RESUMO
Amyotrophic lateral sclerosis (ALS) is an unrelenting progressive neurodegenerative disease causing progressive weakness, ultimately leading to death. Despite aggressive research, the pathways leading to neuronal death are incompletely understood. Riluzole is the only drug clinically proven to enhance survival of ALS patients, but its mechanism of action is not clearly understood. In this article, the proposed pathophysiology of ALS is reviewed including glutamate excitotoxicity, oxidative stress, mitochondrial dysfunction, autoimmune mechanisms, protein aggregation, SOD1 accumulation, and neuronal death. Based on these mechanisms, past major ALS drug studies will be reviewed as well as promising current ALS drug studies, focusing on the advancement of these studies from the bench to the patient's bedside.
Assuntos
Esclerose Lateral Amiotrófica/tratamento farmacológico , Esclerose Lateral Amiotrófica/patologia , Esclerose Lateral Amiotrófica/terapia , Antioxidantes/uso terapêutico , Antagonistas de Aminoácidos Excitatórios/uso terapêutico , Terapia Genética , Humanos , Imidazóis/uso terapêutico , Imunossupressores/uso terapêutico , Pirazinas/uso terapêutico , Transplante de Células-Tronco , Superóxido Dismutase/biossíntese , Superóxido Dismutase/genética , Superóxido Dismutase-1RESUMO
Acute viral myositis is a rare condition that occurs during the recovery phase of an illness, most commonly influenza. It is characterized by muscle pain and weakness with an isolated laboratory finding of elevated serum creatine kinase (CK). We describe three previously healthy patients who were hospitalized after developing myositis following influenza-like illness during the 2009 influenza A (H1N1) virus pandemic. All experienced myalgias and weakness in all four extremities, including distal upper extremities, associated with an elevated CK level that resolved along with their myalgias and weakness within one week with supportive care. These cases serve as a reminder that influenza-related myositis may have atypical characteristics depending on the strain of influenza, and clinicians should be open to this possibility when new outbreaks occur.