Some interesting research conclusions

[Guest guest]

West Wing is doing reruns tonight (we get last week's program this week) so

I'll have time to update the list on some interesting research conclusions

related to Lytico-Bodig. L/B is one of the Parkinsons Plus diseases and

considered by some to be the worst. Lytico-Bodig is a unique disease found

almost entirely among Chamorros on Guam. The disease seemed to rise

especially among Chamorros born on Guam during and after WWII. Because

certain forms of the disease had aspects that were similar to Parkinsons,

Guam has attracted a number of famous neuro researchers. The thinking was

the disease was uniquely found among the indigenous population. It also

seemed to be localized to certain parts of the island. In the 50's, 60's and

70's this meant that the disease could be studied using demographic

techniques. The thought was that sharp demographic work would help determine

whether the disease had an environmental component or whether the disease

had a genetic component. It was also hoped that simple extrapolation would

lead to new insights about Parkinsons. One of the best known of the

demographic researchers was Steele who had become known before he came

to Guam as one of the two people who first described / discovered PSP. Dr.

Steele has spent many years in demographic work with L/B. So far nothing in

his data has made sense. A year or so ago I spent a couple of hours talking

with Dr. Steele about the life of a researcher and the lines of inquiry he

was continuing to study. A most interesting conversation.

More recently the University of Guam and some famous researchers from the

University of California at San Diego got together to study Lytico-Bodig

using the new genetic research techniques, looking at the genome and the

genes and chromosomes. Again it was an attempt to take the human mind where

it had never gone before. These researchers have been working on their study

for several years. I just spent an hour with the project manager of the

study. Their work is not yet published but she indicates that despite all

their effort, no genetic markers of any kind have been identified. Again the

disease baffles the best minds.

Most recently - so recently I don't have the reference - a reputable

neurological journal has published an article, apparently by people from

Hawaii, indicating that Lytico-Bodig is caused by eating fruit bats (locally

called fanihi) that have eaten certain kinds of palm nuts that contain

neuro-toxins. Again this is interesting. First of all, although they are

currently almost extinct on Guam, fruit bats are considered a great delicacy

and occasionally you will find a boiled fruit bat, wings, fur, ticks and

all, floating in coconut milk at a village fiesta. (No I have not tasted

them although I have been known to eat almost anything. I prefer pulling

pork from the whole roast pigs). Supposedly the bats pick up the toxin in

their proteins and that attacks the brains of those eating the bats. The

fact that fruit bats are now rare on Guam provides a convenient explanation

for why Lytico-Bodig is dying out on Guam.

Interestingly also, the nuts that are thought to be the cause of the problem

are the same kinds of nuts initially thought by some to be the cause of the

disease in some early theories. In these theories, during the most

difficult times in WWII, people would make a kind of flour from the nuts.

People were supposed to rinse the nuts in water to remove the toxins.

However during the war, so this theory went, some weren't able to rinse the

nuts well enough. However this version of the nut theory never held up in

the face of good demographic investigation.

I have not located the article so I can't really critique the statements

made on local radio and television. However the new theory apparently fails

also. Fruit bats are eaten all over the islands of the Pacific. The question

would be why Lytico-Bodig has only appeared on Guam. I don't know if this

theory will bite the dust (or sea water) but it will be interesting to

follow up on what is actually being proposed.

Many tasty fruit bats to you all.

Peg and Jim from Guam

********************************

*** Peg & Jim

*** # 29 Cruz Heights

*** Ipan-Talofofo, Guam 96930-4736

*** USA

***

*** Note: Guam is 15 hours ahead of

*** Eastern Standard Time (EST).

*** 14 ahead of EDT.

********************************

[Guest guest]

Very interesting report Jim. Thanks! Here are some

recent articles that discuss Lytico-Bodig (aka

Parkinson-dementia complex of Guam). Note the 3rd

report is about a strange cluster of PSP-like

disorders on the island of Guadeloupe.

Hugs,

Pam

----

Neurology 2002 Jan 8;58(1):90-7

Clinical features and changing patterns of

neurodegenerative disorders on Guam, 1997-2000.

Galasko D, Salmon DP, Craig UK, Thal LJ, Schellenberg

G, Wiederholt W.

Department of Neurosciences (Drs. Galasko, Salmon, and

Thal), University of California, San Diego.

BACKGROUND: In the 1950s, high-incidence ALS and

Parkinson-dementia complex (PDC) were identified among

Chamorros, the native inhabitants of Guam. Brains of

patients with these syndromes showed widespread

neurofibrillary tangles. Although ALS and PDC were

reported to have dramatically declined in the 1980s,

new cases are still encountered. Late-life dementia

has received little study among Chamorros. METHODS:

From 1997 to 2000, the authors evaluated newly

referred and previously identified patients. They

screened first-degree relatives of previous

registries, and subjects aged 60 or older. Subjects

who scored below a cognitive test cutoff or had

symptoms or signs consistent with parkinsonism or ALS

underwent psychometric testing, assessment by a

neurologist, and laboratory studies as appropriate.

Consensus diagnoses were made. RESULTS: The authors

identified 194 Chamorros with ALS (n = 10), PD (n =

11), PDC (n = 90), or late-life dementia (n = 83).

Mean ages at onset were 55 for ALS, 68 for PDC, 63 for

PD, and 74 for dementia. Late-life dementia was more

common in women, and met criteria for probable or

possible AD. The APOE-epsilon4 allele frequency was

uniformly low regardless of neurologic diagnosis.

CONCLUSIONS: The rapid decline of high-incidence ALS

on Guam over the past 40 years suggests the

contribution of a modifiable environmental factor. PDC

remains relatively common, with an unchanged clinical

picture apart from later age at onset. Dementia among

elderly Chamorros (termed " na dementia " )

resembles AD. Autopsy studies will clarify whether

this dementia is related to AD pathology or represents

a late-life neurofibrillary tangle syndrome more

closely allied to PDC.

PMID: 11781411 [PubMed - in process]

----

Neurology 2002 Mar 12;58(5):765-73

ALS and PDC of Guam: Forty-year follow-up.

Plato CC, Galasko D, Garruto RM, Plato M, Gamst A,

Craig UK, JM, Wiederholt W.

BACKGROUND: It was noticed in the mid-1950s that the

incidence of ALS and parkinsonism--dementia complex

(PDC) were much higher on Guam than anywhere else in

the world. In 1958, a registry of patients and

controls was established to ascertain the familial and

genetic aspects of these diseases. Patients and

individually matched controls and their relatives were

registered from 1958 to 1963. The registry was updated

and analyzed in 1998 through 1999. OBJECTIVE: To

ascertain whether first-degree relatives of patients

had a higher risk for developing ALS or PDC than

relatives of controls. Methods: During the period of

1958 to 1963, 126 new patients and 126 individually

matched controls and their respective first-degree

relatives and spouses were evaluated neurologically

and registered. Forty years later, the number of new

cases among the patient and control relatives were

compared to an expected number of new cases based on

the age- and sex-specific incidence of ALS and PDC in

the population at large. RESULTS: From 1958 to 1999,

there were 102 new ALS or PDC cases among relatives of

patients and 33 among relatives of controls. These

values were compared with the derived expected values.

There were more observed than expected new cases among

patients' relatives, and less observed cases than

expected among the controls' relatives. CONCLUSIONS:

Relatives of patients with ALS or PDC have

significantly higher risks for developing the disease

than the Guamanian population, whereas relatives of

controls have significantly lower risks.

PMID: 11889241 [PubMed - in process]

----

Brain 2002 Apr;125(Pt 4):801-811

Guadeloupean parkinsonism: a cluster of progressive

supranuclear palsy-like tauopathy.

Caparros-Lefebvre D, Sergeant N, Lees A, Camuzat A,

S, Lannuzel A, Brice A, Tolosa E, Delacourte A,

Duyckaerts C.

Neurologie, Centre Hospitalier Universitaire de la

Guadeloupe, Pointe a Pitre, French West Indies,

Laboratoire INSERM U 422, Lille, INSERM U 289 and.

Laboratoire de Neuropathologie, CHU Pitie-Salpetriere,

Paris, France, Hospital Clinici Provincial de

Barcelona, Spain and. Reta Lila Weston Institute of

Neurological Studies, Royal Free and University

College London Medical School, London, UK.

An unusually high frequency of atypical Parkinson

syndrome has been delineated over the last 5 years in

the French West Indies. Postural instability with

early falls, prominent frontal lobe dysfunction and

pseudo-bulbar palsy were common and three-quarters of

the patients were L-dopa unresponsive. One-third of

all patients seen had probable progressive

supranuclear palsy (PSP). This new focus of atypical

parkinsonism is reminiscent of the one described in

Guam and may be linked to exposure to tropical plants

containing mitochondrial complex I inhibitors

(quinolines, acetogenins, rotenoids). Two hundred and

twenty consecutive patients with Parkinson's syndrome

seen by the neurology service at Pointe a Pitre,

Guadeloupe University Hospital were studied. Currently

accepted operational clinical criteria for Parkinson's

syndromes were applied. The pathological findings of

three patients who came to autopsy are reported.

Fifty-eight patients had probable PSP, 96 had

undetermined parkinsonism and 50 had Parkinson's

disease, 15 had amyotrophic lateral sclerosis with

parkinsonism and one had probable multiple system

atrophy. All three PSP patients in whom post-mortem

study was performed had early postural instability,

gaze palsy and parkinsonian symptoms, followed by a

frontolimbic dementia and corticobulbar signs.

Neuropathological examination showed an accumulation

of tau proteins, predominating in the midbrain. There

was an exceptionally large accumulation of neuropil

threads in Case 1. Biochemical studies detected a

major doublet of pathological tau at 64 and 69 kDa in

brain tissue homogenates. All cases were homozygous

for the H1 tau haplotype, but no mutation of the tau

gene was observed. Clinical, neuropathological and

biochemical features were compatible with the

diagnosis of PSP, although some unusual pathological

features were noted in Case 1. A cluster of cases

presenting with atypical parkinsonism is reported.

Guadeloupean parkinsonism may prove to be a tauopathy

identical or closely related to PSP.

PMID: 11912113 [PubMed - as supplied by publisher]

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