Neurofibrillary tangles in the primary motor cortex in Guamanian amyotrophic lateral sclerosis/parkinsonism-dementia complex

The amyotrophic lateral sclerosis/parkinsonism-dementia complex is a chronic neurodegenerative disorder with high prevalence among the native Chamorro population of Guam. The cortical pathology of the disease is characterized by the widespread occurrence of cortical neurofibrillary tangles that exhibit a specific laminar and regional distribution different from that seen in Alzheimer's disease (AD). In spite of the major motor symptomatology, the degree to which the primary motor cortex is affected in this disease has not been investigated in detail. We report here that the primary motor cortex in Guamanian cases contains high numbers of neurofibrillary tangles, contrasting sharply with the situation in AD and in non-Chamorro cases of amyotrophic lateral sclerosis. Furthermore, the cases with predominant parkinsonism-dementia are more severely affected than amyotrophic lateral sclerosis cases. These data suggest that the regional and cellular pathology of Guamanian cases differs radically from that commonly observed in neurodegenerative diseases outside Guam and point to the existence of subgroups in the spectrum of clinical manifestations seen in Guamanian patients.