Behavior Series

Neurologic and Medical Influences on Behavior

Presented by Ron Thibert, DO
Pediatric Epilepsy Specialist and Director of the Angelman Syndrome Clinic at Massachusetts General Hospital in Boston, MA

This module aims to help you:

  • Understand how an increase in seizure activity might result in an increase OR decrease in aggression.
  • Describe the typical patterns of sleep problems in AS and management strategies.
  • Describe the common gastrointestinal, orthopedic and mobility issues in AS.
  • Understand how unrecognized pain may manifest as a direct or indirect change in behavior.

Seizure Activity and Behavior

The bottom line is that seizures have the potential to either exacerbate or suppress difficult behaviors depending on the individual, the seizure type, and the behavior. With an increase in epileptic activity in the brain, seizures can draw out more aggressive, hyperactive, and/or defiant behaviors. Our clinical experience has led us to believe that these changes may be related to discomfort, frustration, confusion, or fatigue in the setting of the seizures.

Conversely, we have also seen many children with AS in whom a decrease in seizure activity parallels an increase in difficult behaviors. This type of change is more common when the baseline seizure activity is quite severe, with epileptic activity occurring almost continuously throughout the day and/or night. This kind of persistent epileptic activity can create a sort of barrier between the individual and the outside world, and ultimately have a sedating effect. When this type of severe and persistent aberrant brain activity is suppressed with anticonvulsant medications or dietary therapy, challenging behaviors may in fact increase due to a heightened sensitivity and awareness of the outside world. 

One way to think about this is to imagine wearing earplugs, dark sunglasses, and mittens for six months straight. Over time, your brain would acclimate to this decreased level of awareness and sensory input. Seizure activity can act like those earplugs, sunglasses, and mittens, blocking out some of the noise and intensity of the outside world. Then imagine the doctor finds the right medication or combination of treatments to quiet the seizure activity. With this change, suddenly the earplugs come out and the sunglasses and mittens come off. The brain now can perceive and process information from the outside world to a much greater degree. For the individual, this kind of change may feel overwhelming or frightening, and parents and caregivers may see a corresponding increase in aggressive, hyperactive, or defiant behaviors. 

Tracking Seizures 

To further understand the relationship between an individual’s behavior and his or her seizure activity, it may be important to learn more about the timing and frequency of both the epileptic events and the episodes of challenging behavior. To help determine if seizure activity may be exacerbating an individual’s behavior, it is often helpful to gather data about when seizures are occurring during the day and/or night, and how the events relate to behavioral outbursts.

Example: John’s caregiver kept a log of both his seizure activity as well as his aggressive behavior over several weeks. Four mornings a week, just before breakfast John had a brief seizure. Over the course of two months, on average, at least three out of four of those mornings, less than two hours after the seizure John had a meltdown or displayed aggression towards his aide. In this case, the seizures themselves, or possibly how John felt after a seizure (his ‘post ictal’ state) may have been driving the behavioral outbreaks.

The more data we can gather about seizure timing and frequency, the more equipped you and your doctor will be to make informed treatment decisions, so as to optimize seizure control and potentially improve behavior.

www.seizuretracker.com is a helpful website for logging seizures over time. 

Seizures in AS can be quite challenging to treat, but it is very important for clinicians and parents to relentlessly pursue seizure control, because with time and sometimes multiple treatment trials, the majority of individuals will achieve a reduction in seizure frequency.

Individuals with AS, in most cases, should be routinely followed by a neurologist and/or epilepsy specialist who can work to optimize treatments as well as monitor for medication side effects and toxicities. Levetiracetam (Keppra), lamotrigine (Lamictal), and clobazam (Onfi) are three antiepileptic medications that we commonly use to treat epilepsy in the AS population; however, every person is different and treatments must always be uniquely tailored to the individual.

Additionally, we have had wonderful success with the treatment of AS-related seizures using dietary therapy. Specifically, high fat, low carbohydrate treatments, including the ketogenic diet and the low glycemic index treatment  (LGIT), have proven effective in AS.  For more information about diets, go to Resources and select Diet: Low-Glycemic Index Treatment (LGIT) & Ketogenic from the category drop down menu.

Common sleep issues

Difficulties may include:

  • trouble falling asleep
  • reduced total sleep time
  • frequent night wakings
  • irregular sleep-wake cycles

Fortunately, sleep problems tend to improve with age.

  

Sleep Improvement Strategies

There are many sleep treatment strategies to try to improve sleep:

  • improving an individual’s sleep hygiene
  • external sleep factors like bedtime routines
  • minimization of distractions in the sleep environment (sound, light, toys)
  • an increased exercise, and/or dietary modifications such as limiting sugar intake can improve sleep
  • medications have proven very helpful for sleep (discuss melatonin and gabapentin (Neurontin) with your neurologist)