title: [Amyotrophic lateral sclerosis--diagnosis and treatment].
abstract: Amyotrophic lateral sclerosis (ALS) represents the most common motoneuron disorder in adulthood. It is characterized by selective degeneration of the motoneurons. About 10% of patients have a genetically determined ALS. Clinically, ALS is characterized by coexistence of signs of the first motoneuron, such as spasticity and hyperreflexia, as well as the second motoneuron, such as muscular atrophy and fasciculations. If such signs are present in at least three regions and if other possible causes have been excluded, a definite diagnosis of ALS can be made based on the revised El-Escorial criteria. Initial manifestations are often focalized and generalization develops during the course. The glutamate antagonist riluzole is worldwide the only approved ALS treatment. However, symptomatic treatments to ameliorate spasticity, drooling, speech and swallowing problems, and assisted ventilation to treat respiratory failure are essential.
authors: Klinik für Neurologie und Neuromuskuläres Zentrum, Universitätsspital Zürich. hans.jung@usz.ch
authors: H H
authors: HH
authors: Jung
authors: M
authors: M
authors: Neumann
authors: K E
authors: KE
authors: Bloch
mesh_terms: D000203:Activities of Daily Living / Q000145:classification; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000188:drug therapy* / Q000235:genetics / Q000401:mortality; D000818:Animals; D002423:Cause of Death; D003430:Cross-Sectional Studies; D004252:DNA Mutational Analysis; D003937:Diagnosis, Differential; D004195:Disease Models, Animal; D018691:Excitatory Amino Acid Antagonists / Q000008:administration & dosage / Q000009:adverse effects; D005260:Female; D020022:Genetic Predisposition to Disease / Q000235:genetics; D006801:Humans; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000473:pathology; D009460:Neurologic Examination; D010166:Palliative Care; D019782:Riluzole / Q000008:administration & dosage / Q000009:adverse effects; D013116:Spinal Cord / Q000473:pathology; D016019:Survival Analysis; D057177:TDP-43 Proteinopathies / Q000473:pathology
chemical_list: D018691:Excitatory Amino Acid Antagonists; D019782:Riluzole