Disinhibition-Dementia-Parkinsonism-Amyotrophy Complex (DDPAC) is a non-Alzheimer’s frontotemporal dementia
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.
KeywordsMotor Neuron Disease Frontotemporal Dementia Autosomal Dominant Trait Akinetic Mutism Mental Depression
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- Brun A (ed) (1987) Frontal lobe degeneration of non-Alzheimer type. Arch Gerontol Geriatr 6: 193–208Google Scholar
- Brun A (ed) (1993) The 2nd international conference on frontal lobe degeneration of non-alzheimer type. Dementia 4: 123–236Google Scholar
- Fahn S, Mayeux R, Rowland LP (1995) A new eponym: Wilhelmsen-Lynch disease. Neurology 44: 1980Google Scholar
- Mitsuyama Y, Kogoh H, Ata K (1985) Progressive dementia with motor neuron disease. An additional case report and neuropathological review of 20 cases in Japan. Eur Arch Psychiatry Clin Neurosci 235: 1–8Google Scholar
- Perry, TL (1976) Hereditary mental depression with taurine deficiency: further studies, including a therapeutic trial of taurine administration In: Huxtable R, Barbeau A (eds) Taurine. Raven Press, New York, pp 363–374Google Scholar
- Simma AAF, Defendini RF, Keohane C, et al (1995) The chromosome 17-linked disinhibition-dementia-parkinsonism-amyotrophy complex is characterized pathologically by progressive limbic lobe sclerosis (unpublished)Google Scholar
- Wszolek ZK, Lynch T, Wilhelmsen KC (1995) Rapidly progressive autosomal dominant parkinsonism and dementia with pallido-pontonigral (PPND) and disinhibition-dementia-parkinsonism-amyotrophy complex (DDPAC) are clinically distinct conditions that are both linked to 17q21–22 (unpublished)Google Scholar