Neuropathology of the cortical lesions of the Parkinsonian-dementia (PD) complex of Guam.

The Guam amyotrophic lateral sclerosis and Parkinsonian-dementia (ALS and PD) complex still presents a challenge to research. Previous papers have highlighted the lesions in the hippocampus and basal nuclei to explain the bizarre clinical syndrome. In our study of 20 to 62 cases of PD with or without ALS, significant widespread and often severe lesions were found throughout the entire cerebral cortex. These changes included variable degrees of nerve cell loss, spongy change, gliosis, increased lipofuscin content of nerve cells, the presence of granulovacuolar bodies and neurofibrillary tangles within neurones and, less frequently, Hirano bodies. The lesions show a characteristic topographical distribution. The global involvement of the cerebral cortex correlated well with the loss of higher mental function which was present in the patients.