Symposium for Patients & Caregivers

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

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

One Size Doesn’t Fit All

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

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

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

Aggression and Rage

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

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

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

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

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

So Why The Variability?

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

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

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

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”

Treatments

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

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

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

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

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

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

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”

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

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

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

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