Amyotrophic lateral sclerosis (ALS) is a chronic progressive neuromuscular disorder characterized by loss of motor neurons in the cerebral cortex, motor nuclei of some cranial nerves and anterior horns of the spinal cord. At present, there is no therapy able to stop the neurodegenerative process, and ALS patients usually die due to respiratory failure. Riluzole is the first drug that has been shown to limit disease progression, in two double-blind, placebo-controlled trials. In the present study we investigated the effects of riluzole using the ALS Health State Scale in a series of 84 consecutive patients with definite or probable ALS, according to WFN diagnostic criteria. The scale allows the easy classification of disease progression based on self-sufficiency of the patient. The results show that riluzole is particularly effective in limiting disease progression when given in the early clinical stages of ALS. Under these conditions (stage 1 of the scale), stabilization was observed for at least 6 months in 79% of patients. Conversely, therapy with riluzole in late stages (stage 2 and 3 of the scale) did not affect disease progression as compared to the natural history of ALS. The efficacy of riluzole in the early stages of ALS is in accordance with the neuroprotective effects of the drug, and indicates that pharmacological therapy should be undertaken since the beginning of illness.
|Number of pages||8|
|Journal||Giornale di Neuropsicofarmacologia|
|Publication status||Published - Mar 2001|
ASJC Scopus subject areas
- Pharmaceutical Science
- Neuropsychology and Physiological Psychology