Disinhibition-Dementia-Parkinsonism-Amyotrophy Complex (DDPAC) is a non-Alzheimer’s frontotemporal dementia

  • K. C. Wilhelmsen
Conference paper


DDPAC was defined based on the cardinal symptoms of the syndrome found in family Mo. Investigation of DDPAC cases in family Mo shows non-specific pathological changes in a distribution that is consistent with the cardinal features of the disease. Genetic analysis identified a locus on chromosome 17q21–22 that produces this syndrome which is inherited as an autosomal dominant trait. DDPAC overlaps clinically and pathologically with a variety of named neurodegenerative syndromes and falls within the spectrum called frontotemporal dementia. The localization of the disease locus for DDPAC allows the testing of whether other familial neurodegenerative conditions also map to the same chromosomal regions. It seems possible that ultimately these conditions that have thus far been difficult to categorize will be subject to a nosolgy based on genetic etiology.


Motor Neuron Disease Frontotemporal Dementia Autosomal Dominant Trait Akinetic Mutism Mental Depression 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer-Verlag/Wien 1997

Authors and Affiliations

  • K. C. Wilhelmsen
    • 1
    • 2
  1. 1.Department of NeurologyUniversity of CaliforniaSan FranciscoUSA
  2. 2.The Gallo Clinic and Research CenterSan Francisco General HospitalSan FranciscoUSA

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