the last 15 years. The best known diseases affecting the indigenous Chamorro people are a type of amyotrophic lateral sclerosis (ALS), apparently unique to Guam,1 where, in addition to the classical histological changes of ALS, Alzheimer's neurofibrillary changes are present in specific parts of the central nervous system (CNS); and parkinsonism dementia complex (PD) in which the same neurofibrillary changes are consistently observed.2 At this point one may obviously ask whether these diseases are two distinct entities or are in some way related. This relationship has recently been discussed from the clinical3 and epidemiological4 stand-$ points. In this paper we would like to provide neuropathological evidence for such a relationship. For this purpose we have examined tissues associated with both diseases regardless of the principal clinical diagnosis.
MaterialMaterial utilized in the present study came from 111 cases from the Mariana Islands: 35 of ALS, 47 of PD or parkinsonism, and 29 controls (adult Chamorros who died on Guam with clinical diagnoses other than ALS or PD). The ALS and PD cases were collected during the past five years and do not include the 22 cases of ALS 1 or the 17 cases of PD 2 previously reported. ALS Cases.\p=m-\The condition of all 35 ALS patients from the Mariana Islands was clinically diagnosed during the four-year period through 1964, and they died in that same period. Typical ALS features were evident in 33 cases ; pyramidal signs were not found in two. In the majority of cases, there was no clinical evidence of mental or extrapyramidal dysfunction. There were 34 patients who were Chamorros from Guam. One non-Chamorro ALS patient was a Korean man who had been a resident of Saipan and Tinian of the Marianas for over 15 years. A family history of ALS was recorded in three cases. Of the patients 27 were men and 8 were women (3:1). The mean age of death was 50 years (range, 28 to 71). The mean duration of illness was 4.5 years (range, 2 to 11) ; 11 of the 35 patients had a duration of illness of five or more years.Among the 34 Guamanian cases, 5 were autopsied in 1961, 9 in 1962, 9 in 1963, and 11 in 1964. The brain and spinal cord from each autopsy were examined in all 35 cases. PD Cases.\p=m-\The 47 cases of PD complex or parkinsonism were clinically diagnosed and autopsied on Guam from the middle of 1960 through 1964. Of these, 4 were autopsied in 1960, 4 in 1961, 15 in 1962, 14 in 1963, and 10 in 1964. The condition of 39 patients was clinically diagnosed as PD ; in 24 of these patients, definite lower motor neuron deficits, signs of pyramidal tract involvement, or both were mentioned in the clinical record later in the course of the illness. In the eight other patients, clinical evidence of an organic mental syndrome was not observed,; six of these patients showed parkinsonian features alone, without obvious dementia or signs of ALS.Of the 47 cases 46 occurred among Chamorros on Guam. The one exception was a Filipino plumber who had been a resident of Guam for 17 years.A family hi...