1	Investigating the Cognitive and Behavioral Characteristics of Children and Adolescents with Alternating Hemiplegia of Childhood Joshua Magleby, MS University of Utah
2	Neuropsychology Essentially, the study of the brain bases of behavior Involves the assessment of cognitive, adaptive, and behavioral abilities of an individual Aptitude, attention, memory, reasoning, processing speed, academic achievement, visual processing, auditory processing, problem solving, impulse control, etc. We have moved into the assessment phase of this study
3	Chicago Conference Cognitive assessment Motor and speech difficulties significantly impacted completion of some tasks Therefore these evaluations were considered invalid Rapid Object Naming (WJ-III) – 2 out of 4 subjects “cascaded” from 1^st line through 2^nd and to the 3^rd line
4	Rapid Naming A R N E D K S A R S D C R K C A N T D K A S C K E K S A S T K E A C K T
5	Chicago Conference Behavior assessment 26 subjects Average age = 9.5 years Three age scales: preschool (2.5-5.11), child (6.0-11.11), adolescent (12.0-18.11) Preliminary findings were not stratified; all three age scales were analyzed together Some behaviors are not valid for all ages
6	Chicago Conference “Clinically significant” Hyperactivity, atypicality, attention problems, leadership “At-risk” Depression, withdrawal, somatization “Average” AKA Typical Aggression, conduct problems, anxiety, adaptability, social skills
7	Chicago Conference Adaptive behaviors Vineland Adaptive Behavior Scale (Vineland) Communication, Social Skills, Daily Living Skills, Motor Skills (up to age 5.11) In general, all areas significantly below average No statistically significant relationship (patterns) between AHC, age, and adaptive skill However, social skills were generally elevated Vineland may not be specific enough to elicit patterns in adaptive behavior
8	SLC Conference – Cognitive 6 completed assessments, 3 not completed due to hemiplegic attack or inability to complete test items Cognitive Assessment Results Verbal IQ/Nonverbal IQ/Full Scale IQ – ranged from Borderline to Very Low General cognitive functioning was also borderline to very low Attention and Memory were significantly below average in comparison to same-age peers Motor Speed (Finger Tapping) was well below average bilaterally, and is probably not sensitive enough Visual processing was atypical
9	SLC Conference – Visual Processing Clock Drawing Test
10	SLC Conference – Visual Processing Out of 5 clocks successfully completed (basic parts intact), 4 of 5 were drawn with 1-12 on the right side of the clock only Visual neglect? Inability to pay attention to or notice stimuli from one-half of the visual field (i.e., the right or left side of a scene or object) even though more basic visual field abilities are intact. Often occurs on the left side of the visual world as the result of right parietal lobe damage. Planning deficit? Frontal/prefrontal lobe damage Other?
11	SLC Conference - Behavior Assessment results showed rated levels of behaviors similar to results from Chicago For adaptive functioning, switched to the Scales of Independent Behavior-Revised (SIB-R) Tends to be more specific than the VABS Subjects were rated anywhere from the age-appropriate to very low ranges Social skills were rated generally as age-appropriate Communication skills were rated generally as low Daily living skills and Motor skills were rated generally as low to very low
12	Goals Primary goals Continue with assessment stage Find neuropsychological patterns unique to AHC Brain localization if possible (e.g. left vs. right hemisphereàparticular lobeàparticular structures) Secondary goals Academic interventions Behavioral interventions Develop neuropsychological tests designed for use with motor impairments Other?
13	Aptitude AKA Intelligence “The aggregate or global capacity of the individual to act purposefully, to think rationally and to deal effectively with his environment” (Wechsler, 1958) “…mental activity involved in purposive adaptation to, shaping of, and selection of real-world environments relevant to one’s life” (Sternberg, 1986) As measured by an Intelligence Quotient (IQ) Can be difficult to assess in individuals with AHC due to motor and speech difficulties Age factors as well
14	Aptitude AKA Intelligence Verbal IQ - Peabody Picture Vocabulary Test-Third Edition (PPVT-III) Nonverbal IQ - Raven’s Progressive Colored Matrices Also is good at gauging overall general cognitive functioning Full Scale IQ - Wechsler Scales of Intelligence (WISC-IV, WPPSI-III) General Cognitive Functioning – Adapted from Folstein Mini-Mental Status Exam Orientation and Memory – Children’s Orientation and Amnesia Test (COAT)
15	Language New area of assessment Naming - Boston Naming Test Comprehension - Woodcock-Johnson Tests of Achievement-Third Ed. (WJ-III) Repetition - Test of One-Word Reading Expression (TOWRE) and Wide Range Assessment of Memory and Language-Second Ed. (WRAML-2) Fluency - Delis-Kaplan Executive Functioning Scales (D-KEFS) Rapid Object/Number/Letter Naming - Comprehensive Test of Phonological Processing (CTOPP) Phonological processing – CTOPP
16	Executive Functioning Higher-order abilities believed to be localized in the frontal lobes of the brain Reasoning, planning, strategizing, problem solving, inhibition, maintaining focus, cognitive flexibility, sequencing. Raven’s Progressive Colored Matrices D-KEFS Verbal Fluency Used to a certain degree in all other tests
17	Attention Attention is part of nearly every task one attempts Attentional difficulties are associated with most forms of brain injury and disease, congenital learning disabilities, and emotional or psychiatric disorders (Ponsford, 2000) Multidimensional (shifting, sustained, focused, working memory) Digit Span-Forward, CMS Faces, CVLT-C
18	Memory Three types: Short-term (primary memory store), Working (executive and attentional with limited capacity), Long-term (secondary memory store) Two modes: Recall and Recognition. Faces subtest from the Children’s Memory Scale (CMS) Digit Span-Backward subtest from the Wechsler Intelligence Scale for Children-Fourth Edition (WISC-IV) California Verbal Learning Test for Children (CVLT-C)
19	Visual Processing How visual stimuli are processed Visual processing – Clock Drawing Test Visual-motor integration - Beery-Buktenica Developmental Test of Visual-Motor Integration (VMI) Visual-motor precision – Developmental Neuropsychological Assessment (NEPSY) Visual scanning and recognition of objects - CTOPP
20	Processing Speed Appears to reflect the ability to employ a high degree of concentration and attention in processing information rapidly by scanning an array (Sattler, 1992) Very difficult to assess in AHC Coding subtest from WISC-IV/WPPSI-III for individuals without motor compromise
21	Academics Information learned through school experience Highly verbally loaded Subtests from the Woodcock-Johnson Tests of Achievement and Cognition, 3^rd Edition (WJ-III) Word decoding, reading comprehension, reading/math fluency, calculation
22	Behavior Are there “typical” behaviors in children with AHC? Behavior Assessment System for Children Externalizing behaviors: aggression, hyperactivity, conduct problems Internalizing behaviors: anxiety, depression, somatization Other clinical scales: atypicality, withdrawal, attention problems Adaptive scales: adaptability, social skills, leadership
23	Behavior continued… Scales of Independent Behavior-Revised (SIB-R) Assesses adaptive skills: communication, social, motor, daily living Specifies behaviors in these domains: language fluency, language production, gross motor, fine motor, dressing, eating, etc.
24	Neuropsychological Evaluations Approximately 2 hours Direct (testing) and indirect (parent ratings of skills) assessment Tests will be scored within the next month Scores will be sent along with a summary of the results within 6 weeks Results from SLC will also be sent at this time