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. 1994 Dec;55(6):1159–1165.

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

K C Wilhelmsen 1, T Lynch 1, E Pavlou 1, M Higgins 1, T G Nygaard 1
PMCID: PMC1918447  PMID: 7977375

Abstract

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.

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Selected References

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