Guideline “Motor neuron diseases” of the German Society of Neurology (Deutsche Gesellschaft für Neurologie)
DGN · 2023 · Germany
Source and licence
- Authors
- Susanne Petri, Torsten Grehl, Julian Grosskreutz et al.
- Title
- Guideline “Motor neuron diseases” of the German Society of Neurology (Deutsche Gesellschaft für Neurologie)
- Journal
- Neurological Research and Practice, 2023
- Licence
- CC BY 4.0
- Changes
- Verbatim excerpts; formatting adapted
Full text retrieved 2026-10-27 from Europe PMC · PMC10268388
Licence statement of the original publication
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This page reproduces excerpts of the original publication; the original is authoritative. It has not been reviewed by a physician, does not state a recommendation of Medelix and does not replace medical advice. The guideline may have been updated since.
Open the original publication →Full text on Europe PMC
Recommendations
We found no explicitly labelled recommendations in this publication; its recommendations are in the running text of the original.
Abstract
Introduction: In 2021, the Deutsche Gesellschaft für Neurology published a new guideline on diagnosis and therapy of motor neuron disorders. Motor neuron disorders affect upper motor neurons in the primary motor cortex and/or lower motor neurons in the brain stem and spinal cord. The most frequent motor neuron disease amyotrophic lateral sclerosis (ALS) is a rapidly progressive disease with an average life expectancy of 2–4 years with a yearly incidence of 3.1/100,000 in Central Europe (Rosenbohm et al. in J Neurol 264(4):749–757, 2017. 10.1007/s00415-017-8413-3). It is considered a rare disease mainly due to its low prevalence as a consequence of short disease duration.
Recommendations: These guidelines comprise recommendations regarding differential diagnosis, neuroprotective therapies and multidisciplinary palliative care including management of respiration and nutrition as well as provision of assistive devices and end-of-life situations.
Conclusion: Diagnostic and therapeutic guidelines are necessary due the comparatively high number of cases and the aggressive disease course. Given the low prevalence and the severe impairment of patients, it is often impossible to generate evidence-based data so that ALS guidelines are partially dependent on expert opinion.
Contents of the original publication
The section headings of the original.
- Introduction
- What’s new?
- The most important recommendations
- Guidelines in detail
- Diagnosis
- Diagnostics
- Genetic testing
- Therapy
- Disease modifying pharmacotherapy
- Physical and occupational therapy
- Chronic respiratory insufficiency
- Prevention of pneumonia
- Palliative care/terminal respiratory insufficiency
- Sialorrhea
- Laryngospasms
- Prevention of thrombosis
- Treatment of dysphagia and catabolism
- Dysarthria
- Depression
- Emotional lability
- Pain
- Muscle cramps
- Spasticity
- Anxiety
- Assistive devices
- Psychosocial care
- Coordination of care
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