Amyotrophic lateral sclerosis-parkinsonism-dementia complex of Guam (Q86928)

A high prevalence focus of ALS and Parkinsons-dementia complex (PDC) has been noted in the Chamorro people of Guam. Patients presenting with motor-predominant disease display features consistent with classical ALS, and a smaller proportion of these patients may display superimposed dementia or parkinsonism. Patients with PDC commonly demonstrate manifestations of motor system dysfunction such as limb weakness, fasciculations and hyperreflexia. There are familial groupings of disease but genetic inheritance patterns have not been defined. Unique neuropathology has been identified with brain changes typical of ALS admixed with accumulations of neurofibrillary tangles. The incidence of ALS and PDC has fallen recently.