Application of a quantitative scoring system in the investigation of some hereditary neurologicaI disorders

Abstract

A scoring system for neurological signs based on the clinical examination was applied to a randomized population sample of adults and to three kindreds with hereditary neurological disease. To achieve qualitative properties of the system, nine subtests were defined, each constituting major functional units of the status. Age and sex differences in scores were corrected through the use of score prevalence figures in the normal population. Corrected wore distributions within subtests were rcmarkably simmilar, and justified their summation to a sum score.About 95 % of the population sample had sum scores under 3.5, which was defined as the limit of normality.In the kindreds examined, an unexpectedly large number of persons had “unspecific neuropathy”, i.e. high sum scores without the full clinical picture of the main disease. Various interpretations of this phenomenon are discussed.