Lipomembranous polycystic osteodysplasia (brain, bone, and fat disease): a genetic cause of presenile dementia.

@article{Bird1983LipomembranousPO,
  title={Lipomembranous polycystic osteodysplasia (brain, bone, and fat disease): a genetic cause of presenile dementia.},
  author={Thomas D. Bird and R M Koerker and B J Leaird and B W Vlcek and David R Thorning},
  journal={Neurology},
  year={1983},
  volume={33 1},
  pages={81-6}
}
Progressive presenile dementia with lipomembranous polycystic osteodysplasia was first described by Jarvi and Hakola in an isolated region of Finland. We report the occurrence of this disorder in 4 of 10 siblings in an American family of Czechoslovakian ancestry. Characteristics of the disease include multiple bone cysts with pathologic fractures, progressive dementia with seizures and abnormal EEG, calcification of basal ganglia, and death in the fourth to six decades. Autosomal-recessive… CONTINUE READING
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