We, as a community of patients and caregivers, have long struggled to understand the reasoning behind what we are told are conditions that are “likely” related to HH and which ones are “probably are not related”. We continue to struggle to understand the complex and challenging behaviors that we experience either as patients or witness in our loved ones every day. Hope for HH wants to change the dialog with the upcoming HH Comorbidity Survey that will be shared with this community in the coming months. We encourage everyone to participate in the survey and let your voice be heard!
Some of the challenging behaviors and conditions often reported by families and patients include:
• Autistic behaviors
• Anxiety – general and social
• Mood instability
• Obsessive Compulsive behaviors
While there have been some studies done in these areas over the last 10-15 years, the data is often limited. Perhaps because the focus has been on seizure control and advancing surgical treatment. Also, the number of centers seeing HH patients and including extensive neuropsychological evaluations as part of a comprehensive pre and post-surgical workup has been limited.
The topic of psychiatric and behavior related comorbidities is indeed complex. There are many factors that could come into play when trying to identify a cause and effect. Some of the factors include:
HH Syndrome itself –Two studies done on HH specifically reported increased rates of psychiatric behaviors. First, in Aggression and psychiatric comorbidity in children with hypothalamic hamartomas and their unaffected siblings it is stated: “Children with gelastic seizures and hypothalamic hamartomas displayed a statistically significant increase in comorbid psychiatric conditions, including oppositional defiant disorder (83.3%) and attention-deficit/hyperactivity disorder (75%). They also exhibited high rates of conduct disorder (33.3%), speech retardation/learning impairment (33.3%), and anxiety and mood disorders (16.7%). Significant rates of aggression were noted, with 58% of the seizure patients meeting criteria for the affective subtype of aggression and 30.5% having the predatory aggression subtype. Affective aggression was significantly more common (p < .05)”. Second, in Neuropsychiatric aspects of patients with hypothalamic hamartomas it is reported “Major neuropsychiatric symptoms comprised aggression that is only partial context dependent, compulsive behavior, psychotic symptoms not responding to treatment, and organic mood instability. HH should therefore be considered a neuropsychiatric syndrome with a highly variable expression that can be best captured by a thorough description of behaviors, symptoms, sequelae of epilepsy, and hypothalamic dysfunction.”
Epilepsy - known to affect behavior and has its own set of psychiatric comorbidities. In a recent paper titled Psychiatric comorbidities go unrecognized in patients with epilepsy: "You see what you know" it was reported “Patients with epilepsy (PWE) have a significantly higher prevalence of psychiatric comorbid disorders involving depression, anxiety, psychotic, and attention-deficit disorders compared with the general population or patients with other chronic medical conditions. Currently, there is no systematic approach in the evaluation and management of psychiatric comorbidities in these patients. In addition, neurologists are not trained to recognize these disorders, and consequently, they remain undertreated.”
Medications – many seizure medications have negative cognitive and behavioral side effects and HH patients are often on more than one at a time, further complicating potential interactions and toxicity. In a paper titled Epilepsy, Antiepileptic Drugs, and Aggression: An Evidence-Based Review, it is reported: “Behavioral side effects that have been associated with AEDs include depression, aberrant behaviors, and the development or worsening of irritability, impulsivity, anger, hostility, and aggression. Although prior reviews have focused on the associations between AEDs and depression or aberrant behaviors, the specific topic of aggression in response to AEDs has been largely neglected. We have endeavored in this evidence-based review to explore the neurobiology, epidemiology, presentation, clinical relevance, and management of issues relating to aggression in children, adolescents, and adults with newly diagnosed and chronic epilepsy exposed to a range of established and modern AEDs.
Chronic illness – just having a diagnosis of a chronic illness in general can affect mood, anxiety and feelings of helplessness. An excellent paper titled Emotional dimensions of chronic disease states: “Such patients must cope with a chronic condition and yet the emotional dimensions of these conditions are frequently overlooked when medical care is considered. Concepts such as the “sick role” and “illness behavior” have helped us understand the impact of disease and are familiar to most clinicians. Yet challenges still exist in the recognition and management of the psychological and social dimensions of chronic illness.”
With all of these factors coming into play for individuals with HH, it is easy to see why there is so much discussion among families as to what to do for their loved ones.
We as a community have an opportunity to have our voices heard and educate medical professionals. By sharing your experiences in the upcoming HH Comorbidity Survey, you can encourage more in-depth studies on HH and the entire spectrum of psychiatric comorbidities.
What behavior(s) do you or your loved one struggle with most? Let us know (firstname.lastname@example.org) so we can be sure to include it in the survey!