Twenty-four children with Williams syndrome underwent systematic neuro
logic evaluations. Abnormalities of mental status, motor coordination,
tone, and gait were most prevalent. Tone abnormalities varied as a fu
nction of age, with younger children frequently exhibiting decreased t
one and older subjects almost exclusively having increased tone. Tile
gait and coordination abnormalities persisted among older subjects, in
dicating that they were not simply maturational problems. Physicians c
aring for such youngsters need to be aware that a variety of neurologi
c abnormalities are common in Williams syndrome and may change or prog
ress over time. Neurologic examinations that reveal findings beyond th
e typical pattern that we report may raise suspicion for added neurolo
gic insult and warrant further investigation.