Gait function in adults with Williams syndrome

Despite early neurological reports of gait abnormalities in Williams syndrome (WS), a rare genetically based neurodevelopmental disorder, there has not yet been any systematic investigation of gait dysfunction in this disorder. The current study examined the gait characteristics in adults with WS and a neurologically normal control group as they walked at self-selected slow, preferred and fast speeds using the GAITRite walkway. The WS group showed hypokinetic gait, which manifested as reduced gait speed and stride length, but with a disproportionate increase in cadence (stepping frequency) as speed was increased. The WS group also showed increased variability of stride length and a broad based stepping pattern implicating a compensatory strategy for postural instability. Performance IQ correlated significantly with stride length in the WS group. While these results should be considered preliminary due to the small sample size, these findings have implications for our understanding of the neural basis of gait dysfunction in WS.

[1]  J. Brock,et al.  To Match or Not to Match? Methodological Issues in Autism-Related Research , 2004, Journal of autism and developmental disorders.

[2]  M Illert,et al.  Comparative analysis of the gait disorder of normal pressure hydrocephalus and Parkinson's disease , 2001, Journal of neurology, neurosurgery, and psychiatry.

[3]  U Bellugi,et al.  Cerebral morphologic distinctions between Williams and Down syndromes. , 1993, Archives of neurology.

[4]  Jeffrey M. Hausdorff,et al.  Gait variability and basal ganglia disorders: Stride‐to‐stride variations of gait cycle timing in parkinson's disease and Huntington's disease , 1998, Movement disorders : official journal of the Movement Disorder Society.

[5]  Paul M. Thompson,et al.  3D pattern of brain abnormalities in Williams syndrome visualized using tensor-based morphometry , 2007, NeuroImage.

[6]  A. Deckel Hemispheric control of territorial aggression in Anolis carolinensis: effects of mild stress. , 1998, Brain, behavior and evolution.

[7]  U Bellugi,et al.  Evidence for superior parietal impairment in Williams syndrome , 2005, Neurology.

[8]  Steve Hansen,et al.  The visual regulation of goal-directed reaching movements in adults with Williams syndrome, Down syndrome, and other developmental delays. , 2006, Motor control.

[9]  A. du Plessis,et al.  Neurologic Findings in Children and Adults With Williams Syndrome , 1996, Journal of child neurology.

[10]  P. Strømme,et al.  Prevalence Estimation of Williams Syndrome , 2002, Journal of child neurology.

[11]  A. Karmiloff-Smith,et al.  Brain biochemistry in Williams syndrome , 1998, Neurology.

[12]  P. Thier,et al.  Specific influences of cerebellar dysfunctions on gait. , 2007, Brain : a journal of neurology.

[13]  M. Hallett,et al.  Gait in patients with cerebellar ataxia , 1998, Movement disorders : official journal of the Movement Disorder Society.

[14]  M. A. Frens,et al.  Visual depth processing in Williams–Beuren syndrome , 2005, Experimental Brain Research.

[15]  N. Rinehart,et al.  Fronto-parietal and cerebellar contributions to motor dysfunction in Williams syndrome: A review and future directions , 2008, Neuroscience & Biobehavioral Reviews.

[16]  U. Nayak,et al.  The effect of age on variability in gait. , 1984, Journal of gerontology.

[17]  John L Bradshaw,et al.  Gait function in newly diagnosed children with autism: Cerebellar and basal ganglia related motor disorder. , 2006, Developmental medicine and child neurology.

[18]  Xiaonan Xue,et al.  Cognitive processes related to gait velocity: results from the Einstein Aging Study. , 2006, Neuropsychology.

[19]  G. Carlesimo,et al.  Procedural learning deficit in children with Williams syndrome , 2001, Neuropsychologia.

[20]  G. Deuschl,et al.  Typical features of cerebellar ataxic gait , 2002, Journal of neurology, neurosurgery, and psychiatry.

[21]  J. Summers,et al.  Abnormalities in the stride length‐cadence relation in parkinsonian gait , 1998, Movement disorders : official journal of the Movement Disorder Society.

[22]  M R Pierrynowski,et al.  Enhancing the ability of gait analyses to differentiate between groups: scaling gait data to body size. , 2001, Gait & posture.

[23]  G M Earhart,et al.  Selection and coordination of human locomotor forms following cerebellar damage. , 2001, Journal of neurophysiology.

[24]  Aftab E Patla,et al.  Balance during obstacle crossing following stroke. , 2008, Gait & posture.

[25]  Janette Atkinson,et al.  Neurobiological Models of Visuospatial Cognition in Children With Williams Syndrome: Measures of Dorsal-Stream and Frontal Function , 2003, Developmental neuropsychology.

[26]  J. Summers,et al.  Ability to modulate walking cadence remains intact in Parkinson's disease. , 1994, Journal of neurology, neurosurgery, and psychiatry.

[27]  M. Morris,et al.  Three‐dimensional gait biomechanics in Parkinson's disease: Evidence for a centrally mediated amplitude regulation disorder , 2005, Movement disorders : official journal of the Movement Disorder Society.

[28]  Stephan Eliez,et al.  IV. Neuroanatomy of Williams Syndrome: A High-Resolution MRI Study , 2000, Journal of Cognitive Neuroscience.

[29]  C. Mervis,et al.  Neural Basis of Genetically Determined Visuospatial Construction Deficit in Williams Syndrome , 2004, Neuron.

[30]  J. Atkinson,et al.  Visual and visuospatial development in young children with Williams syndrome , 2001, Developmental medicine and child neurology.

[31]  O. Blin,et al.  Quantitative analysis of gait in Parkinson patients: increased variability of stride length , 1990, Journal of the Neurological Sciences.

[32]  T. L. Jernigan,et al.  Specific neurobehavioral profile of Williams' syndrome is associated with neocerebellar hemispheric preservation , 1992, Neurology.

[33]  C. Mervis,et al.  Williams syndrome: cognition, personality, and adaptive behavior. , 2000, Mental retardation and developmental disabilities research reviews.

[34]  C. Davis,et al.  Multiple comparison procedures within experimental research. , 1984 .

[35]  S. M. Morton,et al.  Relative contributions of balance and voluntary leg-coordination deficits to cerebellar gait ataxia. , 2003, Journal of neurophysiology.

[36]  Nir Giladi,et al.  Impaired regulation of stride variability in Parkinson's disease subjects with freezing of gait , 2003, Experimental Brain Research.

[37]  U Bellugi,et al.  Enlarged cerebellar vermis in Williams syndrome. , 2001, Journal of psychiatric research.

[38]  U. Bellugi,et al.  Neurologic features of Williams and Down syndromes. , 1989, Pediatric neurology.

[39]  R. Borgatti,et al.  Evolution of neurologic features in Williams syndrome. , 2007, Pediatric neurology.

[40]  E. Tolosa,et al.  Comparative analysis of gait in Parkinson's disease, cerebellar ataxia and subcortical arteriosclerotic encephalopathy. , 1999, Brain : a journal of neurology.

[41]  U. Bellugi,et al.  Cerebellar abnormalities in infants and toddlers with Williams syndrome , 2002, Developmental medicine and child neurology.