Symposium for Patients
& Caregivers
Cognitive Impact of HH
(and what can we do about it)
Jennifer V. Wethe, Ph.D.*
Clinical Neuropsychologist
Hook Rehabilitation Outpatient Services
Community Hospital Network
Indianapolis, Indiana
*Formerly with Barrow Neurological Institute/SJHMC
Outline
• Cognitive functioning in individuals with epilepsy and HH
• Cognitive outcome of neurosurgical interventions for HH
• Interventions for cognitive difficulties
• Working with schools
Cognitive Functioning
Cognitive Functioning in Epilepsy
• Epilepsy is associated with impaired/abnormal cognitive
functioning
• High rates of mental retardation (MR) in patients with
childhood-onset epilepsy
• Increased risk of MR if intractible seizures with onset
during the first 2 years of life, especially if daily seizures
• Refractory epilepsy is associated with cognitive decline,
particularly in children
•
Bjornes et al., (2001), Dodrill (2004), Herman & Seidenberg (2007), Vasoncellos et
al., 2001
Cognitive Functioning in HH Patients
• Berkovic et al 1988
• 4 pediatric/adult patients with follow-up
• All had cognitive deficits with 3 showing deterioration
over time
• Frattali et al 2001
• All 8 children displayed cognitive deficits, ranging from
mild to severe
• Gelastic/CPS seizure frequency and severity correlated
with broad cognitive ability scores
• Relative weakness in long term retrieval and
information processing speed
• Relative strength in visual processing
Cognitive Functioning in HH Patients
• Harvey et al 2003
• 29 patients aged 4-23
• 72.4% of patients in series had intellectual disability
• Mullatti 2003
• 14 patients whose HH was discovered at age 16 or later. No
or minimal seizure difficulties
• Compared to series of younger patients:
• Fewer “learning difficulties,” although 2/14 had
moderate to severe learning difficulties and were in
residential care; 6 had “mild” learning difficulties
• More patients with “normal” IQ, although they may not
show typical patterns of cognitive functioning
• Fewer behavior problems
Cognitive Functioning in HH Patients
• Quiske et al 2006
• 13 juvenile and adult patients
• IQ ranged from moderate MR to good
• 54% had below average IQ
• Memory impaired in most patients-both verbal & visual
• Impairments in attention, executive systems functioning and
visuospatial abilities was common
• Regis et al 2006
• 27 patients aged 3 to 50
• Mental retardation in 30% and low average IQ in an
additional 26% of patients
• Difficulties with sustained attention, impulsivity,
disinhibition
Cognitive Functioning in HH
• Prigatano et al 2008
• 49 HH patients aged 5-55.
• Three patterns were identified
• Pattern 1: (“near normal”) average or above average IQ with no
significant verbal-nonverbal split (17 patients; 35%)
• Pattern 2: (“transitional”) Notable disparity between verbal and
visuospatial skills -- One at least 1 SD below mean with other
score normal (9 patients; 18%)
• Pattern 3a: Mentally retarded, but testable (16 patients; 33%))
• Pattern 3b: Mentally retarded, untestable (7 patients; 14%)
• Wethe, Prigatano et al
• 32 pediatric & adult patients evaluated prior to surgery
• Mean pre-surgical IQ in the low average (mildly impaired) range
• Mildly to moderately impaired new learning and memory
• Mildly to moderately impaired speed of processing
• Severely impaired mental flexibility (e.g., multi-tasking)
• Low average basic language and motor abilities
Cognitive Functioning in HH:
(Pre-surgical) Summary
• Highly variable, ranging from essentially or near normal to
profoundly impaired
• High proportion of mental retardation
• Abnormalities in cognitive functioning even in patients
with “normal” IQ
• Attention, memory, visuospatial skills, speed, mental
flexibility
• Individuals with later onset of seizure disorder (e.g., late
adolescent or adulthood) and less disabling seizures tend
to have better cognitive functioning
Surgical Outcome
Surgery
• Surgical advances in the treatment of HH have
been shown to improve seizure outcome, but little
is known about cognitive and behavioral outcome.
• HH is located deep within the brain and
neuroanatomical structures important for
memory may be placed at risk by the surgical
approach.
Outcome of GK Surgery
• Regis et al 2006
• 27 patients at least 3 years post GKS
• 59% had “dramatic behavioral and cognitive
improvement” and many had “developmental learning
acceleration at school” but details not provided
• No complaints of worsening cognitive abilities or shortterm memory complaint
• Mathieu et al 2010
• 9 patients aged 12-57
• Quality of life and verbal memory improved
Outcome of Interstitial Radiotherapy
Quiske et al 2007
14 adolescent and adult patients did not demonstrate any
significant cognitive changes 3 months following interstitial
radiotherapy
Outcome of Radiofrequency Thermocoagulation
• Kameyama et al 2009
• 25 patients aged 2-36 years
• 56% MR pre-surgery
• Intellectual improvement and resolution of behavior
disorder
Outcome of TC surgery
• Harvey et al 2003
• 29 patients aged 4-23
• 14 patients had early short-term memory impairment. This
persisted in 4 patients, 2 of which had undergone prior
surgery
• Ng et al 2006
• 26 patients (no formal post-op testing)
• Subjective report of improved cognitive functioning in 65%
of patients
• Transient post-operative memory impairment in 58%,
persisted in 8% (2 patients)
• Anderson and Rosenfeld 2010
• 4 of the patients
• Improvement in perceptual/visuospatial functioning
• ¾ patients showed decline in memory
Outcome of TC and Endoscopic Resection:
Barrow Series
• Pediatric and adult patients (3-39 yo; mean 12 yo) with
refractory epilepsy
• 11 TC; 20 Endoscopic, 1 combined
• Mostly sessile Type II HH (within 3rd ventricle)
• Early onset of epilepsy (most within 1st months, all by age
5)
• Mean follow-up interval was nearly 2 years (range 5 – 47
months)
Cognitive Outcome of TC and Endoscopic
Surgery: Barrow Series
• Performance on key and summary measures of intellectual
functioning was improved
• FSIQ (12): 83  91.3 (Range -1 to 18)
• Performance on measures of attention and speed was improved
• No clear pattern for memory outcome (no overall decline)
• List Learning (17): 32.2 29.9 (Range -29 to 28)
• List delayed recall (14): 30.4  24.4 (Range -25 to 17)
• Trend toward decline on delayed verbal recall (n.s.)
• Some patients improved their memory performance while
others clearly declined
• Patients with MRI Type III HH may be at greater risk of
memory decline than patients with MRI Type II HH
• Verbal Fluency and nondominant hand finger tapping improved
Outcome of TC and Endoscopic Surgery:
Barrow Series
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Younger patients and those with shorter
duration of epilepsy were more likely to improve
their intellectual functioning
Patients with mental retardation at pre-surgery
were more likely to have improved their
intellectual functioning post-surgery
Lower intellectual functioning and shorter
duration of epilepsy at time of surgery was
associated greater gains in intellectual
functioning at post-surgical follow-up
Complete seizure cessation not necessary for
cognitive gains
Cognitive Outcome Post Neurosurgical
Intervention: Key Points
• HH with refractory epilepsy is associated with cognitive decline
(epileptic encephalopathy). Successful neurosurgical
intervention can halt and even reverse the cognitive and
behavioral decline.
• Complete seizure cessation may not be necessary for
improvements to be observed.
• Temporary and permanent surgical complications are a risk
with the invasive approaches and may negatively impact
cognitive functioning (e.g., memory is an area of particular risk,
although some patients experience improved memory
functioning with successful surgery)
• Early intervention is important. Greatest gains with shortest
duration of epilepsy.
Interventions
Professional Assistance
• Cognitive Rehabilitation
• Speech therapy—address cognitive skills (e.g.,
attention, memory, problem solving) and
compensations
• Occupational therapy—Address activities of daily living,
cognitive skills-particularly as they relate to ADLs, and
compensations
• Neuropsychology
• Tutoring and special education assistance
Learning and Memory
• Types of long term memory
• Episodic
• Semantic – knowledge base
• Procedural
• Stages of learning and memory
• Attention
• Encoding - learning
• Storage – memory/retention
• Retrieval – use what has been learned; recall,
performance
Strategies for Severe Memory Impairment
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All these techniques rely on or can be used with errorless learning. They are used
with specific tasks and have poor generalization to other tasks.
Errorless learning
• “You teacher’s name is ____. What is your teacher’s name?”
• “A verb is an action word. What is a verb?”
Spaced retrieval
• Errorless learning combined with asking the individual to recall information
over progressively longer intervals (e.g., Immediate, 15 sec., 30 sec., 1 min.,
…days)
Chaining—Train individual to perform sequence of steps via procedural memory
• Each step serves as the cue to perform the next step. Errorless learning is used.
• Complex task broken down into series of discrete steps
• Train step 1. Then train step 1 with step 2, and so on.
• May be helpful for daily routines. E.g., brushing teeth, bathing, bedtime routine
• Haskins et al (2011)
Strategies for (Mild) Learning &
Memory Problems
• Mnemonics
• Association techniques
• Visual – Verbal Association or Schematics
• Visual Peg Method, Method of Loci
• Organization and Elaboration techniques
• First letter mnemonics (e.g., ROY G BIV- ex. of
chunking as well)
• PQRST (Preview, Question, Read, State, Test) – Good
for students
• Use of humor or storytelling
• Haskins et al (2011)
General Strategies to Facilitate Learning
(and Memory)
• Make it an active process
• Take notes, Organize the information
• Use multiple modalities
• Visualize—drawing, mental imagery
• Make meaningful, personalize
• Link to information already known
• Input  Output
• Frequent review and rehearsal
• Short repeated practice; build knowledge base
• even beyond the point of mastery – greatly increases
speed of processing
General Strategies to Facilitate
Learning and Memory
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Studying helps recognition, testing helps recall (e.g., flash cards)
Emotional enhancement
Use advance organizers
Context/state dependent learning—when possible learn,
practice in the environment where information/skill will be
needed.
• Healthy lifestyle
• Sleep
• Stress reduction
• Diet
• Exercise
Compensations / External Aids for
Memory and other Deficits
• Must be highly individualized
• Examples
• Calendars/memory notebooks/assignment books
• Can be checked and signed off on my teachers and
parents
• Schedules (pictoral or written)
• Procedural checklists
• Task checklists
• Electronic devices and reminders
• Organizers
Compensations / Interventions for
Attention Deficits
• Reduce distractions
• Make sure you have the individual’s attention
• Keep instructions short, simple and concrete. One step at a
time.
• Short practice/rehearsal sessions
• Consider training in attention and working memory (often
need involvement of therapist/individual/coach)
• Attention process training
• CogMed
• Lumosity.com
Working with Schools
Education
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504: Section 504 of the Rehabilitation Act of 1973
IDEA
IEP: Individual Educational Plan
504 Plan
Section 504 of the Rehabilitation
Act of 1973
• Protect the rights of individuals with disabilities in programs and
activities that receive federals funds…
• Physical or mental impairment that causes a substantial limitation on a
major life activity
• Requires schools to provide a “free appropriate public education” to
each qualified person with a disability
• An appropriate education could consist of education in regular classes,
education in regular classes with the use of supplementary services, or
special education and related services in separate classrooms for all or
portions of the day. Special education may include specially designed
instruction in classroom, at home, or in private or public institutions,
and may be accompanied by related services as speech therapy,
occupation therapy and physical therapy, and psychological
counseling and medical diagnostic services necessary to the child
Shepard, Leon, & Fowler (2009) www.acdl.com; www.ade.az.gov/ess
IDEA
• Individuals with Disabilities Education Act
• Free and appropriate education (FAPE)
• Child Find
• Special Education and related services tailored to
child’s unique needs
• Prepare for further education, employment, and
independent living
Eligibility Categories
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Autism (A)
Emotional Disability (ED)
Hearing Impairment (HI)
Mental Retardation
Multiple Disabilities (MD)
Multiple Disabilities—Severe
Sensory Impairment (MDSSI)
• Orthopedic Impairment (OI)
• Other Health Impairment
(OHI)
• Specific Learning Disability
(SLD)
• Speech Language Impairment
(SLI)
• Traumatic Brain Injury (TBI)
• Vision Impairment (VI)
• Preschool Moderate Delay
(PMD)
• Preschool Severe Delay (PSD)
• Preschool Speech/Language
Delay (PSL)
IDEA / IEP Process
• Family can request an initial evaluation (in
writing)
• Once the school district receives written parental
consent, they have 60 days to complete the
evaluation
• Can use outside sources of information
• Private school students: district in which the
school is located is responsible for performing the
evaluation, not the district of residence
IEP
• Describes how the school tailors education to meet child’s
unique needs
• How the school will provide related services (e.g., ST, OT,
PT, etc.) that are necessary for the child to benefit from
special education
Who attends the IEP?
• Multidisciplinary Evaluation Team (MET)
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Parents
Regular education teacher
Special education teacher
Representative of the public agency
Someone who can interpret test results and explain the
educational implications of tests
• If needed, additional individuals knowledgeable of the
student
• Student, if appropriate for transition services
Considerations / Elements in the IEP
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Strengths
Parent concerns
Evaluation data
Needs
Special factors (e.g.,
behavior)
• English fluency
• Extended School Year
(ESY)
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Other services
Modifications
Accommodations
Placement decisions
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Regular classes
Special classes
Special school
Home
Hospital/institution
• Least restrictive
environment (LRE)
Least restrictive environment (LRE)
• To the extent possible, children with disabilities are
educated with nondisabled children (mainstream)
• Use of supplementary aids and services to maintain
placement in regular classroom
504 Accommodation Plan
• Written plan listing the necessary accommodations to
minimize the impact of impairment
http://www.ade.az.gov/ess/
Accommodations
• Provisions made to allow a student to access and
demonstrate learning. These do not substantially change
the instructional level, the content, or the performance
criteria. The changes are made to provide the student
equal access to learning and equal opportunities to
demonstrate knowledge.
AZ Department of Education (2008)
• Examples of Accommodations:
http://www.osepideasthatwork.org/parentkit/school_acc
om_mods_eng.asp
Adaptations
• Changes made to the environment, curriculum, instruction,
and/or assessment practices for a student to be a
successful learner. Adaptations include accommodations
and modifications. Adaptations are based on an individual
student’s strengths and needs.
AZ Department of Education (2008)
Modifications
• Substantial changes in what a student is expected to learn
and to demonstrate. Changes may be made in the
instructional level, the content or the performance criteria.
Such changes are made to provide a student with
meaningful and productive learning experiences,
environments, and assessments based on individual needs
and abilities.
AZ Department of Education (2008)
Accommodation Examples
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Preferential seating
Additional time
Reduced distractions
Lecture outlines; copies of notes
Test format (font, recognition vs free response)
Mode of responding
Other Adaptations to Consider
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Quality over quantity
Open book, open note tests
Intermediate goals for longer assignments
Memory or assignment book checked by teachers
Use of an aid
Many children with HH have similarities to children ADHD
or Autistic Spectrum disorder. Similar strategies may be
useful.
Resources and References for
Parents/Students
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http://idea.ed.gov/
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http://www.c-c-d.org/task_forces/education/IdeaUserGuide.pdf
PACER Center (http://www.c-cd.org/task_forces/education/IdeaUserGuide.pdf)
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Part B: Ages 3-21
Part C: Ages birth – 2
FAPE (http://www.fape.org/)
http://www.help4adhd.org/en/education/rights/idea
Executive Skills in Children & Adolescents by Dawson & Guare
Late, Lost, & Unprepared by Cooper-Kahn & Dietzel
A Special Thanks to our Sponsors
• Aesculap
• Barrow Neurological Institute @ St. Joseph’s Hospital
• Barrow Neurological Institute @ Phoenix Children’s Hospital
• Great Council for the Improved
• Hope for Hypothalamic Hamartoma Foundation
• KARL STORZ Endoskope