Monomelic amyotrophy: clinical features
Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .
Monomelic amyotrophy: clinical features. This typically affects the distal limb muscles: the C7-T1 innervated muscles.
Weakness and wasting
- This typically affects the distal limb muscles: the C7-T1 innervated muscles
- Weakness may demonstrate cold paresis: transient worsening in the cold
- Reflexes may be normal, reduced, or increased
Patterns of weakness and wasting
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Other features
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Preserved functions
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Differential diagnosis: major types
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Progress and outcome
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Synonyms
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Acronym
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References
- Talbot K. Monomelic amyotrophy: Hirayama’s disease. Pract Neurol 2004; 4:362-365.
- Hassan KM, Sahni H. Nosology of juvenile muscular atrophy of distal upper extremity: from monomelic amyotrophy to Hirayama disease-Indian perspective. Biomed Res Int 2013; 2013:478516.
- Hirayama K. Juvenile muscular atrophy of distal upper extremity (Hirayama disease). Intern Med 2000; 39:283-290.
- Sagui E, Correa E, Ricobono D, Bregigeon M, Brosset C. A woman with forearm amyotrophy. BMJ Case Rep. 2009; 2009: bcr09.2009.2292.
- De Freitas MR, Nascimento OJ. Benign monomelic amyotrophy: a study of twenty-one cases. Arq Neuropsiquiatr 2000; 58:808-813.
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