Localization of disinhibition-dementia-parkinsonism-amyotrophy complex to 17q21-22

Am J Hum Genet. 1994 Dec;55(6):1159-65.


Disinhibition-dementia-parkinsonism-amyotrophy complex (DDPAC) is defined by familial adult-onset behavioral disturbance, followed by frontal lobe dementia, parkinsonism, and amyotrophy in variable proportions. A genetic etiology of DDPAC was suspected because of the familial clustering in family Mo, despite their wide geographic distribution. We have mapped the DDPAC locus to a 12-cM (sex averaged) region between D17S800 and D17S787 on chromosome 17q21-22. The basis for the variability of the clinical findings and pathology in DDPAC is unknown but suggests that the DDPAC locus should be screened as a candidate locus in family studies of conditions with behavioral abnormalities and neurological degeneration.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Chromosome Mapping
  • Chromosomes, Human, Pair 17 / genetics*
  • Dementia / etiology
  • Dementia / genetics*
  • Female
  • Genetic Markers
  • Genotype
  • Humans
  • Lod Score
  • Male
  • Muscular Diseases / etiology
  • Muscular Diseases / genetics*
  • Neuromuscular Diseases / genetics
  • Parkinson Disease / etiology
  • Parkinson Disease / genetics*
  • Pedigree


  • Genetic Markers