Autofluorescence emission spectra of neuronal lipopigment in a case of adult-onset ceroidosis (Kufs' disease).

The study reports characteristics of the autofluorescence emission spectra from abnormal accumulations of intraneuronal lipopigment in a case of adult-onset neuronal ceroidosis (Kufs' disease). These are compared with emission spectra from 14 non-diseased brains, and from cases of childhood-onset neuronal ceroidoses (Batten's disease) and animal ceroidoses which have been previously reported. The emission spectra derived from the case of Kufs' disease could be distinguished from the spectra from the non-diseased brains and from the cases of Batten's disease and animal ceroidoses. The characteristics of an emission spectrum probably reflect the composition of the lipopigment, and spectral analysis may aid the identification of different pathogenic mechanisms which underly the various types of neuronal ceroidosis.