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Lytico-bodig disease
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Lytico-bodig disease
Lytico-Bodig (also Lytigo-bodig) disease, Guam disease, amyotrophic lateral sclerosis-parkinsonism-dementia complex (ALS-PDC), and Western Pacific amyotrophic lateral sclerosis-parkinsonism-dementia complex is a rare, terminal neurodegenerative disease of uncertain etiology endemic to the Chamorro people of the island of Guam in Micronesia. Lytigo and bodig are Chamorro language words for two different manifestations of the same condition. ALS-PDC, a term coined by Asao Hirano and colleagues in 1961, reflects its resemblance to amyotrophic lateral sclerosis (ALS), Parkinson's disease, and Alzheimer's disease.
First reports of the disease surfaced in three death certificates on Guam in 1904 which made some mention of paralysis. The frequency of cases grew amongst the Chamorro until it was the leading cause of adult death between 1945 and 1956. The incidence rate was 200 per 100,000 per year and it was 100 times more prevalent than in the rest of the world. Neurologist Oliver Sacks detailed this disease in his book The Island of the Colorblind. Sacks and Paul Alan Cox subsequently wrote that bats had been feeding on Federico nuts (Cycas micronesica) and concentrating β-methylamino-L-alanine (BMAA), a known neurotoxin, in their body fat. The hypothesis suggests that consumption of the bats by the Chamorro exposed them to BMAA, contributing to or causing their condition. Decline in consumption of the bats has been linked to a decline in the incidence of the disease.
Lytico-bodig disease presents itself in two ways:[citation needed]
As with bodig, the symptoms and forms of lytico present themselves differently from patient to patient.[citation needed] Patient presentations include muscle atrophy, maxillofacial paralysis, inability to speak or swallow and subsequent choking. Some patients retain mental lucidity throughout the illness until death, much like ALS patients.[citation needed]
Diaphragm and respiratory accessory muscles can become paralyzed, necessitating mechanical ventilation to facilitate breathing. Saliva must be suctioned from the mouth to prevent aspiration. This form of lytico-bodig is fatal in all cases.[citation needed]
No standard form of bodig has been reported and the documented cases of the disease manifested in many different clinical presentations.[citation needed]
The doctor visited a patient who had just suddenly come down with a virulent form. His symptoms had begun 18 months before, starting with a strange immobility and a loss of initiative and spontaneity; he found he had to make a huge effort to walk, to stand, and to make the least movement—his body was disobedient. The immobility attacked with frightening speed, and within a year, he was unable to stand alone and could not control his posture (2006).
—Oliver Sacks, The Island of the Colorblind, Vintage Books, 1996
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Lytico-bodig disease
Lytico-Bodig (also Lytigo-bodig) disease, Guam disease, amyotrophic lateral sclerosis-parkinsonism-dementia complex (ALS-PDC), and Western Pacific amyotrophic lateral sclerosis-parkinsonism-dementia complex is a rare, terminal neurodegenerative disease of uncertain etiology endemic to the Chamorro people of the island of Guam in Micronesia. Lytigo and bodig are Chamorro language words for two different manifestations of the same condition. ALS-PDC, a term coined by Asao Hirano and colleagues in 1961, reflects its resemblance to amyotrophic lateral sclerosis (ALS), Parkinson's disease, and Alzheimer's disease.
First reports of the disease surfaced in three death certificates on Guam in 1904 which made some mention of paralysis. The frequency of cases grew amongst the Chamorro until it was the leading cause of adult death between 1945 and 1956. The incidence rate was 200 per 100,000 per year and it was 100 times more prevalent than in the rest of the world. Neurologist Oliver Sacks detailed this disease in his book The Island of the Colorblind. Sacks and Paul Alan Cox subsequently wrote that bats had been feeding on Federico nuts (Cycas micronesica) and concentrating β-methylamino-L-alanine (BMAA), a known neurotoxin, in their body fat. The hypothesis suggests that consumption of the bats by the Chamorro exposed them to BMAA, contributing to or causing their condition. Decline in consumption of the bats has been linked to a decline in the incidence of the disease.
Lytico-bodig disease presents itself in two ways:[citation needed]
As with bodig, the symptoms and forms of lytico present themselves differently from patient to patient.[citation needed] Patient presentations include muscle atrophy, maxillofacial paralysis, inability to speak or swallow and subsequent choking. Some patients retain mental lucidity throughout the illness until death, much like ALS patients.[citation needed]
Diaphragm and respiratory accessory muscles can become paralyzed, necessitating mechanical ventilation to facilitate breathing. Saliva must be suctioned from the mouth to prevent aspiration. This form of lytico-bodig is fatal in all cases.[citation needed]
No standard form of bodig has been reported and the documented cases of the disease manifested in many different clinical presentations.[citation needed]
The doctor visited a patient who had just suddenly come down with a virulent form. His symptoms had begun 18 months before, starting with a strange immobility and a loss of initiative and spontaneity; he found he had to make a huge effort to walk, to stand, and to make the least movement—his body was disobedient. The immobility attacked with frightening speed, and within a year, he was unable to stand alone and could not control his posture (2006).
—Oliver Sacks, The Island of the Colorblind, Vintage Books, 1996