Blasco Helene, Guennoc Anne-Marie, Veyrat-Durebex Charlotte, Gordon Paul H, Andres Christian R, Camu William, Corcia Philippe
UMR INSERM U930, Université François Rabelais de Tours, Tours, France.
Amyotroph Lateral Scler. 2012 Oct;13(6):585-8. doi: 10.3109/17482968.2012.706303. Epub 2012 Aug 8.
Amyotrophic lateral sclerosis (ALS) is the most frequent motor neuron disorder in adults. This fatal condition, due to degeneration of upper and lower motor neurons in spinal and bulbar myotomes, leads to death from respiratory failure after median disease duration of 36 months. ALS is sporadic in more than 90% of cases and familial in the remaining cases. Most studies show male predominance with a gender ratio of 3:2, but gender differences are age related. The phenotype of ALS is also different in males and females with a predominance of limb onset in males and bulbar onset in females. While age and site of onset impact survival rate, and are both related to gender, gender by itself has not clearly been shown to have an effect on survival. Given this complex relationship between gender and ALS, we developed a hypothesis about hormone involvement in ALS aetiology by suggesting protective effect of oestrogens and adverse effect of androgens.
肌萎缩侧索硬化症（ALS）是成人中最常见的运动神经元疾病。这种致命疾病是由于脊髓和延髓肌节中的上、下运动神经元变性所致，在疾病中位持续时间36个月后，会因呼吸衰竭而死亡。超过90%的ALS病例为散发性，其余为家族性。大多数研究表明男性占主导，性别比为3:2，但性别差异与年龄有关。ALS的表型在男性和女性中也有所不同，男性以肢体起病为主，女性以延髓起病为主。虽然年龄和起病部位会影响生存率，且都与性别有关，但尚未明确表明性别本身对生存率有影响。鉴于性别与ALS之间的这种复杂关系，我们提出了一个关于激素参与ALS病因的假说，认为雌激素具有保护作用，而雄激素具有不良作用。
