Quality of Life in Sporadic Adult-Onset Ataxia

  • M. Abele
Reference work entry


 Sporadic adult-onset ataxia (SAOA) is a usually slowly progressive disorder that accounts for a considerable part of the neurodegenerative cerebellar diseases. Patients with SAOA suffer from a predominant cerebellar syndrome with ataxia of stance and gait, irregular limb movements, dysarthria, and oculomotor abnormalities. In addition, extracerebellar features like disturbances of the peripheral or autonomic nervous system but also sleep related complaints and depression are frequently found. The impact of progressive motor disability and non-motor complaints on health-related quality of life (Hr-QoL) has only been studied in one small sample of german SAOA patients by means of the medical outcome study short form (SF-36). Compared to a large german control group, SAOA patients had lower scores in all SF-36 dimensions except for bodily pain. The greatest differences were seen in the domains physical functioning, followed by social functioning, role limitations (emotional problems), and general health perception. There was a significant negative correlation of all non-motor SF-36 dimensions with a depression score. Walking aid dependency was significantly correlated with poorer health status perception in several motor and non-motor domains. In addition, impaired sleep quality was correlated with an impaired general health perception and with bodily pain. The available data demonstrate a great impact of motor and non-motor symptoms of SAOA on Hr-QoL. Adequate treatment of depression, motor disability, and impaired sleep quality is therefore essential to improve Hr-QoL in ataxic patients.


Sleep Quality Multiple System Atrophy Epworth Sleepiness Scale Pittsburgh Sleep Quality Index General Health Perception 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

List of Abbreviations:


aquired immunodeficiency syndrome


Beck depression inventory


bodily pain


cerebrospinal fluid


 excessive daytime sleepiness


standardised instrument for use as a measure of health outcome


Epworth sleepiness scale


Friedreich ataxia


general health perception


human immunodeficiency virus


health-related quality of life


heart rate variability


mental health


magnetic resonance imaging


 multiple system atrophy


Parkinson’s disease


physical functioning


Pittsburgh sleep quality index


role limitations (emotional problems)


role limitations (physical problems)


sporadic adult-onset ataxia


spinocerebellar ataxia


spinocerebellar ataxia type 6


standard deviation


somatosensory evoked potential


social functioning


medical outcome study short form




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© Springer Science+Business Media LLC 2010

Authors and Affiliations

  • M. Abele
    • 1
  1. 1.Department of NeurologyUniversity of BonnBonnGermany

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