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Epidemiological surveillance of amyotrophic lateral sclerosis and parkinsonism-dementia in the commonwealth of the Northern Mariana Islands.

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Epidemiolo,$cal Survedlance
of Amyotrophc Lateral Sclerosis
and Parhnsonism-Dementia
in the Commonwealth of
the Northern Mariana Islands
R. T. Yanagihara, MD, R. M. Garruto, PhD, and D. C. Gajdusek, MD
Amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia (PD), two fatal neurological diseases of unknown
cause, occur in high incidence among the Chamorro people of Guam, the largest and southernmost island within the
Mariana archipelago. To reassess and extend our present epidemiological knowledge of these degenerative diseases in
this focal geographical region, a systematic search for both disorders was conducted on the remaining inhabited islands
of Rota, Tinian, Saipan, and the four remote islands of Anatahan, Alamagan, Pagan, and Agrihan within the Marianas
chain. One case of ALS (on Saipan), 2 cases of PD (on Rota and Saipan), and 6 cases of parkinsonism without dementia (2
on Rota, 3 on Saipan, 1 on Tinian) were encountered among the approximately 17,000 inhabitants. No cases of either
ALS, PD, or parkinsonism were found in the four remote Northern Islands. An additional 22 cases of ALS and 8 cases of
PD were identified from reports of previous case-finding surveys, hospital records, and death certificates. Among
Chamorros born on Rota, the average annual age-adjusted mortality rates of ALS per 100,000population were 37.7 for
the 15-year period 1756 to 1770 and 22.5 for the past decade, 1771 to 1780. Among Chamorros born on Saipan, the
average annual mortality rates were 7.2 and 3.2 per 100,000, respectively, for the two periods. The mortality rates of PD
were also significantly lower on Saipan than on Rota. In general, the age-adjusted mortality rates of ALS and PD on
Rota were similar to those currently observed on Guam. Since the origins and current genotypic composition of
Chamorros on all the Mariana Islands are indistinguishable, the strikingly lower mortality rates of ALS and PD on
Saipan suggest that environmental factors are far more important than genetic factors in the pathogenesis of these
diseases.
Yanagihata RT, Gatruto RM, Gajdusek DC: Epidemiological surveillance of amyotrophic lateral sclerosis and
patkinsonism-dementiain the Commonwealth of the Northern Matiana Islands.
Ann Neutol 13:79-86, 1983
Previous epidemiological surveys have established the
unusually high incidence and mortality rates of amyotrophic lateral sclerosis (ALS) and parkinsonismdementia (PD) among the indigenous (Chamorro) people of Guam and Rota. Comparatively low incidence
rates, however, have been found among the Chamorros and Carolinians of Saipan, further to the north 113,
14, 20). In order to reassess and extend our current
epidemiological knowledge of ALS and PD within the
Mariana Islands, case-finding surveys were conducted
during 1980 and 1981 on Rota, Tinian, Saipan, and the
remote, more northerly islands of Anatahan, Alamagan, Pagan, and Agrihan. The surveillance included
extensive inquiry within all villages, repeated neuro-
logical examinations and follow-up of all patients suspected of having motor neuron disease or parkinsonian
syndromes, an evaluation of all death certificates, and a
careful review of all available medical records of individuals examined during previous case-finding surveys.
All examinations were conducted by neurologists from
the National Institute of Neurological and Communicative Disorders and Stroke (NINCDS) Research
Center on Guam.
One case each of ALS and PD on Saipan and one
case of PD on Rota were encountered in Chamorros
during the present survey. Six cases of parkinsonism
without dementia (P) ( 5 Chamorros, 1 Carolinian)
were also found. No cases of ALS, PD, or P were
From the Laboratory of Central Nervous System Studies, National
Institute of Neurological and Communicative Disorders and Stroke,
National Institutes of Health, Bethesda, MD.
Received Feb 24, 1982, and in revised form Apr 12. Accepted for
publication Apr 16, 1982.
Address reprint requests to Dr Yanagihara, Bldg 36, Room 5B-25,
National Institutes of Health, Bethesda, MD 20205.
0364-5 1341831010079-08$1.500 1982 by the American Neurological Association
79
/f
P
i
MARlANA
ISIANDS
lLPHILIPPINES
0
100km
AUSTRALIA
found among the inhabitants of Anatahan, Alamagan,
Pagan, and Agrihan. A n additional 22 patients with
ALS (20 Chamorros, 1 Carolinian, 1 Korean) and 8
with PD (all Chamorros) were identified from reports
of previous case-finding surveys, hospital records, and
death certificates. Although the average annual mortality rates of ALS among the Charnorros o n Rota and
Saipan have decreased during the past decade, the disparity between the rates of ALS a m o n g t h e inhabitants
of these islands has persisted. Since t h e Chamorro
community o f Saipan is composed entirely of direct
descendants o f Chamorros who migrated from Guam
and Rota, the persistently lower incidence and mortality rates of ALS and PD a m o n g Chamorros born o n
Saipan suggest that, rather than a primary genetic involvement, environmental factors o n Guam and Rota
are of major importance in t h e pathogenesis of both
disorders.
Geographical and Ethnic Background
The Mariana archipelago comprises 15 islands arranged in an
arcuate chain extending 672 km in the western Pacific Ocean
(13'14' and 20"33' north latitude, 1 4 4 7 4 ' and 156"05' east
longitude) with Farallon de Pajaros at the extreme northern
end of the chain and Guam at the extreme southern end (Fig
I). Although previously part of the Trust Territory of the
Pacific Islands, the islands north of Guam are now collectively referred to as the Commonwealth of the Northern
Mariana Islands. Geologically, the islands of Rota, Aguijan,
Tinian, and Saipan are similar to the northern part of Guam
in that they are composed of pyroclastic cores covered by
limestone terraces [31. In contrast, the more remote islands
80 Annals of Neurology
Vol 13 No 1 January 1983
Fig 1. Map of the uJesterrzPaczj'ic region depicting the geographical location of Guam and the Northern Mariana islands. The
inset shows the islands north of Guam where the current survey
for amyotrophic lateral sclerosis (ALS) and parkinsonismdementia (PD) was conducted.
extending northward from Saipan (collectively called the
Northern Islands) are geologically more recent in origin, and
are purely volcanic. With the exception of Pagan, which until
recently had a primitive grassy airstrip, they are accessible
only by sea.
According to the 1780population census of the Commonwealth of the Northern Mariana Islands, Rota, the fourth
largest island in the group (85.2 sq km), had a population of
1,274 people, almost exclusively Chamorro. Tinian, with a
land area of 101.8 sq km, had 899 people. The second largest
island in the chain, Saipan (120.7 sq km), had a population of
14,585 people: Chamorro, 9,200; Carolinian, 3,000; other
(Filipino, Korean, Japanese, Caucasian), 2,385. The four inhabited Northern Islands had a total population of 108 people (Pagan, 40; Alamagan, 22; Agrihan, 36; Anatahan, lo),
approximately 60% of whom were Chamorro and the remainder Carolinian E31). Earlier census figures from 1958
were as follows: Rota, 967; Tinian, 467; Saipan, 6,654 (1,300
Carolinians); the Northern Islands, 262 1321.
Genetically, phenotypically, and culturally, the Chamorros
of all the Northern Mariana Islands are indistinguishable
from the Chamorros of Guam [ l , 10-12, 17, 27) and are
culturally and biologically distinct from the Carolinians [lo,
18, 19,22,25]. Although marriages between Chamorros and
Carolinians are becoming more common (particularly on
Saipan), the two groups have generally maintained separate
ISLANDS
I
SAIPAN
1,
NORTHERN
ISLANDS
[TlNlAN
SAI PAN
/---
4
/
(
7
3
GUAM
GUA
A
B
Fig 2. (A) Forced migration of Chamorvos during the final yearf
of the Spanzsh-Chamorro w a n 11694-1 6981. 1B) Actzve resettlement of Saipan, Tinian, and the Northern Islands by
Guamanian Chamorvos from circa 1880 t o 1948.
communities with culturally distinct life-styles and endogamous marriage practices [251. At present, the Chamorros of
Rota maintain a traditional culture with a life-style that closely
resembles that of the Chamorros of southern Guam, where
ALS and PD are found in highest incidence [9, 131.
History of Chamorro and Carolinian Settlements
in the Mariana Islands
Although the precise origin of the aboriginal people of the
Mariana Islands remains uncertain, it is believed that they
migrated from the Indo-Malayan region circa 1530 BC [4,26,
28). The first known contact between native Chamorros and
Europeans occurred in 152 1 with the landing of Ferdinand
Magellan on Guam. In 1565, Andres Miguel Lopez de
Legazpi claimed the islands (known then as the Ladrone Islands) for Spain, but no attempt was made to colonize them
until 1668, when Father Diego Luis de Sanvitores, a Spanish
Jesuit, arrived to found a mission o n Guam. At the time of
his arrival, an estimated 40,000 to 100,000 Chamorros lived
throughout the islands, which he later renamed Mariana {25,
291. The ensuing three decades were marked by recurrent
warfare with the Spanish. Epidemics of influenza, measles,
and smallpox and destructive typhoons further reduced the
native population such that in 1698, with the conquest of the
Mariana Islands by Spain, the Chamorro population had been
almost exterminated. The first census conducted by the Spanish in 1710 revealed a population of only 3,539 Chamorros
[25, 291. The Chamorro population continued to decline,
reaching a nadir of less than 1,500 during the latter half of the
eighteenth century [291. It was during this period and the
ensuing 50 years that matings flourished between Chamorros
and members of other ethnic groups, particularly Filipino and
Spanish, resulting in the formation of a hybrid group of “neoChamorros” which repopulated the Mariana Islands { 1,2, 15,
19, 25, 30).
During the final years of the Spanish-Chamorro wars
(1694- 1698), the inhabitants of Rota, Tinian, Saipan, and the
Northern Islands were forcibly expelled and transported to
Guam (Fig 2A) 125, 291. However, a few hundred Chamorros on Rota managed to escape capture [21. Thus, Rota and
Guam are the only islands within the Mariana group which
have been occupied continuously by Chamorros.
For more than a century following this massive forced migration, the islands north of Rota remained uninhabited. Resettlement of these islands began in 1815 with the arrival on
Saipan of a small party of Carolinians who had fled the typhoon-devastated region of Lamotrek Atoll in the Western
Caroline Islands 12, 23, 251. Subsequently, other small
Yanagihara et al: ALS and PD in the Northern Mariana Jslands
81
groups of Carolinians arrived from Elato, Satawal, and Woleai
[17, 21, 251. It was not until the last two decades of the
nineteenth century, particularly during the German regime
(1877-1714), when Carolinians were already well established on Saipan, that Chamorros began to return in sizable
numbers from Guam, with a few also emigrating from Rota
(Fig 2B) [2, 251.
With the resettlement of Saipan already under way, similar
attempts were made on Tinian, but with much less success.
Permanent Chamorro settlements on Tinian were virtually
nonexistent until 1747 and 1948, when several hundred
Chamorros migrated to Tinian from Saipan and Yap, respectively (Fig 2B) [ 5 , 241. The Chamorro migrants from Yap had
gone there from Guam during the period of active population movement in the latter half of the nineteenth century.
Thus, all Chamorros currently residing o n Saipan and Tinian
are directly descended from the Chamorros of Guam.
During both the German and ensuing Japanese administrations of the Mariana Islands (1899-1944), Chamorro and
Carolinian settlers living on the islands north of Saipan were
engaged chiefly in the production of copra. In 1044, the
entire population of several hundred natives, along with several thousand Japanese soldiers, were transported to Saipan.
The second native resettlement of these islands began in
1948, when 25 Carolinian families (127 people) arrived on
Agrihan and 11 Chamorro families (57 people) arrived on
Alamagan. Three years later 10 Chamorro families (58 people) settled on Pagan, and in 1953, 12 Carolinian families (60
people) settled on Anatahan (Johnson JB: unpublished data,
1757). This ethnic composition and the formation of separate
endogamous communities have been preserved for the past
10 years, but the population has declined sharply with the
cessation of copra production following Typhoon Jean in
1768 {16}.
tients (15 ALS, 10 PD) was verified clinically by one or more
neurologists from the NINCDS Research Center. Only 3 of
the 23 ALS patients and 2 of the 10 PD patienrs were autopsied, and neuropathological findings were confirmatory in
these cases.
Epidemiological Findings
ROTA. During the present survey, 1 patient with P D
(Patient 29) and 2 with parkinsonism (Patients 34 and
35) were encountered (Table I). In the 25 years of
surveillance, 9 Rotanese Chamorros have died of ALS
and 5 of PD (Table 1). Of these, 6 ALS patients and all
P D patients lived their entire lives on Rota. The remaining 3 ALS patients spent at least the first 20 years
of life on Rota before migrating to a neighboring island
within the Mariana group, where onset of disease occurred.
The average annual mortality rate for ALS per
100,000 population (age-adjusted to the 1958 population of Rota) was 37.7 for the 15-year period 1956 to
1970 and 22.5 for the past decade, 1971 to 1980. For
PD, age-adjusted rates were 13.2 and 19.0 per
100,000, respectively, for the two periods (Table 2).
The mean age at onset of ALS on Rota was 50.9 years
(range, 40 to 64), with a mean duration of illness of 5.5
years (range, 1 to 17). Among P D patients on Rota, the
mean age at onset was 62.2 years (range, 5 5 to 70) with
a mean duration of illness of 4.6 years (range, 1 to 7).
Male to female sex ratios for ALS and PD among
Chamorros born on Rota were 4 :5 and 5 : 1, respectively.
No patients with either ALS or P D were
located on Tinian during the present survey. A 64year-old Chamorro man (Patient 39), who had migrated to Tinian 10 years earlier from Saipan, was noted
to have parkinsonism but without dementia. The only
well-documented cases of ALS on Tinian were those of
a 64-year-old Rota-born Chamorro who lived on Tinian for 25 years before onset of disease (Patient 9) and
a 56-year-old Korean migrant who lived 12 years on
Tinian and a total of 27 years in the Northern Mariana
Islands prior to onset of symptoms (Patient 10).
Neuropathological findings in the latter patient were
typical of classic ALS, lacking the prominent neurofibrillary changes usually seen in Chamorro cases.
Case Ascertainment
TINlAN.
With the establishment of the NINCDS Research Center on
Guam in 1956, medical and public health personnel on
Saipan and Rota have been aware of our interest in ALS and
P D and are able to recognize the clinical features of these
disorders. They have continued to refer suspected patienrs to
the Center and have provided vital logistical support during
earlier surveys C6, 13, 14, 201 as well as during the present
surveillance. Case-finding surveys of ALS and P D were conducted on Rota, Tinian, Saipan, and the four inhabited
Northern islands of the Mariana archipelago in April, May,
and October, 1980, and in March, 1981. Neurological examnations of suspected cases (uncovered through extensive inquiry within each village) and of referrals from local physicians, medical practitioners, or public health nurses were
performed by members of the NINCDS research team. In
addition, the medical records of cases found during previous
surveys and death certificates on Rota and Saipan for the past
25 years were reviewed. Cases identified from death
certificates were verified by locating and interviewing the
patients’ relatives as well as by reviewing existing medical
records.
All 33 patients (23 ALS, 10 PD) had anamnestic histories
strongly suggestive of ALS and P D (or lytico and raypat.
respectively, as the Chamorros of the Northern Marianas call
these familiar disorders). O f these, the diagnosis for 25 pa-
SAIPAN. One case each of ALS (Patient 22) and P D
(Patient 33) were examined on Saipan during the current survey (see Table l). In addition, 3 patients with
parkinsonism (Patients 36 to 38) were seen, 1 of whom
was a 72-year-old Carolinian who had lived on Saipan
since birth and had typical paralysis agitans with onset
of symptoms in 1960 (Patient 36). An additional 10
cases of ALS (9 Chamorros, 1 Carolinian) and 3 cases
of P D were identified from death certificates and clini-
82 Annals of Neurology
Vol 13 N o 1 January 1983
Table 1 , Clinical and Epidemiological Summary of Patients with Amyotrophic Lateral Sclerosis
and Parkinsonism-Dementia in the Commonwealth of the Northern Manana Islands
Birth
Patient NINCDS
No.
ID
Sex
Ethnicity
Onset of Disease
Place
~
~
Year
Place
Age
~~~
Migration Prior to Onset
Year
~
Place
~
~
Age
Years
Death
Age
Year
57
43
49
50
64
59
59
62
1957
1958
1958
1959
1967
1974
1969
1979
65
1975
58
1964
69
60
68
57
58
56
51
54
46
62
57
1971
1959
1061
1962
1965
1973
1956
1967
1976
1971
1958
~~
AMYOTROPHIC LATERAL SCLEROSIS
1
2
3
4
5
6
7
8
MDA
MJT
OMM
OJM
BAB
SSM
9028
9394
9480
F
F
M
M
Chamorro
Chamorro
Chamorro
Chamorro
Chamorro
Chamorro
Chamorro
Chamorro
Chamorro
Rota
Rota
Rota
Rota
Rota
Rota
Rota
Rota
Rota
1898
1915
1909
1909
1903
1915
1910
1917
1910
Rota
Rota
Rota
Rota
Rota
Rota
Saipan
Guam
Tinian
54
40
47
44
59
NA
42
57
64
1952
1955
1956
1953
1962
NA
1952
1974
1974
KJK
M
Korean
Korea
1905
Tinian
56
1961
11
922 1
M
Chamorro
Guam
1902
Rota
60
1962
12
13
14
15
16
17
18
19
20
21
22
SLT
MLG
RJC
CJT
CAM
AMF
MCC
9278
TJLG
WVD
9489
M
M
Saipan
Saipan
Saipan
Saipan
Saipan
Saipan
Saipan
Saipan
Guam
Truk
Alamagan
1899
1892
1905
1906
1917
1905
1913
1929
1909
1901
1944
Saipan
Saipan
Saipan
Saipan
Saipan
Kosrae
Rota
Guam
Sapan
Saipan
Saipan
57
68
50
M
M
M
F
M
M
M
M
Chamorro
Chamorro
Chamorro
Chamorro
Chamorro
Chamorro
Chamorro
Chamorro
Chamorro
Carolinian
Chamorro
53
46
50
41
60
57
34
1956
1960
1955
NA
1970
195 1
1963
1972
1969
1958
1978
23
GJM
M
Chamorro
Yap
1914
Saipan
51
1967
9
10
F
F
M
M
F
M
NA
Saipan
Guam
Saipan
Tinian
Saipan
Rota
Tinian
Saipan
Rota
22
42
37
39
29
Kosrae
Rota
Guam
Saipan
Saipan
Saipan
Alamagan
Australia
Saipan
25
22
27
28
7
< l ; 14
33
44
2
32
4
<1
18
20
15
2
25
4
11
12
30
28
21
28
I6
32
50
24
10
18
35
Alive
56
1970
63
70
67
71
61
Alive
60
75
78
Alive
1962
1962
1968
1976
1977
PARKINSONISM-DEMENTIA
24
25
26
27
28
29
30
31
32
33
MJD
2062
AJJT
MIA
QDS
9496
BJA
AJC
9284
9411
M
M
M
M
M
Rota
Rota
Rota
Rota
Rota
Rota
Saipan
Saipan
Saipan
Guam
Chamorro
Chamorro
Chamorro
Chamorro
Chamorro
C hamorro
Chamorro
Chamorro
Chamorro
ChamiJpn
F
M
M
F
F
1899
1892
1901
190s
1916
1914
1904
1891
1896
1907
Rota
Rota
Rota
Rota
Rota
Rota
Saipan
Saipan
Saipan
Saipan
1934
1911
1908
1017
1929
1916
Rota
Rota
Saipan
Saipan
Saipan
Tinian
59
65
60
70
55
64
56
68
73
68
1958
1957
1961
1975
1971
1978
1960
1959
1969
1975
44
69
52
63
50
64
1978
1980
1960
1980
1979
1980
Saipan
13
>45
1965
1966
1975
PARKINSONISM WITHOUT DEMENTIA
~
34
35
36
37
38
AMT
AAC
RP
ARM
MVM
EF
39
~~
F
Chamorro
Chamorro
Carolinian
Chamorro
Chamorro
Chamorro
M
M
F
M
M
~
NA
=
~~
~~
Rota
Rota
Saipan
Saipan
Saipan
Saipan
~
~~
~
Alamagan
26
11
Tinian
54
10
Alive
Alive
Alive
Alive
Alive
Alive
~
information not available, C h d J p n = half Chamorro, half Japanese
cal records (see Table 1). Of these, 8 patients were
born on Saipan, 1 on Yap, and 1 on Truk. Five of the 8
patients with ALS born on Saipan had onset of disease
there (Patients 12 through 16); 1 developed symptoms
on Kosrae (Patient 17), another on Rota (Patient 18),
and 1 on Guam (Patient 19). The lone Carolinian (Patient 21) developed ALS after long-term residence on
Saipan. All 3 patients with P D were born and had onset
of disease on Saipan (Patients 30 through 32).
The average annual mortality rates for ALS and PD
per 100,000 population (age-adjusted to the 1958
population of Saipan) were 7.2 and 2.4, respectively,
for the period 1956 to 1970, and 3.2 and 1.2, respectively, for the decade 1971 to 1980 (see Table 2). O n
Yanagihara e t al: ALS and PD in t h e N o r t h e r n Mariana Islands
83
Table 2. Average Annual Age-adjusted Mortality Rates of Amyotrophic Lateral Sclerosis and ParkinJonism-Dementia
in the Commonwealth of the Northern Marzana Islands, 1956-1 980
Amyotrophic Lateral Sclerosis
Ethnic
Island
Rota
Tinian
Group
Population
Size
1958
1980
Chamorro 969 1,274
Chamorro 467
899
Saipan
Chamorro 5,062 9,200
Carolinian 1,300 3,000
133
62
Northern Chamorro
129
46
Islands' Carolinian
No. of
Cases
Parkinsonism-Dementia
Mortality
No. of
Rated
Cases
Mortality
Rated
1956-70
1971-80
1956-70
1971-80
1956-70
1971-80
1956-70
1971-80
6
3
0
0
3b
0
0
0
37.7
0
7.2
5.1
0
0
22.5
0
3.2
0
0
0
3
0
3
0
1
0
0
0
13.2
0
2.4
0
0
0
19.0
0
1.2
0
6
1
0
0
2
0
0
0
0
0
"Age-adjusted by island and ethnic group to the 1958 population. Rates expressed per 100,000 population
bIncludes Patient 22 (Table l), who was born on Alamagan but lived most of his life on Saipan.
'Includes the islands of Anatahan, Alamagan, Pagan, and Agrihan
Saipan, the mean age at onset of ALS and P D was 53.3
years (range, 43 to 68) and 66.3 years (range, 56 to 73),
respectively, with a mean age at death of 56.8 years
(range, 46 to 68) for the ALS patients and 71 years
(range, 60 to 78) for the PD patients. A substantial
male predominance for both ALS (8: 1) and P D (2 : 1)
prevailed.
Only 26
of the 108 Chamorro and Carolinian inhabitants of the
Northern Islands were older than age 25, and none
exhibited features of motor neuron disease or of parkinsonism at the time of the survey. During the past 25
years, no patient with either ALS or PD has had onset
of symptoms while residing on one of these islands.
Although the single patient with ALS currently living
on Saipan (Patient 22) was born on Alamagan and
spent his childhood on both Pagan and Alamagan, his
infancy, most of his adolescence, and all of his adult life
have been spent on Saipan. His 63-year-old mother, a
native Saipanese Chamorro currently residing on
Saipan, presently has parkinsonism (Patient 37).
ANATAHAN, ALAMAGAN, PAGAN, AGRIHAN.
Discussion
The epidemiological surveillance of ALS and P D
among the inhabitants of the Commonwealth of the
Northern Mariana Islands indicates that incidence and
mortality rates for ALS on both Rota and Saipan are
declining, a trend currently observed on Guam [9, 20).
The actual number of verified cases of ALS on Rota
and Saipan have decreased by 50% during the past 10
years (177 1 to 1980),compared with the preceding 15year period (1956-1970). Similarly, only 93 cases of
ALS were diagnosed on Guam during 1771 to 1980,
compared with 174 cases during 1956 to 1970.
In addition, this investigation confirmed findings
from previous surveys regarding the disparity between
the mortality rates of ALS and P D among Chamorros
84 Annals of Neurology Vol 13 No 1 January 1983
on Rota and Saipan C13, 14, 20). Since the Chamorros
of Saipan are direct and recent descendants of Chamorros from Guam, incidence and mortality rates of ALS
and PD as high as those among their Guamanian antecedents might understandably be expected on
Saipan, if these diseases were primarily heritable in
nature. Yet the rates of both diseases among Chamorros on Saipan have been and continue to be remarkably
low. For ALS, the average annual age-adjusted mortality rate among Chamorros born on Saipan was nearly
equivalent to the crude annual death rate among firstgeneration Chamorro migrants from Guam (5 deaths
per 100,000 population) c7). It was also of the same
order of magnitude as the average annual mortality rate
for ALS among Filipino migrants with long-term residence on Guam C8). These data further support our
conclusion that environmental factors are of major importance in the pathogenesis of ALS in the Mariana
Islands.
Continued epidemiological surveillance of chronic
diseases among the nearly 8,000 Carolinians living in
the neighboring Yap district of the Western Caroline
Islands during the past two decades has not uncovered
cases of ALS or PD (Gajdusek DC: unpublished observations, 1780). Similarly, ALS and P D have not been
found in the small endogamous Carolinian migrant
communities on Guam. An extensive search for these
diseases in non-Chamorros, conducted in the Northern
Mariana and Caroline Islands from 1962 to 1965, disclosed 1 definite case of ALS in a Korean migrant on
Tinian, 2 cases of parkinsonism, and 6 suspected cases
of motor neuron disease among Carolinians on Saipan,
Ponape, and Truk [6}. The Korean case and perhaps
the single verified Carolinian case of ALS seen on
Saipan in 1958 [6) appear to have been mere chance
occurrences.
Of the 6 Carolinians suspected of having motor
neuron disease, all had onset of spasticity and weakness
between 1957 and 1964, and the 4 on Saipan were
examined on subsequent visits. Two died in 1969, 1 in
1971, and 1 in 1979. In each instance, the absence of
progression of neurological deficits or the appearance
of atypical features (such as sphincteric or sensory disturbance) or both made the diagnosis of ALS unlikely.
One of these patients, a 51-year-old man who had resided on Saipan since birth, was hospitalized in 1969 at
the Naval Regional Medical Center on Guam, where
he unexpectedly lapsed into a coma and died. An autopsy revealed pulmonary hemorrhage and metastatic
hepatic carcinoma. Neuropathological findings were
consistent with compressive myeloradiculopathy , not
ALS .
Carolinians, therefore, do not share with Chamorros
the unduly high prevalence of ALS and PD regardless
of their island of residence within the Mariana or
Caroline Islands. Their population in the Marianas,
however, is small: 3,000 in the Northern Marianas,
1,000 on Guam. Other ethnic groups, such as the everincreasing Filipino and Korean migrant communities
o n Guam and Saipan, deserve increasingly closer
scrutiny. Moreover, the continued epidemiological surveillance of the Chamorros of Guam and the offspring
of first-generation Guamanian Chamorro migrants to
the United States, as well as the successive generations
of Chamorros born on Saipan, will provide further evidence to support the contention that ALS and PD
in the Mariana Islands exemplify place-disease phenomena.
We are especially indebted to Drs Francisco Palacios, Jose Chong,
Benusto Kaipat, and Bernard You for continued logistical support
during the past two decades; to Drs Bromeo Mortera and Lawrence
Moher of the Dr. Torres Hospital on Saipan, Dr William Alto of the
Rota Hospital, and Juan Pangelinan of the Tinian Dispensary for
valuable assistance during the last year of the survey; to Dionicia
Hosono and Maria Atalig of the Public Health Center in Chalan
Kanoa, Saipan, for help in locating the relatives of deceased patients;
and to Dr Anton Golbou of Colonia, Yap, for help on Yap and the
outer islands of Yap district. We also thank Francisco C. Leon Guerrero, Jose M. Torres, and Drs Imaharu Nakano and Eiichiro Mukai
for their help in the field surveys and in the verification of cases. We
thank Dr Kwang-Ming Chen, D r Olivia T. Cruz, Manuel T. Cruz,
Luis T. Munoz, Mary E. Hernandez, and Frances T. Vickers of the
NINCDS Research Center for their many years of dedicated service
in the surveillance of neurological diseases in the western Pacific
region.
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