title: [Amyotrophic lateral sclerosis. Multisystem degeneration].
abstract: BACKGROUND
  There is increasing evidence that amyotrophic lateral sclerosis (ALS) has to be regarded as multisystem degeneration rather than as purely a motor neuron disease, as it also includes various dnonmotor symptoms. This modern view has been confirmed by neuropathological and imaging findings.
  
  
  OBJECTIVE
  To review recent findings supporting the idea of multisystem degeneration and to describe the implications for diagnostics and therapy.
  
  
  METHODS
  A discussion of recent clinical, imaging, and neuropathological findings is presented.
  
  
  RESULTS
  Symptoms of ALS include not only motor symptoms but also cognitive impairment, oculomotor abnormalities, and extrapyramidal and sensory symptoms. As a neuropathological correlate, a systematic spreading of "transactive response DNA binding protein 43 kDa" (TDP-43) over functionally connected cortical structures has been described.
  
  
  CONCLUSIONS
  Nonmotor symptoms are regularly seen in ALS, although they usually do not dominate the clinical picture. Recent neuropathological findings offer new perspectives for diagnostics and therapy in ALS.
authors.affiliation: Klinik für Neurologie, Universitätsklinikum Ulm, Universität Ulm, Oberer Eselsberg 45, 89081, Ulm, Deutschland.
authors.forename: A
authors.initials: A
authors.lastname: Hübers
authors.affiliation: Klinik für Neurologie, Universitätsklinikum Ulm, Universität Ulm, Oberer Eselsberg 45, 89081, Ulm, Deutschland.
authors.forename: A C
authors.initials: AC
authors.lastname: Ludolph
authors.affiliation: Klinik für Neurologie, Universitätsklinikum Ulm, Universität Ulm, Oberer Eselsberg 45, 89081, Ulm, Deutschland.
authors.forename: A
authors.initials: A
authors.lastname: Rosenbohm
authors.affiliation: Klinik für Neurologie, Universitätsklinikum Ulm, Universität Ulm, Oberer Eselsberg 45, 89081, Ulm, Deutschland.
authors.forename: E H
authors.initials: EH
authors.lastname: Pinkhardt
authors.affiliation: Klinik für Neurologie, Universitätsklinikum Ulm, Universität Ulm, Oberer Eselsberg 45, 89081, Ulm, Deutschland.
authors.forename: J H
authors.initials: JH
authors.lastname: Weishaupt
authors.affiliation: Klinik für Neurologie, Universitätsklinikum Ulm, Universität Ulm, Oberer Eselsberg 45, 89081, Ulm, Deutschland. johannes.dorst@uni-ulm.de.
authors.forename: J
authors.initials: J
authors.lastname: Dorst
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000628:therapy*; D003072:Cognition Disorders / Q000175:diagnosis* / Q000628:therapy*; D003937:Diagnosis, Differential; D019317:Evidence-Based Medicine; D006801:Humans; D015835:Ocular Motility Disorders / Q000175:diagnosis* / Q000628:therapy*; D063189:Symptom Assessment / Q000379:methods
keywords: Amyotrophic lateral sclerosis; DNA-binding proteins; Motor neuron disease; Nervous system diseases; Protein TDP-43