amyotrophic lateral sclerosis-parkinsonism-dementia complex of Guam

Etiology: unknown - possible genetic susceptibility. - dietary hypotheses - low calcium/magnesium intake in water & vegetables followed by: - secondary hyperparathyroidism - increased intestinal absorption of toxic metals - deposition of calcium and other metals in CNS - neurotoxic plant Cycas circinalis Epidemiology: - marked decline in incidence rates. Pathology: - neurofibrillary tangles in many areas Genetics: - type 1 associated with defects in TRPM7 Clinical manifestations: 1) amyotrophic lateral sclerosis lower motor neuron manifestations 2) parkinsonism 3) dementia 4) progressive motor function loss 5) bulbar paralysis 6) muscle weakness 7) muscle cramps

General

neurodegenerative disease parkinson plus syndrome

Properties

PATHOLOGY: neurofibrillary tangle

References

OMIM 105500