Dementia in Children
Background
Dementia in Children: Neurodevelopmental Regression in Children
Dementia is the more common term for neurodevelopmental regression. The majority of people with dementia are elderly people with such disorders as Alzheimer's disease, Frontotemporal Lobar degeneration including Frontotemporal Dementia and Multi-infarct Dementia (see entry, Dementias)
Conditions that involve dementia include:
- Adrenoleukodystrophy. Except in extremely rare cases, only boys have Adrenoleukodystrophy, a life limiting disorder, which leads to progressive dementia;
- Alexander disease. The infantile form of the disease leads to delayed development and dementia;
- Autism (Infantile) see entry Autism Spectrum disorders including Asperger syndrome. Within the first three years, children have a triad of poor social communication; delayed acquisition of speech or unusual use of language; circumscribed interests or repetitive behaviours. In a small proportion of children with autism, regression and loss of previously acquired skills occur. Usually not progressive;
- Batten disease (infantile): Neuronal Ceroid Lipofuscinosis 1. With an onset of between six months and two years, rapidly progresses with seizures, dementia, blindness and a severe loss of neurones;
- Batten disease (late infantile): Neuronal Ceroid Lipofuscinosis Type 2. With an onset of between two and four years, leads to seizures, blindness, loss of muscle co-ordination, mental deterioration and dementia;
- Batten disease (Juvenile): Neuronal Ceroid Lipofuscinosis Type 3. With an onset of between five and nine years, characterised by visual loss initially, and later seizures, loss of motor abilities and dementia;
- Brain injuries. Depending on the severity of the injury, there can be many possible long-term problems, such as permanent memory problems, speech problems, weakness of the limbs, fits, cognitive, developmental and emotional problems and occasionally dementia;
- Canavan disease. In early infancy, children begin to lose previously acquired skills including vision and hearing;
- Creutzfeldt-Jacob disease (CJD). Results in progressive neurological symptoms including dementia;
- Disintegrative disorder. Children usually develop normally in the first three years of age, but then gradually develop significant social communication difficulties and lose previously acquired skills, including cognitive, motor and social skills. Often progressive;
- Huntington's disease (Juvenile). The cognitive impairment is selective so, despite their difficulties with speech, patients may have a considerable degree of comprehension throughout the illness;
- Metabolic diseases. Dementia can be associated with a number of the very rare metabolic disorders not separately described in this list;
- Niemann-Pick disease Type C. Onset of dementia in a very varied life expectancy of between five and forty years;
- Rett syndrome. Although signs may not be initially obvious, it is present at birth but usually becomes more evident during the second year. Children with Rett syndrome are almost always profoundly and multiply disabled and dependent on others for all their needs throughout their lives but severity may vary considerably;
- Subacute-sclerosing Panencephalitis (SSPE). SSPE is a rare consequence of measles leading to a rapid or, in some cases, lengthy decline in function;
- Tay Sachs disease. A baby usually develops normally until about six months of age but the nervous system is progressively affected.For support and information, relating to these disorders, refer to the separate entries.
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