Application of a Scale for the Assessment and Rating of Ataxia (SARA) in Friedreich's ataxia patients according to posturography is limited.
BACKGROUND
A scale for the Assessment and Rating of Ataxia (SARA) was developed for evaluation of autosomal dominant cerebellar ataxias (ADCA) and was also recommended for clinical trials of Friedreich's ataxia patients (FRDA). FRDA, unlike ADCA, is characterized as being a sensory type of ataxia for which the disease-specific Friedreich ataxia rating scale (FARS) was developed. The objective of this study was to determine whether SARA and FARS scores are associated with posturographic parameters in FRDA patients.


METHODS
Adult patients with genetically confirmed FRDA (n=11) and ADCA (n=13) were evaluated by SARA, FARS and posturography.


RESULTS
FRDA patients' postural stability parameters, in stance with visual control, correlated with balance impairment in FARS (r=0.622; p<0.05) and SARA (r=0.735; p<0.05). Without visual control, only FARS correlated with balance impairment (r=0.732; p<0.05).


CONCLUSIONS
The SARA, in FRDA patients, correlates with stance with visual control but not without visual control which emphasizes sensory ataxia. This suggests that application of the SARA in Friedreich's ataxia patients according to posturography is possible but presumably limited and FARS, although being a more time consuming scale, may have advantages over SARA in FRDA patients.
Department of Neurology, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, V Uvalu 84, Prague 5, 15006, Czech Republic; Department of Paediatric Neurology, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, V Uvalu 84, Prague 5, 15006, Czech Republic. Electronic address: schwabova@gmail.com.
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J
Schwabova
Research Sport Center, Charles University in Prague, Faculty of Physical Education and Sport, Jose Martiho 31, Prague 6, 16252, Czech Republic.
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T
Maly
Department of Neurology, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, V Uvalu 84, Prague 5, 15006, Czech Republic; International Clinical Research Center, St. Anne's University Hospital Brno, Pekařská 53, Brno, 65691, Czech Republic.
J
J
Laczo
Department of Paediatric Neurology, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, V Uvalu 84, Prague 5, 15006, Czech Republic.
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A
Zumrova
Department of Paediatric Neurology, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, V Uvalu 84, Prague 5, 15006, Czech Republic.
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V
Komarek
Department of Biology and Medical Genetics, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, V Uvalu 84, Prague 5, 15006, Czech Republic.
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Z
Musova
Research Sport Center, Charles University in Prague, Faculty of Physical Education and Sport, Jose Martiho 31, Prague 6, 16252, Czech Republic.
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F
Zahalka
D000293:Adolescent; D000328:Adult; D000067528:Ataxins; D002648:Child; D005260:Female; D005621:Friedreich Ataxia / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000235:genetics; D004856:Postural Balance / Q000502:physiology*; D011187:Posture / Q000502:physiology*; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric; D055815:Young Adult
D000067528:Ataxins; D009419:Nerve Tissue Proteins
Autosomal dominant spinocerebellar ataxia; Clinical rating scale; FARS; Friedreich's ataxia; Posturography; SARA