Adherence to riluzole in patients with amyotrophic lateral sclerosis: an observational study

Abstract

ObjectiveRiluzole is the first drug approved to treat amyotrophic lateral sclerosis (ALS). Recently, an oral suspension (OS) of riluzole was made available. Thus, the aim of our study was to evaluate the adherence to 2 formulations of riluzole in patients with ALS.Patients and methodsWe enrolled 45 consecutive patients with ALS. At disease diagnosis, riluzole was prescribed in 2 different formulations depending on the severity of dysphagia (27/45 patients received tablets and 18/45 patients received OS). Side effects (SEs) and treatment adherence were investigated using a clinical questionnaire including the ©Morisky 8-item Medication Adherence Questionnaire.ResultsGastroenteric complaints were the most frequent SEs (58% in the tablet group and 48% in the OS group), followed by those at the nervous system (29% and 40%, respectively). No serious SEs related to treatment were reported. The rate of adherence to riluzole was independent of the formulation of the drug and consistent with other medications assumed for comorbidities (p=0.004). In the tablet group, low adherence was caused by SEs in 55.6% and by dysphagia in 44.4% of patients. In the OS group, SEs caused low adherence in 75% of patients. Independently of the drug formulation, patients with high or medium adherence to riluzole had a higher progression rate (p=0.002 and p=0.009, respectively) and a shorter time to generalization (TTG; p=0.01), compared to those with low adherence.ConclusionGastroenteric symptoms were the most frequent SE related to tablet as well as OS. The rate of adherence was independent of the formulation of riluzole and the number of medications assumed for comorbidities, and it was consistent with the severity of the disease. The low adherence was caused by dysphagia and SEs in the tablet group, whereas it was caused prevalently by SEs in the OS group.

The Impact of Cognitive and Behavioral Symptoms on ALS Patients and Their Caregivers

“…In general, studies of treatment adherence in ALS have not typically characterized non-motor symptoms in patient cohorts [e.g., physiotherapy exercises ( 42 ); respiratory support ( 43 ); tolerability of oral vs. tablet Riluzole ( 44 ); tolerability of early non-invasive ventilation use ( 45 )] and is an exclusionary criteria in some studies [e.g., aerobic exercise therapy vs. cognitive behavioral therapy ( 46 )]. It is therefore not surprising that motor predictors of treatment adherence are often reported.…”

Interstitial pneumonia and other adverse events in riluzole-administered amyotrophic lateral sclerosis patients: a retrospective observational study

“…In this study, ALS patients who showed ALT and AST levels > 3× of the upper limit of the normal level [2, 12] and presented with accompanying subjective symptoms, such as anorexia, nausea, and dizziness, were considered cases/patients with abnormal elevated liver enzymes. In discontinuation cases, biochemical analyses revealed abnormally elevated liver enzymes in five cases and elevated Krebs von den Lungen-6 (KL-6) and surfactant protein (SP)-D levels in four pneumonia cases.…”

“…Data regarding sex, age, disease type, initial symptoms, biochemical analyses before and after riluzole administration, and medical events and histories were recorded. When alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels were > 3× the upper limit of the normal level [2, 12] and accompanied by additional subjective symptoms, such as anorexia, nausea, or dizziness, liver dysfunction was considered and riluzole was discontinued. In addition, in cases of withdrawal, causes of discontinuation and durations of disease and drug administration were recorded.…”

Big data of clinical manifestations combined with neuroelectrophysiologic features in the early diagnosis of motor neuron disease

“…The current TNFα inhibitor thalidomide shows some promising results in animal models of FUS-ALS but does not appear to be effective in regulating disease progression in patients (Kia et al 2018). Riluzole blocks glutamate-mediated excitatory neurotransmission to reduce excitotoxicity and is currently the only drug of life to prolong ALS patients (Introna et al 2018). Bulbus medullae group 3.56/4.00 5.60/5.00 3.80/3.10 8.00/5.00 Cervical spinal cord group 3.79/4.00 2.60/5.00 3.20/3.10 5.50/5.00 Lumbosacral spinal cord group 3.80/4.00 6.70/5.00 3.20/3.10 8.30/5.00 (Koroglu et al 2017).…”