Mixed pathologies mimicking motor neuron disease: a case report and review of the literature.

Mixed pathologies mimicking motor neuron disease: a case report and review of the literature.

Amyotrophic lateral sclerosis (ALS) is classified as a motor neuron disease (MND) that can present with both upper and lower motor neuron signs. Concurrent ALS and frontotemporal dementia (FTD) is also a well-known phenomenon. Examples of other primary disorders mimicking ALS or ALS-FTD have been reported in the literature and recognition of these entities is important to ensure proper clinical management. We present here an unusual case of an 86-year-old male patient, clinically diagnosed with ALS and severe cognitive impairment thought to be due to FTD. Postmortem neuropathological examination of his brain and spinal cord did not reveal the typical findings of ALS or FTD. Rather, it revealed multiple non-ALS pathologies including argyrophilic grain disease (AGD), cerebrovascular disease, and Alzheimer's type pathology. This case raises the possibility that mixed pathologies could mimic motor neuron disease.

肌萎缩侧索硬化（ALS）被归类为一种运动神经元疾病（MND），可同时出现上运动神经元和下运动神经元体征。ALS并发额颞叶痴呆（FTD）也是一种众所周知的现象。文献中已有报道其他类似ALS或ALS - FTD的原发性疾病的病例，识别这些疾病实体对于确保恰当的临床治疗非常重要。我们在此介绍一个不寻常的病例，一名86岁男性患者，临床诊断为ALS以及被认为由FTD导致的严重认知障碍。对其大脑和脊髓进行的尸检神经病理学检查未发现ALS或FTD的典型表现。相反，检查发现了多种非ALS病理改变，包括嗜银颗粒病（AGD）、脑血管疾病以及阿尔茨海默病类型的病理改变。该病例提示混合病理改变可能会模拟运动神经元疾病。