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Congenital and Acquired Brain Damage and Dysfunction in Childhood

Congenital and acquired disorders

Congenital disorders i.e. present before birth
This broad category includes the following:

  • Early genetic defects with fixed effects such as chromosome abnormalities, primary microcephaly, Prader Willi and Angelman syndromes although in the latter plateauing or deterioration in development may occur with epilepsy;
  • Acute intrauterine events such as the early death of a twin with damage to the surviving fetus, a stroke causing hemiplegia, an early lack of blood supply causing damage to selective areas, for example perisylvian polymicrogyria and some children with Worster-Drought syndrome. Intrauterine infection with rubella, cytomegalovirus (CMV) and toxoplasmosis (see entries) come into this group but occasionally these may constitute a more long-term progressive illness, even going into extrauterine life, for example HIV and some cases of CMV;
  • Chronic intrauterine events such as Fetal Alcohol Spectrum disorder, anti-epileptic drugs, or maternal diabetes;
  • Progressive diseases with intrauterine onset such as some peroxisomal disorders (Zellwegers syndrome), early spinal muscular atrophy and Menkes disease, where although dysmorphic or motor abnormalities are present at birth the condition progresses, i.e. the child loses skills.

Acquired acutely in the perinatal period
This group includes the disorders that follow birth asphyxia, markedly pre-term birth, perinatal infection and haemorrhage i.e. causing a proportion of the cerebral palsies. It must however be emphasised that more causes of cerebral palsy are attributable to prenatal events and these causes are usually not identified. The conclusion that there was brain damage at the time of birth is often a very difficult one to be sure of. Quite often when a baby has prenatal abnormalities they will be associated with malpresentation and the baby may be in poor condition at birth, for example babies with dystrophia myotonica, with weak neck muscles, may produce a brow presentation which may cause a slow and difficult delivery.

In both congenital disorders and those acquired acutely in the perinatal period, the family predicament includes having to manage the sadness of not having the child they expected and also having to face uncertainties of diagnosis, prognosis and service provision.

View Background Background  |  Acquired acutely in childhood View Acquired acutely in childhood

Medical text written January 2002 by Professor B Neville. Last updated February 2007 by Professor B Neville, Professor of Childhood Epilepsy, Institute of Child Health, London, UK.

 

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