Créditos de imagem: Greg Dunn.

In this paper I will be refering in part to my experience of almost 40 years working with patients with amyotrophic lateral sclerosis, the last 12 with the ALS Functional Rating Scale, which I introduced in Brasil with Dr Jarise Gouvea in 2005. And the text will be based also on the paper “Is ALS Ready for a Staging Model?, by Fallik, Dawn, published in Neurology Today on 01 March 2012 – Volume 12 – Issue 5 – pp 1,18-19, doi: 10.1097/01.NT.0000413078.55562.25.

The reason we looked for the functional scale is we were introducing a treatment at the time, a high dose cyclophosphamide/ autologous stem cell 15-day course outpatient therapy, and we needed some form of measurement to see whether the treatment worked.  In the paper by Ms Fallik, she says that “patients want to know where they are in the progression of the marathon, what can they expect to find ahead in the course of the disease?”

She refers to a paper published on Jan. 23 online in Brain, in which researchers developed a staging model, in a disease which has been difficult becasue frequently there is overlap of systems involved.

Perhaps the greater difficulty has been the clinical tradition of neurologists, a brand of people adverse to quantification and unification, more apt to thinking of each patient as a separate and unique entity, more so in the hippocratic ethical nature of Medicine.  But these authors made a strong attempt to identify milestones  and trajectories along the ALS disease pathway. The stages are quite variable statistically, which is reflected in the wide confidence limits for each stage presented in the Brain paper, which is published by the King’s College,  London, researchers, who followed 1,471 patients with ALS between 1993 and 2007.

Patients had limb, bulbar, or diaphragmatic onset ALS. The researchers defined symptom onset (stage 1) as functional involvement by weakness, wasting, spasticity, dysarthria or dysphagia of one CNS region defined as bulbar, upper limb, lower limb or diaphragmatic; diagnosis (stage 2A); functional involvement of a second CNS region (stage 2B); functional involvement of a third CNS region (stage 3); the need for gastrostomy (4A); and the need for non-invasive ventilation (stage 4B).

Researchers calculated the time between milestones, standardizing them as proportions of time elapsed through the disease in patients who had died. They reported that diagnosis occurred at 35 percent through the disease course, involvement of a second CNS region at 38 percent; a third CNS region was involved at 61 percent, the need for gastrostomy at 77 percent; and noninvasive ventilation at 80 percent. Providing the staging in the context of months, for example, diagnosis occurred between 11.3-15.7 months and the need for noninvasive ventilation occurred between 26.4-34.2 months.

Dr Paulo Bittencourt ( from the Neurology Today text)

 

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