Contributed by Dr Janice Forster
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| Dr Janice Forster, Pittsburgh Partnership |
PWS is known for hyperphagia and temper
outbursts. Researchers have discovered
that temper tantrums precede the typical food related behaviors in early
childhood. For all children, including
those who have PWS, tantrums occur when the
reward centers of the brain begin to mature.
The reward center enables all children to learn from experience and to
form expectations about things that happen in their world. When a child wants something and does not get
it, a tantrum occurs. Or, when something
occurs that is different from what the child is expecting, a tantrum
occurs. Among children who have PWS,
this tantrum behavior differs from typical children in 2 important ways:
- It is more intense
- The biochemical braking system to stop the tantrum is broken *
Each tantrum is unique to the child, but in
PWS it is usually very quick in onset and may appear to occur out of the
blue. If one looks carefully at the
antecedents of the situation from the perspective of the child, often the etiology
can be determined. Once a tantrum
begins, there is nothing that can be done to stop it, other than to keep
everyone and everything in the environment safe. The tantrum will run its course. After the tantrum is over, some children will
be exhausted and require a nap. Other
children will get back on track fairly quickly and resume their daily
activities; they may or may not acknowledge
their behavior.
The cortex of the brain not only governs
reward, but it is responsible for thinking and reasoning behavior. Another part of the brain is involved in
behaviors such as tantrums and shut downs.
The amygdala directs brain mechanisms associated with survival (fight,
flight or freeze); it is responsible for emotional expression, aggression, and
impulsivity. For this reason, the cortex
has been referred to as the ‘baby sitter’ of the amygdala. Although neural connections to the reward
center of the cortex begin around age 2, the thinking and reasoning behavior is
slower to mature in typical children and takes even longer to develop in
children with PWS. Using the analogy of
a computer, when a child has a full blown tantrum, their thinking and reasoning
ability in the cortex goes “off-line”.
This is why children with PWS appear to be in a ‘blind rage” during the
tantrum; they are literally behaving in survival mode, acting without thinking. In PWS it takes a while for the brain to
“reboot.” After they recover, they may act
as if nothing has happened (brain reboot), and they may or may not remember what
has occurred.
A tantrum is a learning experience for the
caregiver. The keys to successful
behavioral management are determining what
the trigger is, redesigning the daily plan to avoid it, and developing coping
strategies to deal more effectively with it.
[1] GABA (gamma-aminobutyric acid) is the major inhibitory
neurotransmitter in the brain; it regulates muscle tone and provides the
braking mechanism for behavior, allowing a person to “stop and think” before
acting. GABA receptors are reduced in
PWS, resulting in decreased GABA action in the brain that may also contribute
to epilepsy, anxiety disorders and depression.
