Bradley W G, Anderson F, Gowda N, Miller R G
University of Miami School of Medicine, Department of Neurology, Miami, FL 33101, USA.
Amyotroph Lateral Scler Other Motor Neuron Disord. 2004 Dec;5(4):240-4.
To determine if the publication in 1999 of the AAN Practice Parameter on ALS was associated with an improvement in the standard of management of the disease.
Data on 646 patients enrolled in the ALS CARE database and on 465 patients who died in the period May 2001 to November 2002 were compared with similar data obtained from the database from 1996 to May 1999.
The specialty ALS clinics were the most important source of information about ALS. The internet was a source for 39%. The treatment of sialorrhea, pseudobulbar emotional lability, and failure of swallowing and breathing had all improved significantly in the period after the publication of the Practice Parameter. However many patients still did not receive a gastrostomy tube or non-invasive positive pressure ventilation when indicated by the Practice Parameter, mainly because of lack of patient compliance. Cost was the main reason why 41% of patients did not receive riluzole, though they spent a third of the cost of this medication on alternative medicines.
The publication of the AAN Practice Parameter was associated with improvement in the standard of care. Most cases in the database come from specialized ALS centers, and further information on the community care of ALS patients is needed.
确定美国神经病学学会（AAN）1999年发布的肌萎缩侧索硬化症（ALS）实践参数是否与该疾病管理标准的改善相关。
将ALS护理数据库中登记的646例患者以及2001年5月至2002年11月期间死亡的465例患者的数据，与1996年至1999年5月从该数据库获得的类似数据进行比较。
ALS专科诊所是关于ALS最重要的信息来源。互联网是39%的信息来源。在实践参数发布后的这段时间里，流涎、假性延髓情绪不稳定以及吞咽和呼吸功能衰竭的治疗均有显著改善。然而，许多患者在实践参数表明需要时仍未接受胃造瘘管或无创正压通气，主要原因是患者依从性差。费用是41%的患者未接受利鲁唑治疗的主要原因，尽管他们在替代药物上花费了这种药物三分之一的费用。
AAN实践参数的发布与护理标准的改善相关。数据库中的大多数病例来自ALS专科中心，需要进一步了解ALS患者的社区护理情况。
