Together with
Barbara Landau, who is in the Cognitive Science Department at Johns
Hopkins University, I have been attempting to use what we have learned
about spatial cognition in adults to help us understand the nature of
spatial deficits in children with Williams Syndrome. Williams Syndrome
(WS) is a rare (1 in 20,000 births) genetic disorder that results in a
unique phenotypic pa ttern
that includes a characteristic facial profile, disorders of the heart,
and anomalies of the viscera. In addition, they display an unusual and
distinctive cognitive profile consisting of strong language abilities
and severely impaired visuo-spatial skills (see drawings below). This
unique pattern of sparing and impairments in the cognitive domain has
been taken by some investigators as support for the idea that the human
mind consists of separate modules that are responsible for different
areas of mental life such as talking, seeing, and thinking.
The drawings we collected above are just one way to demonstrate the
spatial deficit in people
with WS. This deficit can also be seen in other tests of visuo-spatial
construction ability such as the block construction test which is a
component of many IQ tests. Examples of WS adult performance on this
test are shown here. In general, adults with WS score in the first
percentile on this test. We have also collected eye movements from
children with WS while they attempted to solve a computer-based version
of the block construction test in order to determine if their
difficulties could be attributed to a failure to fixate the model
pattern. You can view a
video showing a
playback of these fixations from one WS participant. This video
illustrates a couple of important points. First, WS children did fixate
the model; in fact, they fixated it as often as mental age-matched
control children for models containing 4 or fewer blocks. Second, WS
children recognized that their constructions were incorrect but didn't
know how to fix them (see
Hoffman, Landau, and Pagani, 2003).
The fact that the WS children seemed to recognize that their
constructions did not match the model suggests that their perception
of the world may be fine and that the basis of their visuo-construction
deficit may lie in some other cognitive process. We have subsequently
examined several task that indicate that their ability to see
how parts are organized into wholes is intact. A good
example of this sort of task is
biological motion perception in which lights are attached to
various joints of a person who is filmed while walking (a
so-called point-light walker or PLW), dancing or performing various
other actions on a dark stage. When the first frame of such a movie is
viewed, the pattern of lights appears to be just that, a collection of
individual lights. However, once the movie is presented, the lights
suddenly cohere into the unmistakable percept of a person walking. This
task requires the observer to integrate the motion of multiple lights
into a coherent percept of and if people with WS have difficulty in
perceptually organizing parts into wholes, we might expect that
they would have trouble correctly seeing these stimuli. In our lab,
Jordan et al. asked children with WS to determine whether a point light
walker was walking towards the left or right. We added various amounts
of noise to the stimuli to make the task difficult (press
here to see an easy
condition and here to
view the most difficult one). Surprisingly, WS children were as good or
better than controls at this task.
Reiss, Hoffman, and Landau (2005) extended these findings to
other motions tasks showing that perception of motion coherence was also
preserved in children and adults with WS although they also found that
performance on a so-called 2D form-from-motion task was impaired in WS,
possibly due to difficulty in segmenting targets from backgrounds.
Landau, Hoffman, and
Kurtz (in press) reported that identification of common objects was
also preserved in children with WS. In this task, the difficulty of
object identification was varied by showing the object in unusual or
noncanonical orientations and by blurring it (see below; press
here to
see the whole set of objects). Overall, WS and control groups performed
quite well in this task with control children showing a slight advantage
for noncanonical orientations and WS children having a comparable edge
in identifying blurred objects. A follow-up task used line
drawings in place of full color pictures in order to to eliminate cues
to identity based on color or texture. The results were similar.

One might question whether identification of objects, even those
presented as line drawings, depends on the correct perception of the
arrangement of object parts. In order to address this, we (Hoffman and
Landau, in preparation) examined performance in a matching task
requiring the correct perception of the spatial arrangement of
parts within an object. To eliminate the effects of prior experience, we
used novel objects which were constructed by rearranging and retexturing
the parts of real objects (see example below). Participants were
required to match a target object to one of three alternatives.
Crucially, all three alternatives had the same parts but in different
spatial arrangements. In the perception condition, the target
object remained on the screen with the choices until response. In the
memory condition, the target was removed and the alternatives
were presented one second later. In both conditions, WS and controls had
the same accuracy suggesting that people with WS can perceive and
remember (at least for a short time) the spatial arrangement of parts.

Very different results were obtained however when the nature of the
discrimination was changed. In this series, participants were required
to discriminate the "handedness" of the object rather than the
spatial arrangement of it's features (see example below). For most
object recognition tasks in the real world, handedness is largely
irrelevant. We don't care whether the cup is seen with the handle on the
left or right; it is still a cup and we need to recognize it as such. In
fact, one of the difficult issues in modeling object recognition is how
to ignore information about orientation. Other research indicates that
an object's orientation is indeed separable from the object's shape. For
example, damage in certain areas of the parietal lobe can result in a
selective inability to discriminate handedness while preserving the
ability to recognize the object (Davidoff & Warrington, 2001). Results
showed that WS participants had trouble making this discrimination
particularly when holding the target object in memory, in which case
performance was close to chance.

Our current hypothesis is that spatial deficits associated with WS may be
due to delayed and arrested development of certain structures in the
dorsal stream of the visual system, particularly regions of the parietal
cortex that are concerned with a wide variety of visual functions
including mirror image discrimination and visually guided action. In contrast, the ventral visual
stream, which is concerned with recognition of objects and faces,
appears to be largely intact in people with WS, consistent with their
ability to identify faces and common objects. Our current research
(supported by NINDS RO1 NS 050876) is designed to track the development
of visuo-spatial functions of these two streams over the course of
development to see if this hypothesis continues to provide a good
account of the nature of spatial deficits in this fascinating
population. |