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
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

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

second
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

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

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

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

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

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

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

C11775 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral nerve system muscle weakness left score FARSPNSNeuExMuscWknsLftScore Score for left-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 left-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

C11807 Friedreich's Ataxia Rating Scale (FARS) - neurological exam total value FARSNeuroExamTotalVal The sum of the scores recorded for Part 3: Neurological Examination, as part of the Friedreich's Ataxia Rating Scale (FARS). The sum of the scores recorded for Part 3: Neurological Examination, as part of the Friedreich's Ataxia Rating Scale (FARS). Total Neurologic Examination 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;36.5;37;37.5;38;38.5;39;39.5;40;40.5;41;41.5;42;42.5;43;43.5;44;44.5;45;45.5;46;46.5;47;47.5;48;48.5;49;49.5;50;50.5;51;51.5;52;52.5;53;53.5;54;54.5;55;55.5;56;56.5;57;57.5;58;58.5;59;59.5;60;60.5;61;61.5;62;62.5;63;63.5;64;64.5;65;65.5;66;66.5;67;67.5;68;68.5;69;69.5;70;70.5;71;71.5;72;72.5;73;73.5;74;74.5;75;75.5;76;76.5;77;77.5;78;78.5;79;79.5;80;80.5;81;81.5;82;82.5;83;83.5;84;84.5;85;85.5;86;86.5;87;87.5;88;88.5;89;89.5;90;90.5;91;91.5;92;92.5;93;93.5;94;94.5;95;95.5;96;96.5;97;97.5;98;98.5;99;99.5;100;100.5;101;101.5;102;102.5;103;103.5;104;104.5;105;105.5;106;106.5;107;107.5;108;108.5;109;109.5;110;110.5;111;111.5;112;112.5;113;113.5;114;114.5;115;115.5;116;116.5;117 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;36.5;37;37.5;38;38.5;39;39.5;40;40.5;41;41.5;42;42.5;43;43.5;44;44.5;45;45.5;46;46.5;47;47.5;48;48.5;49;49.5;50;50.5;51;51.5;52;52.5;53;53.5;54;54.5;55;55.5;56;56.5;57;57.5;58;58.5;59;59.5;60;60.5;61;61.5;62;62.5;63;63.5;64;64.5;65;65.5;66;66.5;67;67.5;68;68.5;69;69.5;70;70.5;71;71.5;72;72.5;73;73.5;74;74.5;75;75.5;76;76.5;77;77.5;78;78.5;79;79.5;80;80.5;81;81.5;82;82.5;83;83.5;84;84.5;85;85.5;86;86.5;87;87.5;88;88.5;89;89.5;90;90.5;91;91.5;92;92.5;93;93.5;94;94.5;95;95.5;96;96.5;97;97.5;98;98.5;99;99.5;100;100.5;101;101.5;102;102.5;103;103.5;104;104.5;105;105.5;106;106.5;107;107.5;108;108.5;109;109.5;110;110.5;111;111.5;112;112.5;113;113.5;114;114.5;115;115.5;116;116.5;117 Numeric Values

Enter numeric value for score of 0-117. 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

C11743 Friedreich's Ataxia Rating Scale (FARS) - daily activities personal hygiene score FARSDailyActivsPrsnlHygnScore Personal hygiene score for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Personal hygiene score for ataxia in subject/participant, as part of the Friedreich's Ataxia Rating Scale (FARS). Personal Hygiene 0;0.5;1;1.5;2;2.5;3;3.5;4 0 - Normal;;1 - Somewhat slow, but no help needed;;2 - Very slow hygienic care or has need for devices such as special grab bars, tub bench, shower chair, etc.;;3 - Requires personal help with washing, brushing teeth, combing hair or using toilet;;4 - Fully dependent 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

C11786 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upright stability stance feet apart trial 1 duration FARSNEUprStbStncFtAprtTry1Dur Duration in seconds of feet-apart 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-apart 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

Stance assessment begins with feet 20 cm apart. Place marker tapes in the exam room 20 cm apart and the insides of the feet are lined up against these. Subsequent stance tests get 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. 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
C17524 Friedreich's Ataxia Rating Scale (FARS) - neurological exam peripheral deep tendon reflex ankle jerk right score FARSPNSNeuExDTenRAnkJRtScore Score for right-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 right-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

C11754 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upper limb coordination finger-finger test right score FARSNeuExULCFingrFingrRtScore The score for the right 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 right 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

C11797 Friedreich's Ataxia Rating Scale (FARS) - neurological exam upright stability tandem stance mean duration score FARSNEUprtStbltyStanceTndmScr Score for mean duration of tandem 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 tandem 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

C11765 Friedreich's Ataxia Rating Scale (FARS) - neurological exam lower limb coordination heel shin slide test position indicator FARSNeuExLLCHeelShinSlTePosInd Indicator of the participant/subject's position while performing heel-along-shin slide test, as part of the Friedreich's Ataxia Rating Scale (FARS). Indicator of the participant/subject's position while performing heel-along-shin slide test, 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

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

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

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
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

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
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

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

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
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

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
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

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

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

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

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

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
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

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

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
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