for stability;4 - Support on all 4 sides for stability Numeric Values

For sitting posture patient can sit in a chair or examination table.

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

C11796 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upright stability tandem stance trial 3 duration FARSNEUpStTndmStTr3Dur Duration in seconds of tandem 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 tandem 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
C11764 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upper limb coordination total value FARSNeuExULCTotalVal The sum of the scores recorded for Part 3: Neurological Examination B. Upper Limb Coordination, as part of the Friedreich's Ataxia Rating Scale (FARS). The sum of the scores recorded for Part 3: Neurological Examination B. Upper Limb Coordination, as part of the Friedreich's Ataxia Rating Scale (FARS). Total Upper Limb Coordination Score 0;1;2;3;4;5;6;7;8;9;10;11;12;13;14;15;16;17;18 0;1;2;3;4;5;6;7;8;9;10;11;12;13;14;15;16;17;18 Numeric Values

Enter numeric value 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

CSV