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P2‐182: A Variant of Frontotemporal Lobar Degeneration with Expanded Trinucleotide CAG Repeats in the Huntingtin Gene
Author(s) -
Sutovsky Stanislav,
Turcani Peter,
Novak Michal,
Alafuzoff Irina,
Zilka Norbert
Publication year - 2016
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.2016.06.1349
Subject(s) - frontotemporal lobar degeneration , huntingtin , trinucleotide repeat expansion , frontotemporal dementia , biology , pathology , c9orf72 , polyglutamine tract , huntingtin protein , sequestosome 1 , genetics , huntington's disease , dementia , gene , medicine , disease , allele , apoptosis , autophagy
started on PD between July 2011 and July 2015 at Queen Mary Hospital, Hong Kong, and repeated one year later. Demographics and clinical data were collected. CI was determined by locally defined MOCA cut-off. Risk factors for CI were analyzed using univariate and multivariate logistic regression. Results:137 patients were included, and 94 patients (69%) underwent the secondMOCA assessment. The prevalence of CI at baseline and 1-year was 34% and 32% respectively. MOCA score was 2365.0 at baseline and 2366.0 after 1 year (p1⁄40.29). Forty-seven percent of patients showed deterioration in MOCA score. Diabetes mellitus, education level of primary school or below, and age greater than 65 years were associated with CI at 1-year after starting PD, but only the later remained a significant risk factor for CI in the multivariate model (OR 2.72, 95%CI 1.04-7.09, p1⁄40.04). Patients with CI at 1-year after starting PD had more frequent PD peritonitis (1 episode in 22 patient months, vs. 1 in 48 patient months in those without CI, p1⁄40.01). Also, their median length-of-stay for emergency hospitalizations was longer (4 days, IQR 0-10, vs. 0 day, IQR 0-4, p1⁄40.01). After excluding patients on helper-assisted PD, the rates of peritonitis and exit-site infection still appeared higher in those with CI although the difference did not reach statistical significance (1 episode in 27 and 18 patient months respectively, vs. 1 in 43 and 32 patient months in those without CI, p1⁄40.07 and 0.06 respectively). Conclusions: Age above 65 is an independent risk factor for CI at one year after commencement of PD, and the latter is associated with adverse clinical outcomes including more hospitalization, peritonitis and exit-site infection.