Clinical Staging of Amyotrophic Lateral Sclerosis in Chinese Patients

Abstract

Objective: It is important to explore the utility of clinical staging systems in the management of amyotrophic lateral sclerosis (ALS). Our aim was to assess the validity of King's College in a Chinese ALS cohort, by evaluating the duration and informativeness of each stage and examining the association between stage and prognosis.Methods: From May 2008 to December 2016, patients with a likely diagnosis of ALS were registered. We prospectively assessed the progression of the patients through the stages and calculated the duration of each stage.Results: The median duration in Stage 1 was 12.00 months, Stage 2 7.50 months, Stage 3 6.50 months, and Stage 4 4.10 months. Subset analysis revealed that the spinal-onset and early-onset patients had a longer median time in Stage 1 compared to bulbar-onset and late-onset patients, respectively. Riluzole treatment extended the durations of Stages 1 and 2, and the effect was maintained in patients with long-term use of riluzole (>6 months). Patients who initiated long-term riluzole therapy early, in Stage 1 or 2, had a longer Stage 2. Patients who received percutaneous gastrostomy endoscopy (PEG) or non-invasive positive-pressure ventilation (NIPPV) showed longer durations of Stage 4. The differences in survival time measured from each stage to death or censor date were significant.Conclusions: We validated the King's College staging system in a Chinese population, and showed this system to be useful in clinical practice. Patients with bulbar-onset or an age of onset>45 years tended to have rapidly progressing ALS. Riluzole may be more effective when initiated in an early disease stage and continued long-term. PEG and NIPPV treatments can extend disease duration of Stage 4.

Predictors of survival in patients with amyotrophic lateral sclerosis: A large meta-analysis

“…The longer pLMN and pUMN are confined to the lower or upper motor neuron symptom, the longer is survival. Our previous study indicated a longer duration from an initial region to the involvement of a second region was associated with better prognosis [62] . This might also contribute to explain why the disease duration was a good prognostic factor in this study.…”

Health-related quality of life in amyotrophic lateral sclerosis using EQ-5D-5L

“…Stage 1 referred to symptom onset or the involvement of the first region. Stage 2A referred to an ALS diagnosis and Stage 2B was defined as functional involvement of a second region [ 19 ]. In addition, Stages 4A and 4B were combined and labeled as Stage 4 for analysis.…”

Canadian best practice recommendations for the management of amyotrophic lateral sclerosis

“…No controlled trials have examined whether riluzole extends life at a specific stage or all stages of ALS. A post-hoc analysis of the original dose-ranging study suggests that riluzole may be effective at prolonging survival only at later disease stages (defined by nutritional or respiratory failure sufficient to require intervention), 26 but results from other cohort studies differ, showing that it may be effective only at earlier stages, 27 or that its effect on survival is short lived. 28 Nevertheless, decades of experience worldwide have shown riluzole to be generally well tolerated with prolonged use and with regular monitoring of liver enzymes and blood counts, as well as screening for nausea and fatigue (class I).…”