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[P2–408]: STRIATUM DOPAMINE TRANSPORTER DISTRIBUTION IN CHINESE FAMILIAL FTDP‐17 PEDIGREE WITH MAPT N279K MUTATION
Author(s) -
Wu Liyong,
Liu Jia,
Jia Jianping
Publication year - 2017
Publication title -
alzheimer's and dementia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 6.713
H-Index - 118
eISSN - 1552-5279
pISSN - 1552-5260
DOI - 10.1016/j.jalz.2017.06.1064
Subject(s) - putamen , parkinsonism , dopamine transporter , striatum , psychology , caudate nucleus , frontotemporal dementia , dementia , medicine , neuroscience , pathology , dopaminergic , disease , dopamine
Background: Frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17) is a rare inherited neurodegenerative disease, and most patients with FTDP-17 are caused by mutation of microtubule-associated protein tau (MAPT) gene. Hypometabolism in temporal lobe has been estimated to appear prior to clinical symptoms. But it remains unclear whether striatum dopamine transporter (DAT) distribution, which is potentially related to the parkinsonism in FTDP-17, could predict the onset of disease. Methods: In order to examine striatum DAT distribution in asymptomatic and symptomatic FTDP-17 mutation carriers. A Chinese familial FTDP-17 pedigree with MAPT N279K mutation was investigated. In total, 2 asymptomatic and 2 symptomatic mutation carriers were enrolled with 6 age-matched normal controls. Standardized clinical evaluation, neuropsychology assessment and (11)C-CFT-PET for striatum DAT distribution were assessed. The striatum was segmented as caudate head, body, tail and putamen. Results:Parkinsonism was the first symptom for 2 symptomatic mutation carriers. Personality, speech changes and dementia accompanied with brain atrophy were developed at the later stage in one of them. 2 asymptomatic mutation carriers showed no clinical, neuropsychology or structural imaging abnormality. Regarding to the [C]CFT PET for striatum DAT distribution, all of them showed asymmetric decreased striatum DAT distribution. In comparison with normal controls, bilateral putamen was the more sensitive DAT decreased region than caudate head, body or tail for FTDP17 mutation carriers. Conclusions: In summary, decreased DAT can predict the disease at least 15 years before the onset of the elder generation of symptomatic mutation carriers, and 7 years before the onset of peer symptomatic mutation carriers. Therefore, striatum DAT distribution could be a potential biomarker in early diagnosis of FTDP-17, even at the asymptomatic stage.