Summary
Amyotrophic lateral sclerosis is the most frequent motoneuron disease in adulthood. It is characterized by a selective loss of motoneurons, typically starting in the 5th to 7th decade of life. The pathogenesis of ALS is not completely clarified until now. Genetic factors, oxidative stress, altered RNA metabolism, mitochondrial dysfunction, inflammatory processes, dysfunction of axonal transport, glutamatergic exzitotoxicity, damage by intracellular protein aggregates and growth factor deficiency are discussed as important pathogenic factors. The clinical presentation is characterized by the occurrence of both central and peripheral pareses. Muscles innervated by caudal cranial nerves and muscles of the extremities are affected. Most often, after a focal disease onset, a rapid generalization occurs. At present the diagnosis is made according to the revised El Escorial criteria whereby diagnostic certainty increases with the number of body regions affected. Electromyographic examination is most important to ensure the diagnosis. Despite numerous drug trials, only the glutamate antagonist riluzole has been approved for the therapy of ALS. The treatment of ALS-associated symptoms such as spasticity, sialorrhoea, muscle cramps or others is of utmost importance.
Keywords
Amyotrophic lateral sclerosis - pathogenesis - clinical symptoms - clinical diagnostics - therapy
