This heterogeneous group of neurological diseases characterised by selective neuronal loss, is grouped together by the lack of known aetiology. As causes of such disease are identified (e.g. metabolic, viral) they have been reclassified in their appropriate category. Of the remaining conditions many are age related or familial and in some there is an identifiable genetic basis.

Characteristically these disorders:

- are gradually progressive

- are symmetrical (bilateral symptoms and signs)

- may affect one or several specific systems of the nervous system

- may demonstrate a specific pathology or just show neuronal atrophy and eventual loss without other features.

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