Bob ,
I found this and thought it very interesting . Apparently it can affect a
non-Chammoro.
I found another article elsewhere stating that this type of Parkinsons
has also been
found in an area of Japan.
Carol Mcleod
[log in to unmask]
PARKINSONISM-DEMENTIA COMPLEX OF GUAM (PDCG)
Clinical Aspects
The combination of parkinsonisn, dementia, and motor neuron disease
was first noted in a population of Guam.{44} In a review of 363 Chammoro
and
three Filipino immigrants with this disease, men were affected twice as
frequently as women, but no differences in age of onset (57 years) or
death
(62 years) were seen between the genders.{115} Besides parkinsonism.
supranuclear ocular motility disorder was reported in all 37 patients in
one series.{67} Patients with parkinsonism usually present later than
those
with amytrophic lateral sclerosis, possibly because patients presenting
with
motor neuron disease do not survive long enough to develop extrapyramidal
symptoms.{114} Furthermore basal ganglia signs may be masked by the motor
neuron disease.
Neurodiagnostic Studies
PET scanning in patients with the parkinsonian form of this disease
reveals
decreased presynaptic l8F-6-fluorodopa uptake similar to patients with
Parkinson's disease, whereas those with amyotrophic lateral sclerosis
have
an intermediate picture between Parkinson's disease and control
populations, suggesting a preclinical lesion.{105}
Neuropathology and Neurochemistry
The finding of increased frequencies of this neurodegenerative disorder
on
the island of Guam suggest a possible environmental etiology.{44} The
neurotoxin beta-N-methylamino-L-alanine (BMAA), a compound found in the
cycad plant and believed to be in high concentrations in flour made from
this plant, produces a similar spectrum of neurologic decline in
monkeys.{106} An analysis of the BMAA content of cycad flour, however,
suggests that the quantities of BMAA normally consumed by the inhabitants
of the endemic areas were not sufficient to produce neurologic
toxicity.{20}
Other hypotheses concerning abnormalities in mineral metabolism and
hypomagnesemia or hypocalcemia also have been suggested, but supporting
evidence is lacking.{27}
Pathologically this condition resembles Alzheimer's disease more than
Parkinson's disease; tau-containing NFTs are present particularly in the
hippocampus{35,101} and other brain and spinal cord areas.{75}
Immunohistochemical studies of autopsied brains of patients with PDCG
showed marked reduction in the number of dopaminergic neurons in both the
lateral and the medial substantia nigra.{39} Despite marked reduction of
nigrostriatal dopamine concentration, the striatal output system was well
preserved and glutamate, gamma-aminobutyric acid (GABA), choline
acetyltransferase, and serotonin were spared.
John Cottingham [log in to unmask]
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