lateral sclerosis
Findings
No curated finding names lateral sclerosis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Primary lateral sclerosis (PLS) is an idiopathic non-familial motor neuron disease characterized by slowly progressive upper motor neuron dysfunction leading to spasticity, mild weakness in voluntary muscle movement, hyperreflexia, and loss of motor speech production.
Definition from the Mondo Disease Ontology (MONDO:0018155), read 2026-09-29. CC BY 4.0.
Features
23 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Abnormal upper motor neuron morphologyHPOHP:0002127
- Very frequent (80% to 99% of cases)
- Babinski signHPOHP:0003487
- Very frequent (80% to 99% of cases)
- Generalized hyperreflexiaHPOHP:0007034
- Very frequent (80% to 99% of cases)
- SpasticityHPOHP:0001257
- Very frequent (80% to 99% of cases)
- Upper motor neuron dysfunctionHPOHP:0002493
- Very frequent (80% to 99% of cases)
- DysphagiaHPOHP:0002015
- Frequent (30% to 79% of cases)
- EMG: chronic denervation signsHPOHP:0003444
- Frequent (30% to 79% of cases)
- Gait imbalanceHPOHP:0002141
- Frequent (30% to 79% of cases)
- Hoffmann signHPOHP:0031993
- Frequent (30% to 79% of cases)
- Impaired smooth pursuitHPOHP:0007772
- Frequent (30% to 79% of cases)
- IncoordinationHPOHP:0002311
- Frequent (30% to 79% of cases)
- Loss of speechHPOHP:0002371
- Frequent (30% to 79% of cases)
Reported absent (3)
- Abnormal lower motor neuron morphologyHPOHP:0002366
- Abnormality of extrapyramidal motor functionHPOHP:0002071
- Somatic sensory dysfunctionHPOHP:0003474
Show the remaining 11
- Progressive spastic paraparesisHPOHP:0007199
- Frequent (30% to 79% of cases)
- Pseudobulbar signsHPOHP:0002200
- Frequent (30% to 79% of cases)
- Spastic dysarthriaHPOHP:0002464
- Frequent (30% to 79% of cases)
- Spastic gaitHPOHP:0002064
- Frequent (30% to 79% of cases)
- Urinary urgencyHPOHP:0000012
- Frequent (30% to 79% of cases)
- Weakness due to upper motor neuron dysfunctionHPOHP:0010549
- Frequent (30% to 79% of cases)
Genes
1 gene
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- SPG7HGNC:11237
- Supportive · Orphanet · Autosomal dominant · 2021
Where it sits
- A kind of
- Narrower terms (2)
Other names
4 names
Resolves to: lateral sclerosis
- Also called
- adult-onset PLSadult-onset primary lateral sclerosisPLSprimary lateral sclerosis