Rare diseases · Sign or symptom
Gowers sign
HP:0003391
What it means
A phenomenon whereby patients are not able to stand up without the use of the hands owing to weakness of the proximal muscles of the lower limbs.
Rare diseases that can present with this22
Common30–79%
12- Alpha-sarcoglycan-related limb-girdle muscular dystrophy R3
- Autosomal dominant adult-onset proximal spinal muscular atrophy
- Autosomal recessive centronuclear myopathy
- Beta-sarcoglycan-related limb-girdle muscular dystrophy R4
- BICD2-related autosomal dominant childhood-onset proximal spinal muscular atrophy
- DNA2-related mitochondrial DNA deletion syndrome
- Glycogen storage disease due to acid maltase deficiency
- Mitochondrial myopathy-cerebellar ataxia-pigmentary retinopathy syndrome
- Neutral lipid storage disease with myopathy
- Plectin-related limb-girdle muscular dystrophy R17
- POMT1-related limb-girdle muscular dystrophy R11
- Pure mitochondrial myopathy
Sometimes5–29%
9- Alpha-dystroglycan-related limb-girdle muscular dystrophy R16
- Autosomal recessive myogenic arthrogryposis multiplex congenita
- Bethlem muscular dystrophy
- Cap myopathy
- Congenital myasthenic syndrome with glycosylation defect
- Gamma-sarcoglycan-related limb-girdle muscular dystrophy R5
- Glycogen storage disease due to muscle phosphorylase kinase deficiency
- Rigid spine syndrome
and 1 more in this range
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: Gower sign · Positive Gower sign · Positive Gowers sign
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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.