1. Symposium for Patients & Caregivers

2. The Impact of HH on Behavior and Mood: Rages, Depression, and Socialization
John B. Fulton, Ph.D.
Pediatric Clinical Neuropsychologist
Barrow Neurological Institute, Phoenix Children's Hospital

3. Outline Common emotional and behavioral difficulties with HH
Psychiatric diagnoses
Other emotional and behavioral difficulties
Factors that influence variability
Treatments

4. One Size Doesn’t Fit All

5. What Does the Hypothalamus Have to do With Behavior?
The hypothalamus receives input from the limbic structures, with output including corticotrophin-releasing hormones
Ultimately leading to release of cortisol
The limbic-hypothalamic-pituitary-adrenal (L-HPA) axis has been tied to psychiatric conditions (e.g. depression and anxiety)
The hypothalamus is heavily involved in the sympathetic nervous system
“fight or flight” readies the body for action in response to stressor
Sex hormones have well established behavioral influences

6. Psychiatric Conditions
Around 80% of individuals with HH and epilepsy meet criteria for a psychiatric condition
Common conditions include:
Attention Deficit Hyperactivity Disorder (AD/HD)
Oppositional Defiant / Conduct Disorder
Pervasive Developmental Disorders
More common in males?
Anxiety Disorders
Obsessive Compulsive Disorder
Depressive Disorders
Psychotic Disorders
Adjustment Disorders

7. Emotional and Behavioral Functioning
Not all behavioral difficulties fit into neat boxes
Confusion in terms
Cognitive disorder, NOS v.s. AD/HD
The same behavior could be representative of differing diagnoses
Examples of behaviors without a single label:
Rage and aggression
Emotional reactivity outside of mood disorder
Poor frustration tolerance
Social impairments that do not fit autism spectrum
Executive function impairments outside of AD/HD
Perseverative behavior

8. Aggression and Rage

9. Aggression / Rage
Aggression and rage commonly occur in HH (80% children in one study)
Rage Attacks
Often more disabling than seizures themselves
Abrupt rage, hitting, biting, kicking, etc.
Sometimes without an identifiable trigger
Can be followed by lack of awareness of event or immediate remorse
Rage attacks v.s. “typical” aggression
Rage attacks v.s. emotional reactivity

10. Aggression / Rage Types of Physical Aggression Affective Aggression:
Non-profitable damaging own property
Out of Control
Explosive
Seemingly without purpose
Predatory
Controls own behavior, while aggressive
Obtains something from aggression
Fights with weaker children
In HH more likely to be affective aggression
Sympathetic nervous system plays a greater role in this form

11. AD/HD Grouped into 3 subtypes:
Predominantly hyperactive
Predominantly inattentive
Combined
Extensive literature on “developmental” AD/HD
Primary seen as a disorder of executive functioning:
Sustained attention (vigilance)
Response inhibition (impulse control)
Behavioral activity

12. Autism Spectrum Disorders
3 main groups of symptoms
Impairments in social functioning
Impairments in language and communication
Stereotyped behaviors OR circumscribed interests
Autism Spectrum Disorders
Autism
Asperger’s Disorder
Pervasive Developmental Disorder, Not Otherwise Specified

13. Adjustment Disorders
Emotional or behavioral symptoms in response to a stressor
Subdivided into
Depressed mood
Anxiety
Anxiety and depressed mood
Disturbance of conduct
Mixed
Unspecified

14. So Why The Variability?

15. Neurological Localization and size of HH Seizure History?
Seizure types
Frequency
Refractory to treatment
Precocious puberty?
Age
Considered a progressive condition

16. How Does Cognition Impact Social Functioning?
Neurological Factors
Family, Environmental, Factors
Adapted from Yeates et al., 2007

17. Medication Effects
Variable outcomes for antiepileptic medication side effects
In some cases, increased agitation and irritability
Other AED’s can be used for mood stabilization
Monitor behavior in response to medications
Double edged sword given the impact of uncontrolled epilepsy on cognitive and behavioral outcomes

18. The Impact of School Functioning
Model of Learned Helplessness
Limited control to improve or reduce a stressor leads to depression and hopelessness
Chronic academic deficits may lead to beliefs that are:
Permanent “I will never do well in school”
Personal “I failed the test because I’m dumb”
Pervasive “Everything I do stinks”

19. Treatments

20. The Effects of Surgery
Multiple studies indicate a positive impact on behavior in the majority of cases
Surgical resection
Vagal nerve stimulation
Gamma knife
Interstitial stereotactic radiosurgery (I-seeds)
Subjective (e.g. physician or family impression)
Greatest improvement for patients with more substantial pre-surgical behavioral impairments
Behavioral improvements seen in a small group of patients without epilepsy

21. The Effects of Surgery More studies are needed
Degree of behavioral improvement?
Which behaviors have greatest improvement?
Relationship between seizure improvement and behavioral outcomes?
Need for objective assessment of behavior
Schulze-Bohnage et al. 2004
8 cases (4 children, 4 adults) with I-seeds placement
Improvements on a quality of life measure (adults)
Child Behavioral Checklist
1 child improvements in aggression, attention, and depression
1 child improved compulsive behavior, but not aggression, attention, depression
2 children no change

22. Psychopharmacology Treatments
ADHD
Stimulant medications (Ritalin)
Non-stimulant treatments (Strattera)
Mood stabilizing medications
Lithium
Some antiepileptic meds
Depression and Anxiety
SSRI’s (e.g. Prozac and Zoloft)
Benzodiazepines (anxiety)
Collaboration is key

23. Psychotherapy Treatments
Individual and family psychotherapy
Considerations include:
Age
Developmental level
Family support
Nature of the problem

24. Behavioral Treatments
Anger management
Behavioral analysis
Is the rage/aggression volitional
Antecedents >> Behavior >> Outcomes
Patient recognition
Identification of triggers
Labeling of physiological markers
Development of strategies for management
Reinforcement for using strategies
Response cost for inappropriate behaviors
The Explosive Child by Ross Greene

25. Behavioral Treatments
AD/HD
Behavior management of hyperactivity and impulsivity at home and school
Reinforcement for on task behavior
Response cost for inattentive, impulsive, or overactive behavior
Scheduling for optimal attention span
“Cognitive sprinter”
Taking Charge of AD/HD: The Complete Authoritative Guide for Parents by Russell Barkley

26. Behavioral Treatments
Anxiety and Depression
Cognitive behavioral therapy (School age children and older)
Behavioral activation (depression)
Systematic desensitization (anxiety)
Stress management techniques
Progressive muscle relaxation
Recognition and alteration of maladaptive thought processes
Example “Mindreading”

27. Behavioral Treatments
Social Impairments
Social skills groups
Friendship Training
Group interactions
Homework-supervised play dates
Social skills training
Navigating the Social World by Jeanette McAfee
Individual therapy

28. Behavioral Treatments
Autism
Applied Behavioral Analysis (ABA)
Provided in home and school
Intensive works best (20+ hours per week)
Earlier intervention is better
Treatment and Education of Autistic and Related Communication Handicapped Children (TEACCH)
Also rooted in behavioral intervention
Focuses on underlying deficits that contribute to behavior

29. Wrap Up
Emotional and behavioral outcomes from HH are variable, but often substantial
High rates of psychiatric illness, as well as behaviors not well defined by existing diagnoses
Neurological and environmental factors affect outcomes
Many treatments options for behaviors exist, but further research is needed to evaluate their efficacy

30. A Special Thanks to our Sponsors
Aesculap
Barrow Neurological St. Joseph’s Hospital
Barrow Neurological Phoenix Children’s Hospital
Great Council for the Improved
Hope for Hypothalamic Hamartoma Foundation
KARL STORZ Endoskope