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The above interpretation of a visuospatial deficit rather than a motor deficit was supported by Julianne’s poor performance on the nonmotor Geometric Puzzles subtest, a purely visuospatial task that assesses mental rotation, visuospatial analysis, and attention to detail. Julianne was presented with a grid containing geometric shapes. For each item, she was required to match two shapes outside of the grid to two shapes within the grid. A point is awarded for each correct shape matched. Julianne earned only 5 out of 24 possible points, a performance that placed her at less than the 2nd percentile for age, Well Below Expected Level.

CLINICAL IMPRESSIONS AND SUMMARY

Julianne D’s history, clinical observations, and the results of her testing revealed a complex and somewhat confusing profile. Attention/executive functions (response inhibition to noise distracters), language, and verbal memory skills were average for age and significantly better than Julianne’s pervasively poor visuospatial abilities. The former were weak even on tasks that were not directly assessing visuospatial functioning, but that had visuospatial stimuli. Therefore, several subtests were only interpreted clinically to demonstrate the negative impact that Julianne’s poor visuospatial abilities can have on activities in other areas in everyday life. Specifically, this effect was seen on NEPSY-II subtests assessing other areas, such as inhibition, naming, and visuomotor precision. Despite her significantly poor visuospatial perception, Julianne displayed facial recognition skills that were excellent. Results will be summarized for each domain in more detail in the following discussion.

On the Attention/Executive Functioning domain subtests administered to her, Julianne displayed average auditory selective attention for age. Her ability to inhibit motor response to interfering auditory stimuli was also average. Julianne had significant difficulty, however, on the Inhibition subtest in both the naming and inhibition conditions because of the visuospatial stimuli on this subtest. Her performance was very weak, not because she could not name or inhibit, but because of her visuospatial deficits. The clinician, therefore, did not interpret this test for executive functioning. However, clinically, the results were valuable for demonstrating the marked difficulty Julianne has with the visuospatial concepts in all domains, not just on specific visuospatial tasks.

Despite apparently good receptive language on Comprehension of Instruction and in expressive language skills observed informally during testing, rapid naming performance was borderline for completion time and very poor for correct naming. These scores were not valid, and it would be wrong to assume from these results that Julianne had a naming deficit. Clinical observation during testing revealed that Julianne did not have a problem with naming per se, because

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she accessed color names with no errors. Rather, the problem appeared to be Julianne’s confusion in recognizing shapes, so she often did not realize that she had identified them incorrectly. Her completion time was borderline due to her marked confusion on the second part of the test where she had to deal with two visuospatial concepts (shape/size) in addition to color that she was able to name with no difficulty. Since her problems with the Speeded Naming subtest were due to visuospatial confusion, not naming problems, this subtest, too, was only interpreted clinically. It did, however, contribute to the picture of Julianne’s difficulty in handling visuospatial stimuli in areas other than on tasks of visuospatial processing.

In the Memory and Learning domain, in addition to Julianne’s average performance for free recall, free/cued recall, and recognition of story details on a subtest of story memory, Narrative Memory, Julianne was also administered the Memory for Faces and Memory for Faces Delayed subtests. Her performance on both the immediate and the delayed MF tasks was excellent, a finding that appears to be uniquely dissociated from her visuospatial deficits.

In the Sensorimotor domain, Julianne’s performance on Visuomotor Precision revealed average time to completion, suggesting no difficulties with motor speed, but poor accuracy in straying outside the tracks. Again, the problem appeared to be visuospatial rather than motoric. The clinician noted that Julianne did not seem to perceive and/or anticipate the curves as she was drawing the pencil lines between the winding tracks. Therefore, her pencil line frequently strayed outside the tracks on a curve, causing numerous errors. Therefore, this subtest was interpreted clinically for total errors, since they seemed to be the result of her visuospatial deficits, rather than poor motor coordination.

On specific visuospatial assessment in the Visuospatial Processing domain, Julianne displayed poor ability to solve nonmotor geometric puzzles and poor design copying overall. Her process scores on Design Copying showed average motor performance, which correlates with Julianne’s Visuomotor Processing Time to Completion performance, mentioned before. However, she displayed very poor ability to perceive and reproduce the outside configuration (global processing) of a figure and poor ability to reproduce and integrate small features within the design (local processing). Although Julianne’s local processing of details was poor, this area was significantly better than global processing for the outside configuration of the design. Visuospatial processing is purported to be subserved by right posterior functioning, particularly right parietal.

On subtests that could be scored reliably, rather than interpreted clinically, Julianne’s profile of strengths, weaknesses, and average abilities in relation to children of her age are summarized (see the following pages).

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Classification of Test Results—Julianne D.

Inhibition of motor response to sound distracters (ability to stand still with eyes closed)

Comprehension of oral language instructions of increasing complexity

Story memory for free recall of details, for free and cued recall, and for recognition

Fine motor coordination for untimed paperpencil design copying task

Motor and visuospatial skills integrated for copying 2-dimensional fi gures

Visuospatial skills were the confounding factor;

fi ne motor coordination for copying was average (see above)

Visuospatial perception, analysis, and reproduction of confi guration of a 2-D design

Mental rotation, visuospatial analysis, and attention to detail on a nonmotor geometric matching task

Summary Graphs of NEPSY-II Scores-Julianne D.

(Graphed in relation to the subtest mean for age of 10 + 3) Average range: 8–12

Attention/Executive Functioning Scaled Scores

Memory and Learning Scaled Scores

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Along with Julianne’s area of strength in facial recognition skills in terms of the typical child of her age (mean = 10 + 3), she displayed relative strengths in language and verbal memory, both at the 37th percentile in relation to her cognitive ability at the 2nd percentile. This is an unusual profile. In addition, Julianne’s NEPSY-II results revealed the following primary and secondary deficits, as well as a diagnostic cluster pointing to a preliminary diagnosis.

Primary deficit: Visuospatial processing (with dissociation of face recognition, which is a strength).

Secondary deficits that stem from this primary deficit include the following:

•Understanding visuospatial words such as inside/outside, and confusion in naming shapes and directions despite average language overall on this assessment. Further assessment of the subtleties of language should be undertaken, however. (A secondary deficits, because Julianne had marked weaknesses in visuospatial perception and processing.)

•Slow naming performance on tasks with visuospatial components.

(Due to visuospatial confusion, not a naming or processing speed deficit. A naming deficit could be ruled out as Julianne had no difficulty naming colors quickly.)

•Poor copying skills and visuomotor precision on tasks with visuospatial components. (Due to primary deficits in perceiving visuospatial forms and stimuli, not due to motor deficits. A primary motor problem could be ruled out, as she performed WNL on the Motor Scaled Score of Design Copying (untimed) and VP Completion Time (timed).)

•Difficulty recognizing the shapes of letters and numbers negatively impacting acquisition of basic reading and math skills. Later, the math learning problems may become accentuated.

The pattern of Julianne’s strengths and primary and secondary deficits along with the physical and behavioral characteristics of short stature, elfin face, friendly nature, possible mild–moderate intellectual disability, very well-developed language and poor visuospatial skills with good facial recognition abilities converge on a diagnostic behavioral cluster consistent with William’s syndrome. This is a genetic syndrome that includes neurological maldevelopment characterized by good language abilities, but also by very significant visuospatial deficits. These children have excellent facial recognition skills that