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 short-term 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

• 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

• 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

• 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

• 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

• 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)• 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

• Strengths• Parent concerns• Evaluation data• Needs • Special factors (e.g.,

behavior)• English fluency• Extended School Year

(ESY)

• Other services• Modifications• Accommodations• Placement decisions

• 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_accom_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

• 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

• 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

• http://idea.ed.gov/• Part B: Ages 3-21• Part C: Ages birth – 2

• http://www.c-c-d.org/task_forces/education/IdeaUserGuide.pdf• PACER Center (

http://www.c-c-d.org/task_forces/education/IdeaUserGuide.pdf)• 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