horizontal gaze palsy with progressive scoliosis
Findings
No curated finding names horizontal gaze palsy with progressive scoliosis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare congenital autosomal recessive disease, presenting in children and adolescents, and characterized by progressive scoliosis along with the absence of conjugate horizontal eye movements and associated with failure of the somatosensory and corticospinal neuronal tracts to decussate in the medulla.
Definition from the Mondo Disease Ontology (MONDO:0011810), read 2026-09-29. CC BY 4.0.
Features
16 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.
- Horizontal supranuclear gaze palsyHPOHP:0007817
- Very frequent (80% to 99% of cases)
- KyphosisHPOHP:0002808
- Very frequent (80% to 99% of cases)
- ScoliosisHPOHP:0002650
- Very frequent (80% to 99% of cases)
- Cognitive impairmentHPOHP:0100543
- Frequent (30% to 79% of cases)
- Delayed skeletal maturationHPOHP:0002750
- Frequent (30% to 79% of cases)
- Duane anomalyHPOHP:0009921
- Frequent (30% to 79% of cases)
- Hypotonia
Show the remaining 4
- StrabismusHPO · MondoHP:0000486
- Frequent (30% to 79% of cases)
- Visual impairmentHPOHP:0000505
- Frequent (30% to 79% of cases)
- SeizureHPOHP:0001250
- Occasional (5% to 29% of cases)
- Sensorineural hearing impairmentHPOHP:0000407
- Occasional (5% to 29% of cases)
Where it sits
Other names
2 names
Resolves to: horizontal gaze palsy with progressive scoliosis
- Also called
- HGPPSprogressive external ophthalmoplegia and scoliosis