Karam Chafic, Scelsa Stephen N, Macgowan Daniel J L
Neuromuscular Division and ALS Center, Beth Israel Medical Center, Albert Einstein College of Medicine, New York, New York, USA.
Amyotroph Lateral Scler. 2010 Aug;11(4):364-8. doi: 10.3109/17482960903513159.
Our objective was to study the clinical course of patients diagnosed with progressive bulbar palsy (PBP). We reviewed all 392 medical records of ALS patients seen between 1 January 2000 and 31 July 2007. Patients with isolated PBP at presentation were selected and classified into those with normal EMG of the limbs (PBP-N) and those with active denervation on EMG (PBP-A). We studied the time to progression of these patients to ALS. We compared patients with PBP-N to patients with PBP-A. Fifteen patients were diagnosed with PBP-N. The remaining 17 had PBP-A. Thirteen of the 15 patients with PBP-N (87%) progressed to definite ALS. The two patients who did not progress to ALS died at 22 and 60 months, respectively. The median survival time was 35 months for the PBP-N group and 40 months for the PBP-A group (p = 0.92). Except for the rate of depression, patients with PBP-N did not differ from patients with PBP-A in the basic demographics, time of presentation, clinical course, survival and treatment received. All patients with FTD died within 40 months of onset of symptoms. In conclusion, almost all PBP patients progress to ALS regardless of the presence of upper motor signs or generalized denervation on EMG of the limbs.
我们的目的是研究被诊断为进行性延髓麻痹（PBP）患者的临床病程。我们回顾了2000年1月1日至2007年7月31日期间诊治的所有392例肌萎缩侧索硬化症（ALS）患者的病历。选取初诊为孤立性PBP的患者，并将其分为肢体肌电图（EMG）正常的患者（PBP-N）和EMG有失神经活动的患者（PBP-A）。我们研究了这些患者进展为ALS的时间。我们将PBP-N患者与PBP-A患者进行比较。15例患者被诊断为PBP-N。其余17例为PBP-A。15例PBP-N患者中有13例（87%）进展为确诊的ALS。未进展为ALS的2例患者分别在22个月和60个月时死亡。PBP-N组的中位生存时间为35个月，PBP-A组为40个月（p = 0.92）。除抑郁发生率外，PBP-N患者与PBP-A患者在基本人口统计学特征、就诊时间、临床病程、生存及接受的治疗方面无差异。所有额颞叶痴呆（FTD）患者在症状出现后40个月内死亡。总之，几乎所有PBP患者都会进展为ALS，无论肢体EMG上是否存在上运动神经元体征或广泛性失神经。
