nonverbal learning disability & asperger’s disorder...

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NONVERBAL LEARNING DISABILITY & ASPERGER’S DISORDER LINDA C. CATERINO, PH.D., ABPP ARIZONA STATE UNIVERSITY

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NONVERBAL LEARNING DISABILITY

& ASPERGER’S DISORDER

LINDA C. CATERINO, PH.D., ABPPARIZONA STATE UNIVERSITY

Objectives

� 1. Participants will learn the specific neuropsychological characteristics of individuals with Nonverbal Learning Disabilities (NLD).

� 2. Participants will learn the current status of the support for the diagnosis of NLD.

� 3. Participants will learn the specific diagnostic criteria for Asperger’s Syndrome (AS).

� 4. Participants will learn about differential diagnosis regarding NLD and AS.

History - Gerstmann Syndrome

� Josef Gerstmann- Austrian-American neurologist

� Wrote 1st published article on Gerstmann Syndrome

� Symptoms

� Finger agnosia

� Right-left orientation confusion

� Agraphia

� Acalculia

Johnson & Myklebust

� Proposed a nonverbal type of disability characterized by absence of serious

language problems and adequate or above skills in reading and writing,

� Higher verbal than performance IQ

� Problems in visual-spatial processing

� Spatial orientation, right-left orientation, body image, motor learning

� Difficulties in temporal perception

� Handwriting

� Mathematics

� Distractible

� Perseveration

� Disinhibition

� Deficiencies in social perception - “unable to comprehend the significance of

many aspects of his environment”

� (Myklebust, 1967;Johnson &Myklebust, 1975; Boshes & Myklebust, 1964)

Byron Rourke

� Neuropsychology of Learning Disabilities: Essentials of Subtype Analysis (1985)

� Syndrome of Learning Disabilities: Neurodevelopmental Manifestations (1995)

� Identified a group of children with a pattern of neuropsychological strengths and weaknesses

Rourke’s Description of NLD

� Neuropsychological assets in auditory perception, auditory attention, and auditory memory, especially for verbal material.

� Adequate skills in rote verbal memory & language, amount of verbal associations and language output.

� Poor visual-spatial organizational, psychomotor, tactile-perceptual, and concept formation skills, simple motor skills may be well developed

� Academic assets = single-word reading & spelling.

� Academic difficulties in (e.g., arithmetic, science)

� Difficulties in informal learning (e.g., as transpires during play and other social situations).

� Psychosocial deficits, primarily of the internalized variety, are usually evident by late childhood and adolescence and into adulthood.

Cognitive/Neuropsychological

� Strengths

� VIQ>PIQ by 10-15 pts or more

� Good receptive& expressive language , High in Similarities

� Verbal Recognition, Repetition, Associations, Storage, Output

� Auditory Perception

� Verbal Attention & Rote Memory

� Focus on Details

� Simple motor skills intact

� Grip strength normal

� Finger tapping average

� Deficiencies

� PIQ<VIQ, Block Design, Object Assembly, Picture Arrangement& Coding

� Bilateral Tactile Perception (more on left )

� Finger localization, fingertip number writing

� Tactile Form Recognition & Memory

� Bilateral Visual Perception

� Visual Memory (particularly as figures become more complex)

� Visual discrimination/Visual figure ground

� Visual spatial organizational abilities

� Perseveration

� Attention

� Selective attention

� Novel Material

� Understanding the big picture

� Concept Formation

� Nonverbal Problem Solving

Specific NLD Proposed Criteria � (1) Less than two errors on simple tactile perception and suppression vs. finger agnosia, finger

dysgraphesthesia, and astereognosis. Simple composite greater than 1 SD below the mean. Simple tactile-perceptual skills are superior to complex tactile-perceptual skills.(2) WRAT /WRAT -R standard score for Reading is at least 8 points greater than Arithmetic. Single word reading is superior to mechanical arithmetic.(3) Two of WISC/WISC- R Vocabulary, Similarities, and Information are highest of the Verbal scale. Straightforward and/or rote verbal skills are superior to those involving more complex processing (e.g., Comprehension).

� (4) Two of WISC/WISC -R subtests Block Design, Object Assembly, and Coding subtests are the lowest of the Performance scale. Complex visual-spatial-organizational skills and speeded eye-hand coordination are impaired.(5) Target Test at least 1 SD below the mean. Memory for visual sequences is impaired.(6) Grip strength within one standard deviation of the mean or above vs. Grooved Pegboard Test greater than one standard deviation below the mean. Simple motor skills are superior to those involving complex eye-hand coordination, esp. under speeded conditions. (7) Tactual Performance Test Right, Left, and Both hand times become progressively worse vis-à-vis the norms. Complex tactile-perceptual and problem-solving skills under novel conditions are impaired.(8) WISC/WISC -R VIQ > PIQ by at least 10 points. Verbal skills are superior to visual-spatial-organizational skills.

� The following criteria are currently under investigation:7 or 8 of criteria = definite NLD

� 5 o 6 of criteria = probable NLD

� 3 or 4 of these criteria - Questionable NLD

� 1 or 2 of these features: Low Probability of NLD

Drummond, C. R., Ahmad, S. A., & Rourke, B. P. (2005). Rules for the classification of younger children with Nonverbal Learning Disabilities and Basic Phonological Processing Disabilities. Archives of Clinical Neuropsychology, 20, 171-182.

Rourke’s Proposed ICD Criteria

Bilateral deficits in tactile perception, usually more marked on the left side of the body.

Bilateral deficits in psychomotor coordination, usually more marked on the left side of the body.

Extremely impaired visual-spatial-organizational abilities.

Substantial difficulty in dealing with novel or complex information or situations. A strong tendency to rely on rote, routinized approaches and memorized responses (often inappropriate for the situation), and failure to learn or adjust responses according to potentially corrective informational feedback.

Impairments in nonverbal problem-solving, concept-formation, and hypothesis-testing.

Rourke’s Proposed ICD Diagnostic Criteria for NLD

Distorted sense of time. Estimating elapsed time over an interval and estimating time of day are both notably impaired.

Well-developed rote verbal abilities (e.g., single-word reading and spelling), frequently superior to age norms, in the context of notably poor reading comprehension abilities (particularly evident in older children).

High verbosity that is rote and repetitive, with content/meaning disorders of language and deficits in the functional/pragmatic dimensions of language.

Substantial deficits in mechanical arithmetic and reading comprehension relative to strengths in single-word reading and spelling.

Extreme deficits in social perception, social judgment, and social interaction, often leading to eventual social isolation/withdrawal. Easily overwhelmed in novel situations, with a marked tendency toward extreme anxiety, even panic, in such situations. High likelihood of developing internalized forms of psychopathology (e.g., depression) in late childhood and adolescence.

Primary Neuropsychological AssetsAuditory Perception -- Simple Motor

Rote Material

Tertiary Neuropsychological AssetsAuditory Memory --Verbal Memory

Secondary Neuropsychological AssetsAuditory Attention --Verbal Attention

Academic Assets Academic DeficitGraphomotor (late)/Word Decoding Graphomotor (Early)/Reading Comprehension

Spelling/ Verbatim Memory Mechanical Arithmetic/Mathematics Science

Socioemotional Assets Socioemotional Adaptive DeficitsAdaptation to Novelty/Activity Level

Social Competence/Emotional Stability

Verbal Neuropsychological Assets Phonology – Verbal Recognition

Verbal Repetition – Verbal StorageVerbal Associations – Verbal Output

Verbal Neuropsychological DeficitsOral-Motor Praxis-- Prosody

Phonology-Semantics --ContentPragmatics -- Function

Tertiary Neuropsychological DeficitsTactile Memory -- Visual Memory

Concept Formation -- Problem Solving

Primary Neuropsychological DeficitsTactile Perception – Visual Perception

Complex Psychomotor --- Novel Material

Secondary Neuropsychological Deficits Tactile Attention -- Visual Attention

Exploratory Behavior

Palombo – 4 Types of NLD

1) core deficits in processing complex and nonlinguistic perceptual tasks who also develop social problems (perceptual + social)

2) meet subtype 1and also have neuropsychological deficits in attention & executive functioning ( perceptual + social + EF)

3) meet subtype 1and have social cognition impairments that manifest in reciprocal social interactions, social communication and emotional functioning (perceptual + social + emotional)

4) meet subtype 2 and have same social cognition impairments as subtype 3 (perceptual + social + EF +

4 Subtypes of NLD

� Davis & Broitman (2011)

� All children with NLD have significant visual-spatial and executive function difficulties

� 1st Core subtype have visual-spatial executive functioning, mild social and academic difficulties

� 2nd Children with visual & executive functioning difficulties that significantly impact their social functioning

� 3rd Children with visual & executive functioning difficulties and significant academic problems

� 4th Children with visual-spatial, executive functioning, social and academic difficulties where all areas are functionally impaired

Davis & Broitman

4. All areas impaired

3. Visual-spatial

Executive

Functioning

Significant Academic Problems

1. Visual –Spatial

Executive functioning

Mild social & Academic

Difficulties

2. Visual-Spatial

Executive Functionin

g that significantl

y impact social

functioning

Hale & Fiorello (2004)

� 2 types

� Executive novel problem solving - frontal

� Posterior visual-spatial holistic problem solving- right hemisphere

Specific Characteristics -

Cognitive

� WISC-III - VIQ greater than PRI by 10 standard score points or more

� 80% have highest standard scores on Information, Similarities or Vocabulary

� 90% had lowest scores on Block Design, Object Assembly, Coding (Drummond, et al., 2005)

� Deficits in:

� Perceptual and Quantitative Reasoning,

� Theory of Mind

Attention

� Attentional Problems – maybe due to Visual Perceptual and tactile difficulties (Rourke, 2000)

� Auditory verbal attention intact

� Problems with Sustained Visual & Divided Attention

� Tend to be diagnosed with ADHD: Predominately Inattentive type (ADHD–PI) but may not be true ADHD (Semrud-Clikeman, 2007).

Executive Functioning

� Hypothesis generation

� Flexible problem solving;

� Self-monitoring;

� Behavior regulation;

� Integration of emotion with context

Speed and Efficiency of

Processing

� Assets -- auditory verbal including sustained auditory attention, reading speed for simple words, and rapid oral word association

� Deficits -- visual attention, tracking, matching, decision making speed, verbal-visual naming, color naming and writing speed

Memory & Learning

� Assets - auditory verbal learning and memory

� Deficits - in visual-visual verbal learning, visual immediate, visual spatial working memory, delayed and long term recall of visual information, Tactile-Kinesthetic Learning and Memory; Location Memory

Visual Processing Deficits

� Visual Perception;

� Scanning-Tracking;

� Figure-ground disturbance

� Visual-spatial

� Constructional

Sensory-Motor Deficits

� Tactile-Kinesthetic;

� Tactile Defensiveness;

� Motor Sequencing,

� Strength

� Gross motor coordination (skipping, bike riding, jump roping)

� Fine Motor coordination (handwriting, coloring, cutting, tracing, fastening, fine motor speed & dexterity)

� Complex psychomotor tasks,

Language

� Asset - but language is pedantic, rote

� Poor prosody and high verbal production

NLD Communication Difficulties

Receptive

May

not under-stand social

Nuances

in conver-sation

(lack

of

tact)

Problems

decoding

prosodic

or

vocal intonation

s

Problems

reading

facial expressio

ns and

gestures

May

lack

sense

of

humor

Concrete interpretation

of

metaphors

Expressive

Good vocabular

y

Lack

of

gestures, facial

expressions or

vocal intonations

Social Deficits

� Facial recognition and emotional expression (visual

attention/memory) (Corbett & Constantine, 2006; Fine, Semrud-

Clikeman, Butcher, & Walkowiak, 2008; Rourke, 2000)

� Emotional nuancing in communication; poor receptivity to

feeling states and states of others;

� Poor prosody and high verbal production does not promote

positive social feed back

� Social judgment secondary to problems with reasoning &

concept formation

� Adaptation to novelty

� Comprehension of humor deficits observed in NLD group with

social perceptual difficulties (not in NLD group with just visual-

spatial difficulties)(Semrud-Clikeman et al., 2008)

NLD Social Difficulties

Limited peer interaction, prefer

one-to-one interaction,, prefer

younger peers

Problems

forming

friendships

Difficulty

reading

social cues

Socially

awkward &

inappropriate

May

withdraw

or

isolate

Rigid,

rule-bound

Disruptive

play

Bossy,

aggressive

& defensive

with peers

Problems

forming

close

personal

attachments

Problems

with

interpersonal

intimacy

Problems

with

adaptability

Emotional

� At risk for internalizing problems, e.g., anxiety & depression (Rourke, 1989; Little, 1993)

� Depression (Cleaver & Whitman, 1998) Children with sxs of right hemisphere white matter dysfunction had arithmetic difficulties and showed significant levels of depression

� Petti et al. (2003) found that children with NLD had a higher percentage of internalizing diagnoses among children at psychiatric treatment facilities.

� Higher risk for suicidal behavior as compared to other LDs (Rourke,1989; Bigler,1989; Fletcher, 1989; Kowalchuk & King,1989) -based on extremely small

Emotional

� Greenham (1999) – evidence not clear if emotional problems are cause

or consequence

� Most individuals with NLD do not develop psychological problems, and

very few commit suicide (Greenham 1999).

� Bloom & Heath (2010) compared 23 12- to 15-year olds with NLD with 23

matching children with “general learning disability” (GLD) and 23 typicals

Self-report scale, an adolescent depression scale,

Facial affect recognition measure,

Ability to make six different facial expressions

Understanding of facial expressions.

GLD did more poorly on recognition, expression, and understanding of

emotions

NLD group did not differ from the typical group

Co-morbidity- ADHD

� Higher rates of ADHD (e.g., Gross-Tur, Shaleve, Manor, &

Amir, 1995; Voeller, 1986)

NLD Emotional Difficulties

Lack of empathy

or compassion

Self critical, perfectionistic,

lack self-confidence

Anxious, worried

Increased risk

for depression

& suicide

Problems modulating

affect

Easily frustrated, loses

control

Academic� Assets

� May initially have trouble with letter or number recognition & learning letter-sound relationships then become good readers

� May lose place in rdg.

� Good spelling skills

� Average or above average in verbal language skills

� Good syntax

� Good rote memories

� Problems generating ideas for essays, but

� Deficits� Arithmetic (rarely achieve >6th grade level,

even as adults (Rourke, 1995), number alignment

� Word problems (money, msmnt., esp. time–calendar & clock);

� Quantitative analysis & size, weight & distance estimation

� Physics , Hypothesis testing

� Organization

� Spatial representation of abstract nonverbal concepts (graphs, diagrams)

� Written expression early problems with orthograhic identification of letters spelling, graphomotor tactile and constructional deficits that affect legibility & accuracy

� Reading comprehension for complex reading material (e.g., main idea & inferences),problems understanding characters/motives

� Story Retelling, question response (Worling, Humphries, & Tannock, 1995)

Handwriting

NLD Assets and Liabilities

Domain Assets Liabilities

Sensory-Motor Auditory-Verbal Tactile-Kinesthetic; Tactile

Defensiveness; Motor Sequencing,

Coordination, strength

Attention Sustained Auditory;

Rote Recall, Verbal

Sustained Visual, Divided Attention

Visual-Spatial None Visual Perception; Scanning-Tracking;

Constructional

Language Verbal

Comprehension;

phonological;

Expressive,

receptive, and

repetitive

Prosody, Semantics; Content;

Memory & Learning Auditory-Verbal;

Learning & Memory

for Rote Information

Visual Immediate, Working; Delayed,

Recognition, Visual-Verbal Learning;

Tactile-Kinesthetic Learning and

Memory; Location Memory

NLD Assets & LiabilitiesDomain Asset Liability

Executive Verbal Problem Solving Hypothesis generation and

flexible problem solving; self-

monitoring; behavior

regulation; integration of

emotion with Context

Speed &

Efficiency of

Cognitive

Processing

Verbal-Auditory; Word Retrieval;

Rapid Verbal Word Association

Visual Tracking and Matching;

Verbal-Visual Naming; Color

Naming; Writing Speed

General Cognitive Verbal Reasoning Perceptual and Quantitative

Reasoning, Theory of Mind

Academic

Achievement

Literal Comprehension; Decoding;

Verbal Arithmetic; Spelling

Mechanical Arithmetic;

Science; Writing; Gist

Reading Comprehension

Social-Emotional General Knowledge; verbal skills Language Pragmatics;

Nonverbal Cues & Behavior;

Social Judgment

Early Signs of NLD 0-6 years

� A. Delays in reaching all developmental milestones, including acquisition of speech, followed by a late, but rapid development of speech & other verbal abilities (particularly rote skills), usually to above-average levels. May speak in a monotone.

B. Below normal amount of exploratory behavior. An apparent aversion for any type of exploration of new stimuli/situations.

C. Impaired development of complex psychomotor skills (e.g., climbing, walking).�

D. Avoidance of novelty & preference for highly familiar objects, situations, & information.�

E. Preference for receiving information in verbal as opposed to visual format.

� F. Strength in rote verbal memory (e.g., reciting the alphabet). Intelligence may be overestimated.

� G. Deficits in perception and attention in both the visual and tactile domains.

� H. Notably better auditory-verbal memory than visual or tactile memory.

� I. Initial problems in oral-motor praxis, and longstanding, mild difficulties in pronouncing complex, � polysyllabic words.

� J. Frequently described as “inattentive”.

Characteristic Evident in Older Children (> 7

Years)

A. Impaired capacity to analyze, organize, and synthesize information, with associated impairments in problem-solving

and concept-formation.

B. Despite high levels of verbosity, very significant impairments in language prosody, content, & pragmatics.

“Cocktail-party" speech patterns, with high volume of verbal output but relatively little content (meaning) & very poor

pragmatics.

C. Strengths in single-word reading/recognition , but may have problems with tracking (30% of NLD children need to

be retrained to read fluently) & reading comprehension.

D. Problems in arithmetic.

E. Fine motor problems, coloring, cutting,& handwriting in early school years, often improving to normal levels but

only with considerable practice.

F. Deficient social perception, social judgment, and social interaction. Poor perception & comprehension /

interpretation of facial expressions of emotion, and marked deficits in providing non-verbal communication signals.

G. May develop stress, anxiety, obsessional preoccupation, depression, self esteem, attentional problems (Palomobo

& Berenberg, 1999)

H. With advancing years, a tendency to become normoactive and then hypoactive. Problems in "attention" in formal

and informal learning environments tend to disappear as the situational stimulus and response demands become

more verbal in nature.

Middle & High School

A. Social skills deficits even more important, difficulty getting along with peers & teachers

B. Math & science even more challenging

C. More emphasis on executive functioning in written expression and reading

D. Increased risk for psychiatric disorders such as depression (Mokros, Poznanski, &Merrick, 1989)

E. May do well in learning a foreign language, drama, language arts

F. Transition planning becomes essential

Etiology

� NLD is the phenotype that may be due to various causes

� Hydrocephalus

� Agenesis of the Corpus callosum

� Turner’s Syndrome

� Fragile X

� Asperger’s Syndrome

� Williams Syndrome – similar strengths & weaknesses (Don,Schellenberg, &

Rourke, 1999) = WS lower cognitive

� Neurofibromatosis

� Velo-cardio-facial syndrome 22p-11q deletion (Lepach, A. C. & Peterman, F.

2011).

� leukomalacia

� spina bifida

Etiology

� Rourke presumed ‘right hemisphere’ dysfunction largely secondary to sub-cortical white matter differences and more association cortex

� Findings from existing neurological cases does suggest involvement of the right hemisphere and possibly of white matter (Voeller, 1995).

� Further study is needed to determine the validity of the right hemispheric white matter hypothesis in NLD.

Etiology

� Filley, 2001

� “the NLD syndrome remains a theoretical construct, and there is little documentation of right hemisphere damage in white (or gray) matter in children with this disability” (p. 262).

� “the relevance of the NLD syndrome to the behavioral neurology of white matter must remain conjectural” (p. 204).

� “careful correlation with neuroradiologic or neuropathologic data [is necessary] . . . to confirm or deny that white matter pathology is in fact present” (p. 262).

Etiology

� Semrud-Clikeman &Fine (2011) found greater number of cysts

on MRIs in children with NLD as compared to children with AS

& controls

� ¼ (7) of NLD had cysts or lesions, generally in the posterior

region--occipital/cerebellar or parietal regions (visual spatial

perception), equally in the right and left hemispheres and

bilateral in the cerebellum.

� Not found as frequently in children with Asperger’s syndrome or

with controls.

� One child w/Turner syndrome in the sample did not have a

cyst/lesion.

� May be a structural abnormality or a genetic disorder that

underlies the expression of nonverbal learning disability.

Asperger’s Syndrome

History

� Described in 1944 by a Viennese pediatrician, Hans Asperger (1906-1980), Asperger Syndrome (AS) “autistic psychopathy”

� Asperger identified a special group of children (4 boys aged 6 to 11) with normal cognitive & linguistic development, but poor social skills

� Impairment in nonverbal communication, vague facial expressions, limited gestures, limited, exaggerated or inappropriate language

� Verbal communication idiosyncratic, precise quality

� Misunderstanding of humor

� Intellectualization of affect

� Clumsiness & poor body awareness

� Special interests

� Conduct problems

� Familial patterns

� Gender patterns (more males)

� Increased anxiety & fear

Lorna Wing, MD

� British psychiatrist� Coined the term “Asperger’s syndrome” &

proposed formal diagnostic criteria� 1979 conducted an epidemiological study in

London�Triad of Impairments:

� Impairment of social interaction� Impairment of social communication

� Impairment of social imagination, flexible

thinking and imaginative play

DSM-IV-TR Diagnostic Criteria

� 299.80 Asperger’s Disorder� A. Qualitative impairment in social interaction, as manifested by at

least two of the following:

� 1. marked impairment in the use of multiple nonverbal behaviors such as eye-to-eye gaze, facial expression, body posture, and gestures to regulate social interaction

� 2. failure to develop peer relationships appropriate to developmental level

� 3. lack of spontaneous seeking to share enjoyment, interests, or achievements with other people

� 4. lack of social or emotional reciprocity

Diagnostic Criteria Continued…

� B. Restricted repetitive and stereotyped patterns of behavior, interests, and activities, as manifested by at least one of the following:

� 1. encompassing preoccupation with one or more stereotyped and restricted patterns of interest that is abnormal either in intensity or focus

� 2. apparently inflexible adherence to specific, nonfunctional routines or rituals

� 3. stereotyped and repetitive motor mannerisms

� 4. persistent preoccupation with parts of objects

Diagnostic Criteria

�C. The disturbance causes clinically significant impairment in social, occupational, or other important areas of functioning.

�D. No clinically significant general delay in language.

�E. No clinically significant delay in cognitive development or in the development of age appropriate self-help skills, adaptive behavior (other than in social interaction), and curiosity about the environment in childhood.

�F. Criteria are not met for another specific Pervasive Developmental Disorder or Schizophrenia.

AS - Social Impairments

May not like physical contact

Inability to engage in appropriate play

Extremely egocentric; self-

centered

Socially awkward Failure to develop developmentally appropriate peer

relationships

Lack of spontaneously

seeking enjoyment

Limited desire for social contact

Difficulty understanding other people’s expressions &

feelings

AS - Motor Impairments

Odd Motor Mannerisms

Delayed Acquisition of Motor Skills

Poor Manipulative

skills

Impaired mirror-image

imitation

Poor Coordination & Balance

AS - Sensory Impairments

Sensitivity to Pain and

Temperature

Sensitivity to taste and

texture of food

Tactile Sensitivity

Visual Sensitivity to

Certain Colors

Sound sensitivity- 3 types of noises:

• 1. sudden, unexpected noise (telephone ringing, dog barking, clicking of a pen)

• 2. high pitched, continuous noise (hair dryer, vacuum cleaner, mix-master)

• 3. confusing, multiple sounds (shopping centers, large classrooms)

Synaesthesia

• A sensation in one sensory system and as a result experiences a sensation in another modality• “Colored hearing” – seeing colors

when hearing a sound

AS – Language Impairments

Adult like speech/’little professors

May have no language delay; Well developed

speech but poor communication

Poor prosody, monotone, strange intonation, rate (too

fast), volume (too loud), poor fluency.

Poor ability to initiate and sustain

conversation with peers

Monologue type speech; egocentric

conversational style

Difficulty understanding metaphors/idioms/jokes

Lack of tact Echolalia

Emotional Co-morbidities

DepressionADHD

ODD Bipolar

Eating Disorders

Substance Use

Anxiety

(GAD, PTSD, Social Phobias, Phobias,

OCD, PTSD

Other Co-morbidities

Seizure

Disorders

Tourette’s

& Tics

Learning Disabilities

Developmental Coordination

DisorderHyperlexia

Other Characteristics

Rigidity in rituals & routines

Above Average Rote Memory

Executive Function Deficits

Intense Interest in circumscribed

subjects

� Nonverbal Learning Disability & Asperger’s Syndrome

NLD & AS

� NLD profile is often associated with AS (@80%), but AS is not always associated with NLD

� Individuals with AS present with virtually all characteristics of NLD (Rourke, 2000)

� Most children with AS have a NLD profile, but not all children

with NLD have AS

� Both NLD & AS have problems with social communication, and

reciprocity, nonverbal communication, pragmatic language,

and visual-spatial skills (Gunter, Ghaziuddin, & Ellis, 2002;

Voeller, 1995).

� Stronger verbal compared to performance skills on cognitive

testing were found for children with AS or NLD (Gillberg &

Billstedt, 2000; Klin et al., 1995).

NLD & AS

� Pennington (1991) –Rourke took 2 different groups & conflated them �1 group problems with spatial cognition�1 group problem with social emotional�only first group should be called NLD�Second group should be on autistic spectrum�Pennington (2009)reviewed NLD again

� “in sum, we do not have sufficient evidence to accept it as a valid learning disorder apart from either autism spectrum disorder (ASD), mathematics disorder (MD) or developmental coordination disorder (DCD) all of which are covered in the DSM-IV-TR (ASD in the category of PDDs)” (p. 248).

NLD vs. AS

� Palumbo (2001)- hypothesized that AS & NLD

could be 2 different subtypes of the same

syndrome

� Children with Asperger’s Syndrome have more

profound social difficulties than children with

developmental NLD (Thompson 1997).

� NLD & AS could be on a continuum of severity

ranging from NLD to Asperger’s and finally

autism (Roman, 1998)

NLD & AS (Klin, Sparrow,

Cicchetti, & Rourke, 1995).

� NLD & AD profiles share many characteristics

� Strong verbal skills

� Poor visual spatial ability

� Problems with executive functioning.

� NLD is evident in many with AS, but not in HFA

� The NLD profile is “an adequate model of neuropsychological assets and deficits encountered in individuals with AS (p. 1133)

� AS is one of several pathways including hydrocephalus, acquired brain injury and Williams Syndrome to the manifestation of NLD

NLD vs. AS

� Children with Asperger’s Syndrome typically

have “restricted interests,” where they

become obsessed with unusual interests

(Stein, Klin, & Miller 2004,

� The presence of stereotyped and restricted

patterns of interest and the need to adhere

to routines present in children with AS but

not NLD (Semrud-Clikeman, 2007).

NLD vs. AS

� Semrud-Clikeman, Walkowiak, Wilkinson, & Minne, 2010

� Compared 24 NLD, 52 AS, 27 ADHD 9- to 15-year-old children

� Found no significant differences in social perception between

the NLD & AS groups (both lower than controls)

� Differences between AS & NLD groups on calculations

� Differences on the Autism Diagnostic Interview–Revised,

particularly in the area of stereotyped and restricted behaviors.

� No significant difference for AS & NLD on Rey-Ostereith

Complex Figure test. Both lower than norms

NLD, AS & ADHD

� Semrud-Clikeman, Walkowiak, Wilkinson, & Christopher, 2010

� Compared NLD, AS, & ADHD-C & ADHD – PI

NLD & AS – No significant

differences

� Semi-structured interview (SIDAC)

� Behavioral Measures –BASC-2 Hyperactivity& Attention

� Social Skills – (SSRS)

� Math calculations

� Mathematics reasoning

� Reading recognition

� Fluid Reasoning (WJCOG-III) (Concept formation & Analysis & Synthesis)

� Motor Skills (Grooved Pegboard)

NLD & AS

� Visual-Spatial – NLD group significantly lower on

VMI than AS (Rey-Osterreith)

� Judgment of Line Orientation test (JLO) –NLD

lower than AS

� AS screener – AS group scored higher

� WASI –

� PIQ –NLD lower than AS

� VIQ - no significant difference among groups

� NLD group showing the largest split between VIQ & PIQ

NLD, AS, & ADHD

� 74% of NLDs had a V > P split of more than 15 SS points & 40% had a split of 25 SS points or higher (15-55)

� 63% of AS group had BIQ &PIQs within 15pts.

� But 37% of AS showed VIQ >PIQ in the AS group.

� While children with NLD are more likely to show a VIQ > PIQ split; there is a sizable minority of children who do not.

� Finding is not sufficient to provide a distinction between NLD & AS

� also found in Pelletier, Ahmad, & Rourke, 2001).

NLD vs. AS

• diagnosed from neuropsychological perspective

• Psychological test scores

• learning disability, discussion of how children learn

NLD

• diagnosed from psychiatric perspective

• observations

• developmental disorderAsperger’s

Prevalence

� .1 – 1%

� 10% (Ozols & Rourke, 1991

� 25% (Bender & Golden, 1990

� 29% (Van der Vluat, 1989)� Higher or equal rates in

females

� 1:1

� Recognized in 4th & 5th

grades

� 1-10 in 10,000

� Higher rates in Males

� Recognized around 3-4 yrs. of age

NLD AS

Neuropsychological

� VIQ > PIQ

� Weak visuospatial skills,

� Visual motor integration

� Right left confusion� Visual memory

deficits� Attentional problems� Hyperactivity as

child)

� May have Theory of Mind deficits

� VIQ>PIQ (X = 23.8 pts.) Ghaziuddin & Mountain-Kimchi (2004) found that only 82% of their AS group had VIQ > PIQ

� Weak visuospatial skills

� Visual motor integration

� Visual-spatial perception

� Nonverbal concept formation

� Visual memory deficits

� Attentional problems

� More likely to have Theory of Mind deficits

NLD AS

Visual Motor

� NLD� Psychomotor coordination

problems

� Clumsy, poor at sports

� Delayed Gross Motor Milestones

� Fine Motor difficulties, drawing, handwriting (Frankenberger, 2005)

� Avoidance of graphomotor tasks

� Difficulties dressing, problems with fasteners

� Poor organizational skills

� May not learn from diagrams, need verbal explanations (Fast, 2004)

� AS� Psychomotor

coordination problems

� Clumsy

� Fine and Gross Motor delays (Tantam, 1988; Gillberg,

1990)

� Stereotypic motor mannerisms (Mamen, 2007)

� Do not have visual issues & may be visual learners (Fast, 2004)

Language� NLD� Generally appropriate language

skills

� Verbal >performance

� Pragmatic language deficits

� Poor prosody

� Verbose, verbal with little content

� Lack of appreciation of incongruities and humor

� Poor nonverbal communication

� Difficulty understanding and using facial expressions, gestures

� AS� Generally appropriate language skills

� More verbose than NLD

� Verbal >Performance

� Pragmatic language deficits

� May have early language delays

� Poor prosody

� Monologues

� Verbose, large vocabulary

� Pedantic conversation, concrete speech

� Literal interpretation

� Poor nonverbal communication

� Difficulty understanding and using facial expressions, gestures, vocal intonation

� Repetitive speech patterns

Academics

Significantly

delayed arithmetic

skills (Rourke, 1989; 1995; Rourke &

Conway, 1997))

Average

or above

word reading

Delays

in

reading compre

-hensio

n

Difficulties in

written expression

Inconsistent reports

of arithmetic performance (some

show good math skills

(Kuipers, 1962)

Reading relatively

intact, may be

voracious readers

NLD AS

Social

� NLD

� Social delays

� Problems developing friendships

� At risk for peer rejection (Little, 2001)

� Poor social perception, judgment & interaction

� Problems in empathy

� Automatized stereotyped ways of

� AS� Social delays

� Problems developing friendships

� At risk for peer rejection (Little, 2001)

� Poor social perception, judgment and interaction

� Problems in empathy

� Lack of spontaneous sharing of enjoyment

� Failure to develop friendships

� Social problems more severe

Social -- NLD Vs.AS

Children with NLD are easier to make a

social connection with (Palumbo,

1991)

Withdrawal in NLD is reactive

Primary in Asperger’s

NLD children crave social contact more

than do children with Asperger’s

Children with NLD may ineptly reach out to other people

Emotional

� Emotional problems (internalizing anxiety & depression) ( Petti, Voelker, Shore &

Hayman-Abello, 2003)

� MMPI - NLD group had no scores above 70 on clinical scales, (Waldo et al., 1999)

� High risk for suicide ( Bigler,

1989; Fletcher, 1989; Rourke, Young, & Lennars, 1989)

� Have normal emotions, but can’t express them or recognizing them in others (Fast, 2004)

� Anxiety (esp. in adolescence) (Klin, 2004)

� Potential of mood disorders is high ( Ellis, Ellis, Fraser,

& Deb, 1994; Gujikawa, Kobayashi, Koga, & Murata, 1987)

� MMPI -2 study with adults elevations on L and Depression, social introversion, social discomfort (Ozonoff, Garcia, Clark &

Lainhart,2005)

� Have flatter affects (Fast, 2004)

NLD AS

Repetitive Behaviors

Inability to Adapt to Change

Inability to adapt to change/novelty

Preoccupation with stereotyped and

established routines

Ritualistic

Focus on special interest

Amass factual information

NLD AS

AS & NLD

Klin, Volkmar, Sparrow, Cicchetti,

& Rourke, 1995

• 21 students with AS (x= 16.11 yrs.) & 19 persons with HFA (X = 15.36 yrs.) were compared

• 18 of 21 with AS presented with NLD profile

Cederlund & Gillberg (2004)

• found that only 51% of AS subjects met criteria for NLD

Semrud-Clikeman

• NLD group did more poorly on PIQ than AS group using the WASI

Model

AS

Preoccupations

Special Interests

Facts

Ritualistic

NLD

Neuropsychological

Difficulties

Math

Reading Comprehension

Written Expression &

Organizational Problems

SocialCommunicationNonverbal CommunicationPsychomotorEmotionalVisual Motor Executive FunctioningTheory of MindAttentionNovelty Problems

Assessment

� Cognitive Assessment

� Thinking &reasoning skills must be at least average

� Although IQ does not have to be average (e.g., average WJIII

Thinking ability or Cognitive Efficiency)

� There must be an impairment in at least one psychological

process related to learning

Neuropsychological

Assessment

� Impairment in at least one psychological process related to learning

� Visual/motor/spatial/tactile memory and attention

� Visual motor or fine motor processing

� Speed

� Pragmatic language

� Executive functioning (e.g., planning, monitoring, selective focusing, organization

Possible Assessment Measures

� WISC-IV

� Woodcock-Johnson Cognitive Battery III (WJ-Cog III) (Woodcock, McGrew, & Mather,2001a) – esp. Analysis & Synthesis & Concept Formation)

� KABC-2(Chow & Skuy, 1999) found that NLD children showed significantly higher successive than simultaneous processing

� WASI

Possible Assessment Measures

� NEPSY (e.g., Design copying, Arrows)

� Delis-Kaplan Visual Motor Integration

(Beery)

� Wisconsin Card Sort (Jing, Wang, Yang, &

Chen, 2004)

� Category Test (Strang & Rourke, 1983)

� The Children’s Category Test (Boll, 1993)

Ris et al. (2007) - good predictor of the NLD

group (Ris et al., 2007)

Possible Assessment

Instruments

� Rey-Osterreith Complex Figure (Osterreith,

1944)

� Grooved Pegboard (Klove, 1963).

� Finger Tapping Test (Reitan & Wolfson, 1985).

� Purdue Pegboard

� Judgment of Line Orientation (JLO) (Benton,

Sivan, Hamsher, Varney, & Spreen, 2004)-

(Semrud-Clikeman, yes, not Benton, Varney, &

Hamsher, 1978)

� VMI (Beery et al., 2006).

Possible Assessment

Instruments

� Children’s Auditory Verbal Learning Test – 2 (CAVLT-2)

� Children’s Memory Scale

Possible Assessment

Instruments� AS Screener (AS screener based on DSM-IV-TR Asperger syndrome criteria)

� ADI-R

� Asperger Syndrome Diagnostic Scale (ASDS)

� Autism Spectrum Rating Scales (ASRS)

� The Structured Interview for Diagnostic Assessment of Children (SIDAC) (Puig-

Antich & Chambers, 1978). (K-SADS)

� Behavior Assessment System for Children–2 (BASC–2) (Reynolds & Kamphaus,

2004).

� Social Skills Rating Scale (SSRS) (Gresham & Elliott, 1990)/SSIS

� Child and Adolescent Social Perception (1995) (Semrud-Clikeman & Glass,2008)

� CBCL

� Conners Parent/Teacher Rating Scales

� Behavior Rating Inventory of Executive Function (BRIEF)

� Children’s Depression Inventory

� NLD scale http://www.nldline.com/

Possible Assessment

Instruments

� WJ-Ach III (Woodcock et al., 2001b) Math calculations &

� Math reasoning (Forrest, 2004) didn’t find that poor

mathematical reasoning predicted NLD

� WIAT-III

� Key Math

� Gray Oral Reading-4

Is NLD a Valid Diagnosis?

� Spreen (2011) NLD remains a hypothesis, but it should not be used in clinical practice unless it is supported by solid research findings.

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