Department of Neurology, University of Kansas Medical Center, Kansas City, KS, USA.
Department of Neurology, Ann Arbor Veterans Affairs Medical Center, University of Michigan, Ann Arbor, MI, USA.
Amyotroph Lateral Scler Frontotemporal Degener. 2020 Nov;21(sup1):3-10. doi: 10.1080/21678421.2020.1837178.
Primary lateral sclerosis is a distinct entity that has recently been classified as a "restricted phenotype" of ALS. It is characterized by a pattern of isolated upper motor neuron involvement that often begins in the legs and spreads diffusely. Distinction from other conditions requires careful consideration of clinical presentation and time course of disease. Mills' Syndrome is a rare unilateral variant of primary lateral sclerosis. Cognitive and behavioral involvement may occur.
原发性侧索硬化症是一种明确的疾病实体,最近被归类为 ALS 的“受限表型”。它的特征是孤立的上运动神经元受累模式,通常始于腿部并广泛扩散。与其他疾病的鉴别需要仔细考虑临床表现和疾病的病程。Mills 综合征是原发性侧索硬化症的罕见单侧变异型。可能会出现认知和行为方面的问题。