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Congenital and Acquired Brain Damage and Dysfunction in Childhood

Progressive brain diseases

These conditions are many in number and diverse in their manifestations. The difficulty in recognising them as progressive diseases include the following:

  • They may start very early, especially prenatally, for example Zellweger's disease;
  • They may show loss of skills in one area of development, such as motor, with continuing improvement in other areas like cognition, for example spinal muscular atrophy;
  • Progression may be sufficiently slow that despite active disease the child gains skills in the area affected by the disease for some of the early years, for example Duchenne muscular dystrophy;
  • Some manifestations of the disease, such as myoclonic epilepsy in late infantile Batten disease, may be treatable;
  • The disease may have a tendency to show episodic deterioration with some incomplete recovery, for example adrenoleukodystrophy, ataxia-telangiectasia, mitochondrial diseases and some metabolic disorders disorders in which a (stepwise) deterioration may occur.

Recognition of these disorders is often on the presence of specific characteristic features, which are beyond the scope of this text. Otherwise careful assessment, follow-up and review of early data, including family videotapes, are the main methods used in trying to answer the question: is this a static or progressive disease? The majority of progressive conditions are genetically determined and although it is now possible to look for many of these conditions by genetic means the main diagnostic tools remain careful parental, educational and medical observations. We have to recognise that a 'diagnosis' which is in problem form (for example, moderate learning impairment with myoclonic epilepsy) is provisional and may stay that way for years. Only a limited amount of treatment is currently available for this group of conditions but more treatments will be coming into use, which will require objective methods of assessing outcome.

View Acquired acutely in childhood Acquired acutely in childhood  |  Overview of diagnosis View Overview of diagnosis

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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