Attention Deficits

Some features of poor attention or concentration, or over-activity affect many children with LKS at some point, and these may be associated with irritability and aggression (that is often towards particular family members) in some cases.

In the most severely affected, these features may be consistent with Attention Deficit Hyperactivity Disorder (ADHD) and the child’s ability to engage meaningfully with their environment is markedly compromised. However, in many cases the characteristics are much milder and may only be noticeable to close family members or teachers (the child is a bit more ‘bouncy’ than usual, has become slightly impulsive, or has difficulty sustaining concentration throughout a whole lesson). In others, the features are marked but episodic, for example, a couple of hours of overactive behaviour in the evening, or may be more pronounced in particular environments, for example, large gatherings where there is a high level of noise and stimulation. The most common features reported are: inattention, hyperactivity, impulsiveness (that is, not thinking before doing or saying something), no sense of danger, verbal and/or physical aggression, mood changes, and disinhibition (failure to inhibit inappropriate behaviour, for example, making rude comments to unfamiliar adults or pulling their trousers down in public).

It is often assumed that these behaviours are purely a response to the frustration felt by the child to the loss of language. Although most children with LKS do experience episodes of extreme frustration and confusion as a result of the condition, there is little evidence to suggest that this is the primary cause of ADHD type behaviours. For example, attention difficulties can present before there is any apparent language deficit. In addition, recovery of most areas of dysfunction, including behaviour, can occur even when significant language difficulty persists. It is therefore thought to be a direct result of the condition (see below). However, the social and emotional impact of a sudden loss of abilities should not be under-estimated and this factor will almost certainly contribute to behaviour patterns.

Most often, ADHD-type problems will show some improvement associated with improvement in control of the underlying seizure activity during sleep, and with recovery from regression (and conversely, deterioration in behaviour is found to be related to the disease worsening). In some cases, the behaviours will resolve completely and dramatically when the disease is effectively treated. In other instances where hyperactivity is very severe or persistent, it may respond to treatment with medication that specifically targets this group of disorders (for example, methylphenidate or Ritalin®). It is important to treat these ADHD-like difficulties in their own right, as they may prevent the child from using other skills to learn and interact. It is often most effective to use a combined approach through a behaviour programme and medication.

It is thought that these behaviours primarily result from interference with the brain’s normal functions, caused by the abnormal electrical activity that is associated with LKS (whether or not there are frequent overt seizures). This means that the child probably has very little control over these aspects of their behaviour.

However, there is a further acquired element that can also influence the occurrence of challenging behaviours. First, in children with a very longstanding disorder, poorly regulated behaviour may in part reflect the fact that one of the most important channels for teaching/learning such behavioural control (that is, oral communication) is not available. Second, through simple association children may ‘learn’ that some of these behaviours produce a desirable outcome, for example, if they have a tantrum and throw things around when the TV is turned off, then someone turns it on again. This means that the behaviour will then occur more frequently as it is ‘rewarded’ by the consequence. It is important that parents should be aware of this possibility and stick firmly to their pre-determined rules where possible and continue to provide as calm and structured an environment as possible. Although allowances must be made because of the involuntary nature of some of these behaviours, it is still important to make clear what is and is not acceptable, and to develop strategies to deal with common situations. Studies have shown that behaviour management techniques remain successful in helping this group of children, despite the fact that the behaviours have a significant organic component (that is, are due to the disease process, not simply a secondary psychological reaction to it).

Useful approaches include:

immediate and consistent responses to behaviour
time out
distraction techniques
rewards for positive behaviour and achievements.

Judging whether a child has control over their behaviour or not, can be very difficult, and the advice and input of a local clinical psychologist (often from the Child and Adolescent Mental Health Service or CAMHS) may be necessary to help resolve situations where behaviours have become very challenging. It is usually helpful to discuss these matters openly with the school, so that appropriate boundaries and responses to the behaviour can be agreed to ensure a consistent response.

In children with milder difficulties involving more ‘cognitive’ inattention and impulsivity these strategies may help:

playing games that require attention and memory to encourage these skills (there are many examples available, for example from Early Learning Centre®) – but particular attention should be paid to the appropriate level of difficulty so the child has the experience of achievement, not failure.
the parent counting to ten before responding to a situation that is upsetting
discussing basic rules to help with impulsivity – “Stop & Think”
creating simple visual mnemonics (memory prompts) to help remember important verbal information.