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
