by Bonny Forrest, Ph.D
email: BonForrest@aol.com
The
purpose of this article is to review similarities and differences between
Asperger (AS) and Nonverbal Learning Disability (NVLD) Syndromes.
The existence of AS as a separate diagnostic entity from Autism remains
controversial. Much of this
controversy stems from the presence of children who have social deficits
characteristic of Autism but exhibit lesser degrees of language impairments, and
from the use of the age of onset of language deficits to distinguish between the
two syndromes. Perhaps
even more contestable is whether a distinction exists between AS and NVLD.
The latter, which has not yet been recognized by the DSM-IV-TR as a
diagnostic entity, has been most frequently defined in the literature by a
specific neuropsychological profile. This
profile can be very similar to that of children with AS.
The main difference between the two disorders, as they are most
frequently defined clinically, is the absence in children with NVLD of
restricted interests or special skills. The diagnostic situation is further complicated, however, by
the complex and still-changing definitions of the social deficits observed in
NVLD.
Asperger
Syndrome
Behavioral
Consequences
Attempts have been made to distinguish children with AS on the basis of the functional aspects of their social impairments. Their social interactions have been described as lacking in empathy, exhibiting poor nonverbal communication and use of speech, and being overly reliant on rote language abilities. These children are usually aware of their inability to connect to others (Volkmar and Klin, 2000). In addition, the child with AS tends to become an expert in a specific area of interest (e.g., the weather or subway lines). Although one subgroup of the children with nonverbal disorders of learning described by Johnson and Myklebust (1967) exhibits social deficits, this group does not generally exhibit restricted interests or special skills that would meet the criteria for diagnostic classification as AS.
Few studies have attempted to validate a neuropsychological profile of children with AS. The results of this handful of studies, although far from providing a definitive template, seem to suggest that individuals with AS have deficits in fine and gross motor skills, visual motor integration, visual-spatial perception, nonverbal concept formation, and visual memory. Language skills are generally intact (Klin, Volkmar and Sparrow, 2000). This profile of neuropsychological assets and deficits is very similar to the NVLD profile described by Rourke (1995), as discussed below.
Johnson and Myklebust (1967) were the first to posit the existence of discrete subtypes of nonverbal learning disorders, including one sub-group of children with nonverbal deficits who have deficiencies in social perception. They defined social perception broadly as the inability of a child to interpret both the emotions expressed by another person and the perception of oneself in relation to the behavior of others. Such children cannot pretend, anticipate, evaluate the significance of certain aspects of their environment, or comprehend the meaning of facial expressions and gestures or the subtleties of emotions. They further described the children with difficulties in social perception as having average or above average abilities in language but difficulty using those language skills.
Byron Rourke’s (1995) recent
research has focused primarily on the combination of neuropsychological assets
and deficits that produces as a byproduct the poor social relatedness that
Johnson and Myklebust (1967) described as characteristic of only one group of
children with NVLD. Rourke
described the neuropsychological deficits in NVLD more specifically in terms of
primary, secondary, and tertiary deficits.
The children he studied had primary deficits in tactile perception,
visual perception, complex psychomotor activities and the ability to process
novel material. Secondary deficits
(secondary because they were related to the basic deficits) included
difficulties in tactile attention, visual attention and exploratory behavior.
Tertiary deficits included tactile memory, visual memory, concept
formation and problem solving. According to Rourke (1995), when these neuropsychological
deficits interact with assets in auditory perception, simple motor skills and
the ability to process rote material, socioemotional or adaptational deficits
result. Children with NVLD as described by Rourke (1995) often exhibit extreme
difficulty in processing new or complex social situations and interpreting
facial expressions. In novel
situations, they rely on repetitious or rote behaviors, because they excel in
these skills. Their interactions
with other children are stereotypical and lacking in reciprocity (Rourke and
Tsatsanis, 2000). Children
encompassed in Rourke’s (1995) description may manifest these symptoms from
birth or as a result of neuropsychological injury or disease.
The young boy examined in the following case report provides a graphic illustration of the difficulties presented when attempting to diagnose a child who exhibits some but not all of the characteristics associated with both AS and NVLD.
Case
Report
M
is an eight-year, eight-month-old right-handed male.
He has worn glasses since the age of 9 ½ months.
M was slow to crawl (11 ½ months) but walked independently at 14 months.
His parents were also concerned that his social skills lagged behind his peers.
M frequently observed other children but was hesitant to engage them in
play. School personnel corroborated these concerns as early as
preschool.
M
had three comprehensive assessments, followed by additional testing as part of a
research study.
At
the time of the first assessment at four years and ten months, later motor
milestones, such as undressing, dressing, and copying shapes, were beginning to
emerge. Language skills were described as quite good. His verbal abilities were
assessed at the 99th percentile.
M’s performance revealed deficits, however, in gross and fine motor
abilities and sensory integration.
M
underwent a neuropsychological evaluation at the age of 5 years, 4 months.
At that evaluation, numbers and their applications (e.g., number of cars
in a train) intrigued M. All verbal
skills during this second evaluation were Average to High Average for his age. Nonverbal skills, in contrast, ranged from 2 ½- to
4-year-old levels. A diagnosis of
PDD-NOS was ruled out during that assessment because of his language skills.
Asperger Syndrome was also ruled out because of his lack of restricted
repetitive and stereotyped patterns of behaviors or interests.
M was given a formal diagnosis at that time of NVLD.
A supplemental assessment by the school approximately 6 months later at 5
years, 10 months showed math abilities at the 56th percentile.
Finally,
at the age of 6 years, 11 months, M underwent a second neuropsychological
evaluation. His performance on the
WISC-III is set forth below. Additional
testing revealed above average verbal and significantly impaired nonverbal
skills. Math reasoning abilities on
the Wechsler Individual Achievement Test were at the 27th percentile
with a standard score of 91. He also had poor vocal tone, prosody and awareness
of the pragmatic uses of language. M’s social relations were better than when
he was younger; however, he was reported to have no best friend.
A diagnosis of Learning Disorder, Not Otherwise Specified was given and
the prior diagnosis of NVLD was confirmed.
Overall
Intellectual Function-WISC-III Scaled and Standard Scores
Arithmetic
07
Picture Arrangement
05
FD
93
Comprehension
11
Object Assembly
08
VIQ
110
Additional
testing completed as part of a research study resulted in the following scores:
Achievement-standard
scores.
Spelling
91
Operations
88
Arithmetic
71
Applications
100
Executive
Functions-Z, scaled, T or percentage scores as reported by instrument.
Trails
A
-.9
Trails
B
-.32
NEPSY-Auditory
Attention
10
NEPSY-Core
Domain Score
90
NEPSY-Design
Fluency
09
NEPSY-Knock
and Tap
<2%
Behavior
Rating Inventory of Executive Function- Parent Form
Elevated
T scores in Behavioral Regulation, Initiation, Planning and Organization and
Working Memory
Language-scaled
scores.
NEPSY-Speeded
Naming
08
NEPSY-Comprehension
of Instructions
09
NEPSY-Finger
Discrimination
Dominate
>75%
Nondominate
>75%
NEPSY-Imitating
Hand Positions
01
Right
-10
Left
-5.8
VMI
87
VMI-Visual
109
VMI-Motor
72
NEPSY-Arrows
06
NEPSY-Block
Construction
08
NEPSY-Memory
for Names
06
NEPSY-Sentence
Repetition
11
NEPSY-List
Learning
09
Social
and Emotional Functioning-z scores.
Total
Emotion Score
-.50
Total
Nonverbal Cue Score
-2.25
Discussion
Although
M fits the general neuropsychological profile for both AS and NVLD, he does not
have some of the behavioral characteristics required for a diagnosis of AS:
he lacks ascribed interests or special skills in the presence of
nonverbal cognitive deficits. Although
M’s interest in numbers at one point was considered a possible precursor to a
restricted interest that might be indicative of AS, by the age of 8 that
interest was not considered clinically significant.
When three neuropsychologists at separate major Northeastern University
Hospitals diagnosed M, each ruled out AS because of M’s behavioral
characteristics and relied on the neuropsychological profile and the deficits in
social skills to assign an NVLD diagnosis.
A diagnosis of Autism was never discussed.
In
their reports, however, each alluded to the difficulties of diagnosing M with
NVLD because he did not exhibit all of its characteristics.
These difficulties would have been even more pronounced if these
clinicians had attempted to apply more recent descriptions of NVLD by Rourke
(2000a) or his proposed ICD-10 criteria.
Consistent with Rourke’s (1995) neuropsychological descriptions of NVLD, M’s verbal skills were above average, his mechanical math score on the WRAT-3 was at least 8 standard points lower than his reading score, and he had difficulties with graphomotor activities, tone and prosody. But M does not exhibit some of the other tertiary and secondary neuropsychological deficits described by Rourke (1995). In addition, a number of M’s strengths are inconsistent with more recent descriptions of the developmental progression of NVLD (Rourke, 2000a). Finally, M’s social abilities are inconsistent with those described in Rourke’s (2000a) proposed ICD-10 criteria.
As
to M’s other neuropsychological abilities, his visual memory and visual motor
integration abilities are relatively intact and are therefore inconsistent with
the secondary deficits described by Rourke (1995). (These abilities may
also be inconsistent with a diagnosis of AS.)
His oral-motor praxis abilities were also average.
Finally, although M had mechanical arithmetic difficulties on the WRAT-3,
these difficulties did not result in deficits in mathematics in general as
described by Rourke (1995). The difficulties are consistent, however, with
descriptions by Badian (1983) of children with poor social skills and
anarithmetria.
Rourke’s
recent developmental definitions of NVLD suggest that most children with NVLD
manifest language deficits at an early age.
M did not. This age-of-onset
criterion, which is not present in earlier descriptions of NVLD, makes NVLD
appear more similar to Autism. See http://www.nldontheweb.org/Byron_Rourke_QA15.htm.
Rourke
has also proposed ICD-10 criteria for NVLD that include behavioral
characteristics of a distorted sense of time and extreme social impairment.
See http://www.nldontheweb.org/Byron_Rourke_QA15.htm
and http://www.nldontheweb.org/Byron_Rourke_QA18.htm.
How the social deficits in these criteria are qualitatively different
from those observed in Autism or AS is unclear.
M did not have a distorted sense of time (e.g., when asked how long it
took to tie his shoes, M replied, “For most kids they can do it in about 20
seconds, me it takes about two minutes.”)
M’s social deficits could also not be characterized as extreme.
M did have difficulty adapting to new situations, engaging in reciprocal
conversations and judging personal space distances. On the Personality Inventory
for Children (Wirt et al., 1977), however, he did not have elevated levels of
depression or withdrawal. His mood
lability resulted in a clinically significant elevation of psychosis. M could name some of the emotions exhibited in the Child and
Adolescent Social Perception Measure (Magill-Evans et al., 1995), although he
could not tell you how he knew what children in the scenes were feeling.
M’s subtle social deficits seem to be more like those of the subgroup
of children described by Johnson and Myklebust (1967) than those described by
Rourke (2000b).
Future
studies that attempt to distinguish AS and NVLD might investigate any
differences in social abilities between the two groups (Volkmar and Klin, 1998).
M’s social abilities were poor but they also seemed less compromised than
those typically observed in AS.
Rourke and Tsatsanis (2000) have stated that the psychosocial deficits
experienced by children who exhibit NVLD “are viewed as the direct
result of the interaction” of the neuropsychological assets and deficits (p.
237). The nonverbal cognitive
profile in AS and NVLD has also been described, however, as limiting a child’s
ability to make full use of social cues because it affects their ability to
process nonverbal stimuli in various modalities.
(Volkmar and Klin, 1998). Still
others have said that children with the NVLD profile may or may not exhibit
social deficits. (Pennington,
1991). Future research
should provide better descriptions of the nature and extent of the social
abilities in children with AS and NVLD. It
would also appear that M might not meet the criteria for PDD-NOS specified in
the practice parameters by the American Academy of Child and Adolescent
Psychiatry and the DSM-IV-TR because his social deficit is not severe and
pervasive. See http://www.guidelines.gov/VIEWS/summary.asp?guideline=001367.
We
are left to speculate as to whether the discrepancies in M’s verbal and
performance scores were relied on too heavily in indicating a diagnosis of NVLD. Given M’s history of poor motor development and current
test performance, it appears that most of his cognitive difficulties stem from
his motor dyspraxia. His social
difficulties are more appropriately addressed, however, by an NVLD diagnosis.
In the absence of social disability criteria independent of Autism, AS or
NVLD, an NVLD diagnosis is frequently used to fill the void and obtain the
necessary services.
In
sum, M meets some but not all of the proposed criteria for NVLD.
How many he meets depends on the description of NVLD one uses.
As this article has indicated, the changing and sometimes conflicting
protocols and descriptions of NVLD complicate the diagnostic process.
For
example, M meets at least five of the eight current criteria for NVLD or
“probable NVLD” as described in Rourke’s protocol for children slightly
older (nine) than M. See www.nldontheweb.org/Byron_Rourke_QA18.htm.
That protocol is separate from and less comprehensive than Rourke’s
qualitative descriptions at www.nldontheweb.org/Byron_Rourke_QA2.htm.
The neuropsychological diagnosis of NVLD is often complicated by the fact
that this protocol requires deficits on the Tactile Performance Test and the
Target Test, which are infrequently used in assessments.
Additionally,
M meets approximately five of the ten “characteristics” described in
children under the age of six, and seven of eight of the characteristics
described in children seven and above. See
http://www.nldontheweb.org/Byron_Rourke_QA15.htm.
Finally, he meets approximately 6 of 10 of the ICD-10 criteria proposed
by Rourke. See http://www.nldontheweb.org/Byron_Rourke_QA15.htm.
Qualitatively, M is more accurately captured by the descriptions of
children with social deficits and nonverbal disorders of learning provided by
Johnson and Myklebust (1967) and Badian (1983).
Although
the neuropsychological descriptions of children with AS and NVLD are similar,
behavioral criteria may be used to distinguish the two disorders.
The diagnosis is complicated, however, by three factors.
First,
at present, most clinicians see NVLD as a neuropsychological rather than
behavioral diagnosis.
Second,
the exact nature and causes of the behavioral and social deficits associated
with NVLD are poorly understood. In
fact, it is not clear that all children with NVLD have these deficits. According to Johnson and Myklebust (1967), children with
nonverbal deficits of learning such as visual-spatial difficulties may or may
not experience social deficits. Rourke’s
(1995) criteria do not seem to require a behavioral or social deficit, and, in
the vast majority of his work, he discusses social deficits primarily as a
consequence of neuropsychological problems.
Rourke’s (2000a) more recent descriptions, however, seem to include a
requirement of extreme social deficits for a diagnosis of NVLD.
Third, different definitions of NVLD carry with them different conclusions about the ability to distinguish it from AS. Rourke and Tsatsanis (2000) have noted that the NVLD description applies to children with AS. Rourke’s proposed ICD-10 criteria would seem to imply, however, that NVLD is a separate diagnostic entity from AS.
Given
the present state of research into and definitions of AS and NVLD, AS and NVLD
can most usefully be seen clinically -- as M’s diagnostic history indicates --
as behaviorally distinct with neuropsychological similarities.
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