For reasons unknown, 50% of individuals with HH and epilepsy have worsening symptoms over time, including cognitive functioning and behavior. Over 80% of individuals with HH reported psychiatric symptoms and have been additionally diagnosed with the following:
- Oppositional Defiant Disorder – a continuing pattern of negative/angry/irritable mood with argumentative/defiant behavior to peers and people of authority.
- Attention Deficit Hyperactivity Disorder – ongoing and persistent difficulty with attention, excessive activity levels, and controlling impulsive behavior that interferes with daily activities.
- Anxiety – excessive and persistent worry that does not go away and may start to interfere with someone’s ability to complete daily activities
- Depression – persistent feelings of sadness or loss of interest that does not go away and interferes with daily activities.
- Conduct Disorder – a child or adolescent’s ongoing pattern of antisocial behavior and aggression towards others with serious violations of rules, which may include breaking the law.
- Mood Disorder – mood is distorted or inconsistent with the circumstances and interferes with a person’s ability to function.
- Autism Spectrum Disorders – developmental challenges in the ability to communicate and socialize, while also demonstrating repetitive/restrictive patterns of behavior or interests.
- Obsessive Compulsive Disorder – repeated and unwanted thoughts and/or the need to engage in certain behavior or perform a certain routine, all of which interferes with daily activities
- Auto-Aggressive Behavior – self harming behavior.
- Extreme decline in executive functioning – processing speed and short-term memory
Individuals with seizures due to HH commonly display a wide range of cognitive impairments. Impairments may range from slight to severe. Most prevalent are problems with working memory, long-term retrieval, and processing speed, as well as visual and verbal learning and memory. Other skills include: concentrating, controlling emotions, time management, and multitasking
The exact relationship between cognitive ability and the influence of seizure type, frequency, and onset is not well understood. There have been reported cases of patients with gelastic seizures and HH with normal cognitive functioning, yet these cases are less common. It is unclear at this point if size and attachment of the HH to the hypothalamus have a direct affect on cognitive functioning.
Compared to children, adults showing symptoms later in life appear to have less severe seizures, less severe learning and behavior difficulties, and better occupation and social connections.
Over the course of a patient’s journey, explosive anger can be one of the most challenging symptoms for the individual and the family. Specifically, children are often described as having behavior aggression with poor frustration tolerance. Approximately 20% of patients engage in these ‘rage attacks,’ which are further described as sudden, out-of-control bursts of anger that can start without warning or an obvious trigger.