Rare diseases · Sign or symptom
Progressive proximal muscle weakness
HP:0009073
What it means
Lack of strength of the proximal muscles that becomes progressively more severe.
Removed logical definition that used anonymous class. Outstanding term request in UBERON for proximal muscle organ.
Rare diseases that can present with this20
Very common80–99%
6Common30–79%
7- Danon disease
- Distal myopathy with anterior tibial onset
- Intellectual disability-hyperkinetic movement-truncal ataxia syndrome
- Late-onset distal myopathy, Markesbery-Griggs type
- Mitochondrial DNA-related progressive external ophthalmoplegia
- Myopathy and diabetes mellitus
- TK2-related mitochondrial DNA maintenance defect, myopathic form
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Also called: Muscle weakness, progressive, proximal
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Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.