monomelic amyotrophy
MONDO:0011224Mondo
Findings
No curated finding names monomelic amyotrophy yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Monomelic amyotrophy (MA) is a rare benign lower motor neuron disorder characterized by muscular weakness and wasting in the distal upper extremities during adolescence followed by a spontaneous halt in progression and a stabilization of symptoms.
Definition from the Mondo Disease Ontology (MONDO:0011224), read 2026-09-29. CC BY 4.0.
Features
10 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.
- Abnormality of the upper limbHPOHP:0002817
- Very frequent (80% to 99% of cases)
- Distal upper limb amyotrophyHPOHP:0007149
- Very frequent (80% to 99% of cases)
- EMG abnormalityHPOHP:0003457
- Very frequent (80% to 99% of cases)
- Muscle weaknessHPOHP:0001324
- Very frequent (80% to 99% of cases)
- Abnormality of peripheral nerve conductionHPOHP:0003134
- Frequent (30% to 79% of cases)
- Degeneration of anterior horn cellsHPOHP:0002398
- Frequent (30% to 79% of cases)
- Abnormality of movementHPOHP:0100022
- Occasional (5% to 29% of cases)
- Abnormality of the immune systemHPOHP:0002715
- Occasional (5% to 29% of cases)
- FasciculationsHPOHP:0002380
- Occasional (5% to 29% of cases)
- TremorHPOHP:0001337
- Occasional (5% to 29% of cases)
Where it sits
- A kind of
- Narrower terms (1)
Other names
5 names
Resolves to: monomelic amyotrophy
- Also called
- benign focal amyotrophyHirayama diseaseJMADUEjuvenile muscular atrophy of distal upper extremityjuvenile muscular atrophy of the distal upper limb