PMID- 28695363
OWN - NLM
STAT- MEDLINE
DCOM- 20180418
LR  - 20181113
IS  - 1432-1459 (Electronic)
IS  - 0340-5354 (Linking)
VI  - 264
IP  - 8
DP  - 2017 Aug
TI  - How does performance of the Friedreich Ataxia Functional Composite compare to 
      rating scales?
PG  - 1768-1776
LID - 10.1007/s00415-017-8566-0 [doi]
AB  - Progression of Friedreich ataxia (FRDA) is often measured using neurological 
      rating scales such as the Friedreich Ataxia Rating Scale (FARS). Performance 
      scales comprising functional measures have been used in other conditions due to 
      their increased sensitivity and reproducibility and may replace examination-based 
      measures. The aims of this study were to examine the relationship between the 
      Friedreich Ataxia Functional Composite (FAFC) measures and characteristics of 
      FRDA to determine if the FAFC is more sensitive to clinical change over time 
      compared to its components. One hundred and twenty-two individuals completed the 
      timed 25-foot walk (T25FW), 9-Hole Peg Test (9HPT) and the low-contrast letter 
      acuity (LCLA) test at baseline, 63 at year 1, 34 at year 2 and 25 at year 3. 
      Composite scores, Z2 (T25FW and 9HPT) and Z3 (T25FW, 9HPT and LCLA) were created. 
      Correlation analyses were conducted. Change in FAFC components were examined over 
      1, 2, and 3 years. The FARS, Z2, Z3 and 9HPT showed significant change over all 
      time points compared to baseline. The T25FW only demonstrated significant change 
      over 3 years. The LCLA demonstrated no significant change over any of the time 
      points. The FAFC shows significant change over time and indicates disease 
      progression, however, this may result from individual components driving the 
      differences. The LCLA showed no change over time, rendering Z3 redundant. The 
      FAFC is of limited value in cohorts with non-ambulant individuals as it leads to 
      skewing of the dataset and is better suited to less affected populations.
FAU - Tai, Geneieve
AU  - Tai G
AD  - Bruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research 
      Institute, Flemington Rd, Parkville, VIC, 3052, Australia.
FAU - Yiu, Eppie M
AU  - Yiu EM
AD  - Bruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research 
      Institute, Flemington Rd, Parkville, VIC, 3052, Australia.
AD  - Department of Paediatrics, University of Melbourne, Parkville, VIC, 3052, 
      Australia.
AD  - Department of Neurology, Royal Children's Hospital, Parkville, VIC, 3052, 
      Australia.
FAU - Delatycki, Martin B
AU  - Delatycki MB
AD  - Bruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research 
      Institute, Flemington Rd, Parkville, VIC, 3052, Australia.
AD  - Faculty of Medicine, Nursing and Health Sciences, School of Psychological 
      Science, Monash University, Clayton, VIC, 3168, Australia.
AD  - Department of Paediatrics, University of Melbourne, Parkville, VIC, 3052, 
      Australia.
AD  - Victorian Clinical Genetics Services, Parkville, VIC, 3052, Australia.
FAU - Corben, Louise A
AU  - Corben LA
AD  - Bruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research 
      Institute, Flemington Rd, Parkville, VIC, 3052, Australia. 
      louise.corben@vcgs.org.au.
AD  - Faculty of Medicine, Nursing and Health Sciences, School of Psychological 
      Science, Monash University, Clayton, VIC, 3168, Australia. 
      louise.corben@vcgs.org.au.
AD  - Department of Paediatrics, University of Melbourne, Parkville, VIC, 3052, 
      Australia. louise.corben@vcgs.org.au.
AD  - Department of Occupational Therapy, Monash Health, Clayton, VIC, 3086, Australia. 
      louise.corben@vcgs.org.au.
LA  - eng
PT  - Comparative Study
PT  - Journal Article
DEP - 20170710
PL  - Germany
TA  - J Neurol
JT  - Journal of neurology
JID - 0423161
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Child
MH  - Cohort Studies
MH  - Disease Progression
MH  - Female
MH  - Fingers/physiopathology
MH  - Friedreich Ataxia/*diagnosis/genetics/physiopathology
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Motor Activity
MH  - Reading
MH  - *Severity of Illness Index
MH  - Visual Acuity
MH  - Walk Test
MH  - Young Adult
OTO - NOTNLM
OT  - Friedreich Ataxia Functional Composite
OT  - Friedreich ataxia
OT  - Performance measures
OT  - Rating scales
EDAT- 2017/07/12 06:00
MHDA- 2018/04/19 06:00
CRDT- 2017/07/12 06:00
PHST- 2017/03/16 00:00 [received]
PHST- 2017/07/03 00:00 [accepted]
PHST- 2017/07/02 00:00 [revised]
PHST- 2017/07/12 06:00 [pubmed]
PHST- 2018/04/19 06:00 [medline]
PHST- 2017/07/12 06:00 [entrez]
AID - 10.1007/s00415-017-8566-0 [pii]
AID - 10.1007/s00415-017-8566-0 [doi]
PST - ppublish
SO  - J Neurol. 2017 Aug;264(8):1768-1776. doi: 10.1007/s00415-017-8566-0. Epub 2017 
      Jul 10.