Congenital and Acquired Brain Damage and Dysfunction in Childhood
Acquired acutely in childhood
This section includes children who suffer brain damage caused by meningitis, encephalitis, head injury (see entry Brain Injury), lack of blood supply during and acute illness, very severe epilepsy, acute metabolic diseases and stroke. This group of disorders is relatively straightforward to diagnose, with a period of normal development, an acute illness with dramatic loss of skills and a slow process of recovery with varying degrees of residual impairments. Occasionally epilepsy may mimic such a process and infantile spasms and Lennox-Gastaut syndrome may present acutely with regression that resembles an acute encephalitic illness but in such illnesses there may or may not be evidence of brain damage on a scan. If there is, it will not change, despite what may be a dramatic change in the child's level and style of functioning.
The parental predicaments in this situation include:
In all of the above situations it is important to emphasise that scans of the brain, now mostly MRI scans, are much more helpful in leading to a cause, i.e. a diagnosis, than in predicting outcome. In some cases such as Cortical malformation or almost total loss of one cerebral hemisphere (see entry, hemiplegia), the clinical condition can be predicted but normally that is not the case. This is illustrated by the fact that some of the most severely impaired children that we see have normal MRI scans.
Three impairments tend to go together, cognitive, behavioural and epilepsy and we broadly relate these to damage to cerebral cortical grey matter. Within the cerebral palsies different weighting of these impairments occurs so that when damage is to mainly cerebral white matter in diplegic cerebral of pre-term babies there is a relatively pure motor disorder. In contrast, some types of congenital hemiplegia, which have extensive cortical grey matter damage, the clinical picture is dominated by cognitive, behavioural and seizure impairments.
Epilepsy is a major influence upon cognitive, behavioural and sometimes motor outcome, which confounds attempts to predict the outcome from the pattern of brain damage seen on scanning. For example, in tuberous sclerosis there may be two children with similar scan appearances: one who had very early onset epilepsy with infantile spasms and the other who did not suffer epilepsy in the first two years of life. The former child has a high chance of moderate/severe cognitive impairment and autistic features and the latter is not at such risk. This process, by which epilepsy, particularly sub-clinical epilepsy in sleep, causes loss of skills and deviant behaviour, both of which may be permanent, is not well understood. It is known as an epileptic encephalopathy, meaning brain dysfunction caused by epilepsy and it may be partially or totally reversible by treatment or may be permanent. Such intercurrent events in any of the above groups of diseases can change the child's developmental trajectory without any change in the scan appearances, i.e. without evidence of additional brain damage. In some disorders, for example hemimegalencephaly and pyridoxine dependent epilepsy, the seizures and encephalopathy may start in the last trimester of pregnancy so that the stage for long-term impairments has already been set by the time the child is born.
Congenital and acquired disorders
| Progressive brain diseases ![]()