Rare diseases · Sign or symptom
Proximal upper limb muscle weakness
HP:0008997
What it means
A lack of strength of the proximal muscles of the arms.
Rare diseases that can present with this27
Common30–79%
13- Anoctamin-5-related limb-girdle muscular dystrophy R12
- Autosomal dominant centronuclear myopathy
- Autosomal dominant Emery-Dreifuss muscular dystrophy
- Autosomal recessive Emery-Dreifuss muscular dystrophy
- Emery-Dreifuss muscular dystrophy
- Hereditary coproporphyria
- ISPD-related limb-girdle muscular dystrophy R20
- Kyphoscoliosis-lateral tongue atrophy-hereditary spastic paraplegia syndrome
- LIPE-related familial partial lipodystrophy
- Multiminicore myopathy
- Overlap myositis
- Variegate porphyria
- X-linked Emery-Dreifuss muscular dystrophy
Sometimes5–29%
11- Acute intermittent porphyria
- Autosomal dominant Charcot-Marie-Tooth disease type 2Y
- Autosomal dominant Charcot-Marie-Tooth disease type 2Z
- Autosomal dominant mitochondrial myopathy with exercise intolerance
- Charcot-Marie-Tooth disease type 1F
- Charcot-Marie-Tooth disease type 4B2
- Distal anoctaminopathy
- Dysferlin-related limb-girdle muscular dystrophy R2
and 3 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: Proximal muscle weakness in upper limbs
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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.