The epidemiology of primary degenerative dementia and related neurological disorders

Observation of cytopathological similarities between the changes of Alzheimer-type dementia, Parkinson's disease and motor neuron disease, as well as of some degree of clinical association between these conditions, has led to the suggestion that all three belong to a common class of degenerative neurological disorders, each of which as a rule first becomes manifest when age-related neuronal attrition is superimposed on subclinical damage caused by environmental noxae earlier in life. The importance of this model lies in its potential relevance to prevention. The epidemiological data reviewed here suggest that, while the three disease groups are all strongly linked with ageing, there may be major differences between their patterns of occurrence in populations, which make it doubtful if the same environmental pathogens are responsible in each instance. The most plausible unifying hypothesis at present is that the predisposing neuronal damage can be caused by a number of widely distributed metallic neurotoxins, each of which has a tendency to pick out specific areas or cell groups within the CNS and thus to give rise to distinct though overlapping clinical syndromes. The evidence bearing on this and other causal hypotheses is, however, still tenuous because of the scarcity of empirical data. Population-based case-control and cohort studies are called for, as part of a co-ordinated research endeavour.