Departments of Neurology, Pathology & Cell Biology, and Neuroscience, College of Physicians and Surgeons, Columbia University, New York, NY, USA.
Adv Neurobiol. 2022;28:323-352. doi: 10.1007/978-3-031-07167-6_13.
Motoneuron diseases (MNDs) represent a heterogeneous group of progressive paralytic disorders, mainly characterized by the loss of upper (corticospinal) motoneurons, lower (spinal) motoneurons or, often both. MNDs can occur from birth to adulthood and have a highly variable clinical presentation, even within gene-positive forms, suggesting the existence of environmental and genetic modifiers. A combination of cell autonomous and non-cell autonomous mechanisms contributes to motoneuron degeneration in MNDs, suggesting multifactorial pathogenic processes.
运动神经元病（MNDs）是一组异质性的进行性瘫痪疾病，主要特征是上运动神经元（皮质脊髓）、下运动神经元（脊髓）或两者同时丧失。MNDs 可发生于从出生到成年的任何时期，临床表现高度可变，即使在基因阳性形式中也是如此，这表明存在环境和遗传修饰因子。细胞自主和非细胞自主机制的结合导致 MND 中的运动神经元变性，提示存在多因素的致病过程。
