Motor Function in Adults with
Williams Syndrome:
Is There Evidence for Basal
Ganglia or Cerebellar
Dysfunction?
-
1
Monash University, Centre for Developmental Psychiatry & Psychology, School of Psychology, Psychiatry and Psychological Medicine, Australia
-
2
University of Wisconsin-Milwaukee, United States
-
3
Monash University, Experimental Neuropsychology Unit, School of Psychology, Psychiatry and Psychological Medicine, Australia
Despite early reports of neurological ‘soft’ signs in Williams syndrome (WS), there has not yet been any systematic investigation of the developmental progression of motor dysfunction in this disorder. These individuals are often described as ‘clumsy’, and show poor motor coordination, for instance when walking across uneven surfaces or negotiating obstacles. The few clinical reports of motor problems in WS have described subtle neurologic signs of cerebellar dysfunction such as mild dysmetria and poor balance during cerebellar sensitization tests (tandem walking). However, the precise neuroanatomical loci for the motor impairments seen in WS are as yet unclear, with some evidence that motor impairments resembling basal ganglia dysfunction appear to increase and persist into adulthood. The aim of this study was to systematically examine neuromotor dysfunction in adults with WS, by employing a kinematic analysis of upper-limb and gait function to determine whether the motor profile is consistent with basal ganglia or cerebellar dysfunction. A further aim of the study was to examine the impact of attention and executive deficits on visuomotor 48 performance in WS. The relationship between cognitive function (assessed on standardized tests of attention and executive function) and motor control was explored using visuomotor tasks with varying levels of task difficulty for individuals with WS. Preliminary results suggest a more pervasive functional impairment that involves deficits in movement control when compared to matched controls, implicating frontostriatal deficits in WS. The results will be discussed in the context of the important interaction between higher-level cognitive function and motor control in WS. Further, we speculate about the possible genetic influences on early brain development that may contribute to the visuomotor deficits seen in individuals with WS.
Conference:
12th International Professional Conference on Williams Syndrome, Garden Grove,CA, United States, 13 Jul - 14 Jul, 2008.
Presentation Type:
Poster Presentation
Topic:
Multidisciplinary Poster Session
Citation:
Hocking
DR,
Rinehart
NJ,
McGinley
J and
Bradshaw
JL
(2009). Motor Function in Adults with
Williams Syndrome:
Is There Evidence for Basal
Ganglia or Cerebellar
Dysfunction?.
Conference Abstract:
12th International Professional Conference on Williams Syndrome.
doi: 10.3389/conf.neuro.09.2009.07.040
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Received:
04 May 2009;
Published Online:
04 May 2009.
*
Correspondence:
D. R Hocking, Monash University, Centre for Developmental Psychiatry & Psychology, School of Psychology, Psychiatry and Psychological Medicine, Clayton, Australia, darren.hocking@med.monash.edu.au