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

• amyotrophic lateral sclerosis lower motor neuron manifestations
• parkinsonism
• dementia
• progressive motor function loss
• bulbar paralysis
• muscle weakness
• muscle cramps

More general terms

• parkinson plus syndrome
• neurodegenerative disease

Database

• OMIM: https://mirror.omim.org/entry/105500