Hu William T, Seelaar Harro, Josephs Keith A, Knopman David S, Boeve Bradley F, Sorenson Eric J, McCluskey Leo, Elman Lauren, Schelhaas Helenius J, Parisi Joseph E, Kuesters Benno, Lee Virginia M-Y, Trojanowski John Q, Petersen Ronald C, van Swieten John C, Grossman Murray
Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Arch Neurol. 2009 Nov;66(11):1359-64. doi: 10.1001/archneurol.2009.253.
Frontotemporal dementia and amyotrophic lateral sclerosis are neurodegenerative diseases associated with TAR DNA-binding protein 43- and ubiquitin-immunoreactive pathologic lesions.
To determine whether survival is influenced by symptom of onset in patients with frontotemporal dementia and amyotrophic lateral sclerosis.
DESIGN, SETTING, AND PATIENTS: Retrospective review of patients with both cognitive impairment and motor neuron disease consecutively evaluated at 4 academic medical centers in 2 countries.
Clinical phenotypes and survival patterns of patients.
A total of 87 patients were identified, including 60 who developed cognitive symptoms first, 19 who developed motor symptoms first, and 8 who had simultaneous onset of cognitive and motor symptoms. Among the 59 deceased patients, we identified 2 distinct subgroups of patients according to survival. Long-term survivors had cognitive onset and delayed emergence of motor symptoms after a long monosymptomatic phase and had significantly longer survival than the typical survivors (mean, 67.5 months vs 28.2 months, respectively; P < .001). Typical survivors can have simultaneous or discrete onset of cognitive and motor symptoms, and the simultaneous-onset patients had shorter survival (mean, 19.2 months) than those with distinct cognitive or motor onset (mean, 28.6 months) (P = .005).
Distinct patterns of survival profiles exist in patients with frontotemporal dementia and motor neuron disease, and overall survival may depend on the relative timing of the emergence of secondary symptoms.
额颞叶痴呆和肌萎缩侧索硬化是与TAR DNA结合蛋白43和泛素免疫反应性病理病变相关的神经退行性疾病。
确定额颞叶痴呆和肌萎缩侧索硬化患者的生存是否受发病症状的影响。
设计、地点和患者：对2个国家4个学术医学中心连续评估的认知障碍和运动神经元疾病患者进行回顾性研究。
患者的临床表型和生存模式。
共确定87例患者，其中60例首先出现认知症状，19例首先出现运动症状，8例认知和运动症状同时出现。在59例死亡患者中，根据生存情况我们确定了2个不同的亚组。长期存活者以认知起病，在长时间的单症状期后运动症状延迟出现，其生存期明显长于典型存活者（分别为平均67.5个月和28.2个月；P <.001）。典型存活者认知和运动症状可同时或分别出现，同时起病的患者生存期短于认知或运动症状分别起病的患者（分别为平均19.2个月和28.6个月）（P = .005）。
额颞叶痴呆和运动神经元疾病患者存在不同的生存模式，总体生存可能取决于继发症状出现的相对时间。
