Rapidly Progressive Neurodegenerative Dementias
Rapidly Progressive Neurodegenerative Dementias
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DOI：
10.1001/archneurol.2008.534
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发表时间：
2009-02-01
影响因子：
--
通讯作者：
Petersen, Ronald C.
中科院分区：
文献类型：
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作者：
Josephs, Keith A.;Ahlskog, J. Eric;Petersen, Ronald C.
背景：神经退行性痴呆的典型特点是发病隐匿，病程进展相对缓慢。快速进展至死亡的患者较少见。目的：探讨神经退行性疾病快速进展至死亡的患者特征。设计、设置和患者：在同一个句子中使用“快速”和“痴呆”的文本词搜索，梅奥诊所医疗记录链接系统用于识别2000年1月1日至2007年9月30日期间评估的所有患者，并在三级保健医疗中心进行脑尸检（N=96）。在这96例患者中，我们只纳入了那些疾病持续时间少于4年至死亡且组织学诊断为神经退行性疾病的患者。主要结局指标：在本院的神经退行性疾病发病和病理诊断后4年内死亡的快速进展性痴呆。结果：纳入22例患者（男性10例）。虽然有克雅氏病（CJD） 8例（36%），其余有额颞叶变性伴运动神经元变性（5例[23%]）、牛头病（进行性核上性麻痹或皮质基底变性）（4例[18%]）、弥漫性路易体病（3例[14%]）或阿尔茨海默病（2例[9%]）。所有CJD患者在发病后12个月或更早死亡，而其他患者的病程超过12个月。值得注意的是，所有3例弥漫性路易体病患者最初均经历了短暂的术后或疾病相关脑病，然后相对正常2年，最后迅速进展为痴呆，并在4至12个月内下降至死亡。结论：基于这一队列，虽然CJD是快速进行性神经退行性痴呆的最可能原因，但额颞叶变性伴运动神经元变性、弥漫性路易体病、牛头病和阿尔茨海默病也可导致快速进行性痴呆。如果病程超过12个月，非CJD神经退行性疾病可能比CJD更容易被诊断。
Background: Neurodegenerative dementias are typically characterized by an insidious onset and a relatively slowly progressive course. Less common are patients with a rapidly progressive course to death.Objective: To characterize patients with a neurodegenerative disease and a rapidly progressive course to death.Design, Setting, and Patients: Using a text word search for "rapid" and "dementia" in the same sentence, the Mayo Clinic Medical Records Linkage system was used to identify all patients evaluated between January 1, 2000, and September 30, 2007, with brain autopsy (N=96) at a tertiary care medical center. Of these 96 patients, we included only those with disease duration of less than 4 years to death and with histological diagnosis of a neurodegenerative disease.Main Outcome Measures: Rapidly progressive dementia with death sooner than 4 years after onset and pathological diagnosis at our institution of a neurodegenerative disease.Results: We included 22 patients (10 men). Although 8 cases (36%) had Creutzfeldt-Jakob disease (CJD), the rest had frontotemporal lobar degeneration with motor neuron degeneration (5 cases [23%]), a tauopathy (progressive supranuclear palsy or corticobasal degeneration) (4 cases [18%]), diffuse Lewy body disease (3 cases [14%]), or Alzheimer disease (2 cases [9%]). All of the patients with CJD died 12 months or sooner after onset, whereas the others had an illness duration longer than 12 months. Notably, all of the 3 patients with diffuse Lewy body disease but no others initially experienced a transient postoperative or illness-associated encephalopathy, then relative normality for 2 years, and finally a rapidly progressive dementia and decline to death in 4 to 12 months.Conclusions: Based on this cohort, although CJD is the most likely cause of a rapidly progressive neurodegenerative dementia, frontotemporal lobar degeneration with motor neuron degeneration, diffuse Lewy body disease, tauopathies, and Alzheimer disease can also cause a rapidly progressive dementia. If illness duration is beyond 12 months, a non-CJD neurodegenerative disease may be more likely than CJD to be the diagnosis.