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Start free with EleplanAmyotrophic lateral sclerosis
ORPHA:803Disease
Also called ALS · Charcot disease · Lou Gehrig disease
What it is
A rare neurodegenerative disease characterized by progressive muscular paralysis reflecting degeneration of motor neurons in the primary motor cortex, corticospinal tracts, brainstem and spinal cord.
Key facts
- Prevalence
- 1-9 / 100 000 (Europe)
- Age of onset
- Adult
- Inheritance
- Autosomal dominant, Autosomal recessive, Multigenic/multifactorial, X-linked dominant
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Common30–79%
32- Abnormality on pulmonary function testing
- Anxiety
- Atypical behavior
- Babinski sign
- Cognitive impairment
- Depression
- Distal amyotrophy
- Drooling
- Dysarthria
- Dysphagia
- Dysphonia
- Dyspnea
- Emotional lability
- Fasciculations
- Fatigable weakness of bulbar muscles
- Fatigable weakness of respiratory muscles
- Fatigable weakness of swallowing muscles
- Fatigue
- Hoffmann sign
- Hyperreflexia
- Lower limb muscle weakness
- Muscle spasm
- Pain
- Paralysis
- Progressive distal muscular atrophy
- Respiratory failure
- Skeletal muscle atrophy
- Spasticity
- Tongue atrophy
- Upper limb muscle weakness
- Weight loss
- Xerostomia
Sometimes5–29%
10- Cachexia
- Foot dorsiflexor weakness
- Frontotemporal dementia
- Jaw hyperreflexia
- Language impairment
- Orthopnea
- Sleep abnormality
- Spastic paraparesis
and 2 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Benefit programs to look at
Programs whose eligibility touches the same ICD-10 categories. Eligibility is decided by the administering agency, never by this page.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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