Clinically undetected motor neuron disease in pathologically proven frontotemporal lobar degeneration with motor neuron disease
Clinically undetected motor neuron disease in pathologically proven frontotemporal lobar degeneration with motor neuron disease
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DOI：
10.1001/archneur.63.4.506
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发表时间：
2006-04-01
影响因子：
--
通讯作者：
Dickson, DW
中科院分区：
文献类型：
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作者：
Josephs, KA;Parisi, JE;Dickson, DW
背景资料：额颞叶变性伴运动神经元病（FTLD-MND）是以运动神经元变性和额颞叶变性为特征的一种疾病。在病理证实的FTLD-MND中检测痴呆和运动神经元疾病的临床体征的能力尚未评估。目的：确定是否所有病理证实的FTLD-MND病例都有额颞叶痴呆和运动神经元疾病的临床证据，并确定误诊的可能原因。回顾所有经病理证实的FTLD-MND病例的历史记录并对运动和运动外病理结果进行半定量分析。在总共17例病理证实的FTLD-MND中，所有病例都有额颞叶痴呆的临床证据，而只有10例（59%）有运动神经元疾病的临床证据。运动和运动外病理结果的半定量分析揭示了一个频谱的病理变化的基础FTLD-MND。海马硬化，主要是下托，在没有运动神经元疾病临床证据的病例中明显更常见（P <0.01）。此外，神经元丢失，胶质增生，皮质脊髓束变性不太严重，在其他3例没有运动神经元diseases.Conclusions的临床证据：FTLD-MND的元素的临床诊断敏感性是温和的，可能会受到影响的事实，FTLD-MND代表一个频谱的病理结果，而不是一个单一的同质实体。当运动神经元变性较轻和海马硬化患者中，临床运动神经元疾病的体征检测也很困难。
Background: Frontotemporal lobar degeneration with motor neuron disease (FTLD-MND) is a pathological entity characterized by motor neuron degeneration and frontotemporal lobar degeneration. The ability to detect the clinical signs of dementia and motor neuron disease in pathologically confirmed FTLD-MND has not been assessed.Objectives: To determine if all cases of pathologically confirmed FTLD-MND have clinical evidence of frontotemporal dementia and motor neuron disease, and to determine the possible reasons for misdiagnosis.Method: Review of historical records and semiquantitative analysis of the motor and extramotor pathological findings of all cases of pathologically confirmed FTLD-MND.Results: From a total of 17 cases of pathologically confirmed FTLD-MND, all had clinical evidence of frontotemporal dementia, while only 10 (59%) had clinical evidence of motor neuron disease. Semiquantitative analysis of motor and extramotor pathological findings revealed a spectrum of pathological changes underlying FTLD-MND. Hippocampal sclerosis, predominantly of the subiculum, was a significantly more frequent occurrence in the cases without clinical evidence of motor neuron disease (P < .01). In addition, neuronal loss, gliosis, and corticospinal tract degeneration were less severe in the other 3 cases without clinical evidence of motor neuron disease.Conclusions: Clinical diagnostic sensitivity for the elements of FTLD-MND is modest and may be affected by the fact that FTLD-MND represents a spectrum of pathological findings, rather than a single homogeneous entity. Detection of signs of clinical motor neuron disease is also difficult when motor neuron degeneration is mild and in patients with hippocampal sclerosis.