Kosseifi Semaan G, Abdel Nour Souheil, Roy Thomas M, Byrd Ryland P, Alwani Anita
Veterans Affairs Medical Center, Mountain Home, TN, USA.
South Med J. 2010 Apr;103(4):378-81. doi: 10.1097/SMJ.0b013e3181d3ceaa.
In Western countries the incidence of amyotrophic lateral sclerosis (ALS) is 1.89 per 100,000 per year and the prevalence is 5.2 per 100,000. The incidence of ALS is lower among African, Asian, and Hispanic ethnicities when compared to Caucasians. The mean age of onset for sporadic ALS is about 60 years and there is a slight male predominance (male to female ratio of 1.5 to 1). Approximately two thirds of patients with ALS have the spinal form of the disease with symptoms presenting in the extremities. Patients typically have evidence of both lower motor neuron degeneration (atrophy, weakness, and fasciculations) and upper motor neuron degeneration (spasticity, weakness, and hyperreflexia). Patients with limb onset ALS typically complain of focal muscle weakness and wasting. The symptoms may start either distally or proximally in the upper and/or lower limbs. Gradually spasticity develops in the weakened atrophic limbs, affecting manual dexterity and gait. Patients with bulbar onset ALS typically present with dysarthria and dysphagia for solid or liquids. Limb symptoms can develop simultaneously with bulbar onset. In the vast majority of patients, limb weakness will occur within 1-2 years of bulbar onset ALS symptoms. A case of bulbar and sporadic limb ALS in a 70-year-old veteran, presenting with right diaphragmatic paralysis and respiratory failure, is presented.
在西方国家，肌萎缩侧索硬化症（ALS）的发病率为每年每10万人中有1.89例，患病率为每10万人中有5.2例。与白种人相比，非洲、亚洲和西班牙裔种族中ALS的发病率较低。散发性ALS的平均发病年龄约为60岁，男性略占优势（男女比例为1.5比1）。大约三分之二的ALS患者患有脊髓型疾病，症状出现在四肢。患者通常同时有下运动神经元变性（萎缩、无力和肌束震颤）和上运动神经元变性（痉挛、无力和反射亢进）的证据。肢体起病的ALS患者通常主诉局部肌肉无力和萎缩。症状可能始于上肢和/或下肢的远端或近端。逐渐地，痉挛在萎缩无力的肢体中出现，影响手部灵活性和步态。延髓起病的ALS患者通常表现为构音障碍和吞咽固体或液体困难。肢体症状可与延髓起病同时出现。在绝大多数患者中肢体无力将在延髓起病的ALS症状出现后的1 - 2年内发生。本文介绍了一名70岁退伍军人患延髓和散发性肢体ALS并伴有右膈神经麻痹和呼吸衰竭的病例。
