Hu William T, Parisi Joseph E, Knopman David S, Boeve Bradley F, Dickson Dennis W, Ahlskog J Eric, Petersen Ronald C, Josephs Keith A
Department of Neurology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA.
Alzheimer Dis Assoc Disord. 2007 Oct-Dec;21(4):S39-43. doi: 10.1097/WAD.0b013e31815bf5e5.
We compared the clinical characteristics of 3 repeat (3R) and 4 repeat (4R) tau-positive cases (tauopathies) presenting as behavior variant frontotemporal dementia (bv-FTD). We identified and retrospectively reviewed demographics and clinical features of patients with pathologically confirmed tau-positive frontotemporal lobar degeneration in a blinded fashion. Those presenting as bv-FTD were divided according to their tau isoform, 3R versus 4R, and compared with age-matched and sex-matched control patients with 4R tauopathies but presenting clinical syndromes other than bv-FTD. Twenty-four cases with tau-positive bv-FTD and 18 4R tau-positive controls were included in the study. Patients with 4R tauopathies had significantly shorter disease duration than patients with 3R tauopathy (median, 6.5 y vs. 9.5 y; P<0.05), despite similar age of disease onset and regardless of whether bv-FTD was the presenting clinical syndrome. Among bv-FTD cases, those with 4R tauopathies were more likely to display behavioral underactivity than those with 3R tauopathy (P=0.03), although 3R and 4R tauopathy patients shared many similar clinical features. In summary, survival in 4R tauopathies seemed independent of the presenting clinical phenotype, and there may be subtle clinical differences between bv-FTD patients with 3R and 4R tauopathies.
我们比较了表现为行为变异型额颞叶痴呆（bv-FTD）的3重复（3R）和4重复（4R）tau阳性病例（tau蛋白病）的临床特征。我们以盲法识别并回顾了经病理证实的tau阳性额颞叶变性患者的人口统计学和临床特征。那些表现为bv-FTD的患者根据其tau异构体（3R与4R）进行分组，并与年龄和性别匹配的4R tau蛋白病对照患者进行比较，这些对照患者表现出bv-FTD以外的临床综合征。本研究纳入了24例tau阳性bv-FTD病例和18例4R tau阳性对照。4R tau蛋白病患者的病程明显短于3R tau蛋白病患者（中位数分别为6.5年和9.5年；P<0.05），尽管发病年龄相似，且无论bv-FTD是否为首发临床综合征。在bv-FTD病例中，4R tau蛋白病患者比3R tau蛋白病患者更有可能表现出行为活动减少（P=0.03），尽管3R和4R tau蛋白病患者有许多相似的临床特征。总之，4R tau蛋白病的生存期似乎与首发临床表型无关，3R和4R tau蛋白病的bv-FTD患者之间可能存在细微的临床差异。
