Amyotrophic Lateral Sclerosis in the Mariana Islands

Guam ALS-PDC is a distinct double-prion disorder featuring both tau and Aβ prions

“…In the 1950s, investigators found that amyotrophic lateral sclerosis (ALS) among the Chamorros of Guam was clinically indistinguishable from ALS seen elsewhere ( 2 – 4 ). Door-to-door surveys for neurologic diseases among the Chamorro people revealed an additional disorder (or possibly a spectrum of disease) consisting of parkinsonism associated with severe progressive dementia (referred to as parkinsonism–dementia complex of Guam, or PDC) ( 5 ).…”

Guam ALS-PDC is a distinct double-prion disorder featuring both tau and Aβ prions

“…In the 1950s, investigators found that amyotrophic lateral sclerosis (ALS) among the Chamorros of Guam was clinically indistinguishable from ALS seen elsewhere ( 2 – 4 ). Door-to-door surveys for neurologic diseases among the Chamorro people revealed an additional disorder (or possibly a spectrum of disease) consisting of parkinsonism associated with severe progressive dementia (referred to as parkinsonism–dementia complex of Guam, or PDC) ( 5 ).…”

On the Familial Incidence of Amyotrophic Lateral Sclerosis

“…An analysis of data provided by the literature concerning the familial forms of ALS indicates that cases of dominant heredity are less frequent ( R o b i n s o n (1917), Montanaro & Lopez (1931), Dittel (1940) , Haberlandt (1950), Barrayuer-FerrC & Barrayuer-Bordas (1951), Koerner (1952), Kzirland & Mulder ( 1954, 1955), Kurland et al (1955Kurland et al ( , 1956, Boudin & Barbizet (1956), Kurland (1956Kurland ( , 1957 and others) while recessive heredity was probably present in most instances. In our cases, dominant heredity seems to have been involved.…”

“…Most authors are of the opinion that amyotrophic lateral sclerosis ( A M ) usually occurs sporadically while familial occurrence is rare ( N e r i (1925), Ottonello ( l 9 % 9 ) , Marhurg (1936), S c h a f f e r (1936), Lawyer & N e t s k y (1953) and others). Cases with a proved family history of ALS can be found in the literature, however, described by Fearnsides (1912), B r e m e r (1922), Kreyenberg (1930), Munch-Petersen (1931), Davidenkow (1932), Pamboukis (1932), Salus (1936), R u t e n i k (19381, Curschmann (1939), S c l i r o d e r (1946), Friedmann & Freedman (19501, Haberlandt (1950), Koerner (1952), v a n Bogaert & Radermecker (19541, K u r l m d & Mrrlder (1954, 1955, Hasarrts (1955), Kurland et al ( 1955Kurland et al ( , 1956, Kurland (1956Kurland ( , 1957, Perry & N e t s k y (1958) Alajouanine & Nick i 1959), Ronduelle, Rouyyues & Faveret (l959), Dierssen (1959) Faueret (195% Klaus et (11. ( l 9 5 9 ) , Buizina (1960), Espinosn rt al.…”

Guam amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) features CTE-like tau seeds in brain and spinal cord

“…Amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) is a rare neurodegenerative disorder of unknown cause that previously occurred in high incidence in the Pacific Island of Guam (Mariana Islands, US) (1)(2)(3)(4)(5) as well as other geographic foci in the western Pacific, i.e., the Kii peninsula of Japan (6,7) and the southern coastal region of Papua (8,9). The disease presents clinically as progressive motor neuron disease (ALS), parkinsonism with dementia (referred to as parkinsonism-dementia complex or PDC), or a combination thereof.…”

Guam ALS-PDC is a distinct double-prion disorder featuring both tau and Aβ prions

“…In the 1950s, investigators found that amyotrophic lateral sclerosis (ALS) among the Chamorros of Guam was clinically indistinguishable from ALS seen elsewhere ( 2 – 4 ). Door-to-door surveys for neurologic diseases among the Chamorro people revealed an additional disorder (or possibly a spectrum of disease) consisting of parkinsonism associated with severe progressive dementia (referred to as parkinsonism–dementia complex of Guam, or PDC) ( 5 ).…”

On the Familial Incidence of Amyotrophic Lateral Sclerosis

“…An analysis of data provided by the literature concerning the familial forms of ALS indicates that cases of dominant heredity are less frequent ( R o b i n s o n (1917), Montanaro & Lopez (1931), Dittel (1940) , Haberlandt (1950), Barrayuer-FerrC & Barrayuer-Bordas (1951), Koerner (1952), Kzirland & Mulder ( 1954, 1955), Kurland et al (1955Kurland et al ( , 1956, Boudin & Barbizet (1956), Kurland (1956Kurland ( , 1957 and others) while recessive heredity was probably present in most instances. In our cases, dominant heredity seems to have been involved.…”

“…Most authors are of the opinion that amyotrophic lateral sclerosis ( A M ) usually occurs sporadically while familial occurrence is rare ( N e r i (1925), Ottonello ( l 9 % 9 ) , Marhurg (1936), S c h a f f e r (1936), Lawyer & N e t s k y (1953) and others). Cases with a proved family history of ALS can be found in the literature, however, described by Fearnsides (1912), B r e m e r (1922), Kreyenberg (1930), Munch-Petersen (1931), Davidenkow (1932), Pamboukis (1932), Salus (1936), R u t e n i k (19381, Curschmann (1939), S c l i r o d e r (1946), Friedmann & Freedman (19501, Haberlandt (1950), Koerner (1952), v a n Bogaert & Radermecker (19541, K u r l m d & Mrrlder (1954, 1955, Hasarrts (1955), Kurland et al ( 1955Kurland et al ( , 1956, Kurland (1956Kurland ( , 1957, Perry & N e t s k y (1958) Alajouanine & Nick i 1959), Ronduelle, Rouyyues & Faveret (l959), Dierssen (1959) Faueret (195% Klaus et (11. ( l 9 5 9 ) , Buizina (1960), Espinosn rt al.…”

Guam amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) features CTE-like tau seeds in brain and spinal cord

“…Amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) is a rare neurodegenerative disorder of unknown cause that previously occurred in high incidence in the Pacific Island of Guam (Mariana Islands, US) (1)(2)(3)(4)(5) as well as other geographic foci in the western Pacific, i.e., the Kii peninsula of Japan (6,7) and the southern coastal region of Papua (8,9). The disease presents clinically as progressive motor neuron disease (ALS), parkinsonism with dementia (referred to as parkinsonism-dementia complex or PDC), or a combination thereof.…”

Guam ALS-PDC is a distinct double-prion disorder featuring both tau and Aβ prions

“…In the 1950s, investigators found that amyotrophic lateral sclerosis (ALS) among the Chamorros of Guam was clinically indistinguishable from ALS seen elsewhere ( 2 – 4 ). Door-to-door surveys for neurologic diseases among the Chamorro people revealed an additional disorder (or possibly a spectrum of disease) consisting of parkinsonism associated with severe progressive dementia (referred to as parkinsonism–dementia complex of Guam, or PDC) ( 5 ).…”

On the Familial Incidence of Amyotrophic Lateral Sclerosis

“…An analysis of data provided by the literature concerning the familial forms of ALS indicates that cases of dominant heredity are less frequent ( R o b i n s o n (1917), Montanaro & Lopez (1931), Dittel (1940) , Haberlandt (1950), Barrayuer-FerrC & Barrayuer-Bordas (1951), Koerner (1952), Kzirland & Mulder ( 1954, 1955), Kurland et al (1955Kurland et al ( , 1956, Boudin & Barbizet (1956), Kurland (1956Kurland ( , 1957 and others) while recessive heredity was probably present in most instances. In our cases, dominant heredity seems to have been involved.…”

“…Most authors are of the opinion that amyotrophic lateral sclerosis ( A M ) usually occurs sporadically while familial occurrence is rare ( N e r i (1925), Ottonello ( l 9 % 9 ) , Marhurg (1936), S c h a f f e r (1936), Lawyer & N e t s k y (1953) and others). Cases with a proved family history of ALS can be found in the literature, however, described by Fearnsides (1912), B r e m e r (1922), Kreyenberg (1930), Munch-Petersen (1931), Davidenkow (1932), Pamboukis (1932), Salus (1936), R u t e n i k (19381, Curschmann (1939), S c l i r o d e r (1946), Friedmann & Freedman (19501, Haberlandt (1950), Koerner (1952), v a n Bogaert & Radermecker (19541, K u r l m d & Mrrlder (1954, 1955, Hasarrts (1955), Kurland et al ( 1955Kurland et al ( , 1956, Kurland (1956Kurland ( , 1957, Perry & N e t s k y (1958) Alajouanine & Nick i 1959), Ronduelle, Rouyyues & Faveret (l959), Dierssen (1959) Faueret (195% Klaus et (11. ( l 9 5 9 ) , Buizina (1960), Espinosn rt al.…”

Guam amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) features CTE-like tau seeds in brain and spinal cord

“…Amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) is a rare neurodegenerative disorder of unknown cause that previously occurred in high incidence in the Pacific Island of Guam (Mariana Islands, US) (1)(2)(3)(4)(5) as well as other geographic foci in the western Pacific, i.e., the Kii peninsula of Japan (6,7) and the southern coastal region of Papua (8,9). The disease presents clinically as progressive motor neuron disease (ALS), parkinsonism with dementia (referred to as parkinsonism-dementia complex or PDC), or a combination thereof.…”