Monomelic amyotrophy: clinical features

Evidence-based neurology checklist on monomelic amyotrophy: clinical features: 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…

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

Other features

Preserved functions

Differential diagnosis: major types

Progress and outcome

Synonyms

Acronym

References

  1. Talbot K. Monomelic amyotrophy: Hirayama’s disease. Pract Neurol 2004; 4:362-365.
  2. 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.
  3. Hirayama K. Juvenile muscular atrophy of distal upper extremity (Hirayama disease). Intern Med 2000; 39:283-290.
  4. Sagui E, Correa E, Ricobono D, Bregigeon M, Brosset C. A woman with forearm amyotrophy. BMJ Case Rep. 2009; 2009: bcr09.2009.2292.
  5. De Freitas MR, Nascimento OJ. Benign monomelic amyotrophy: a study of twenty-one cases. Arq Neuropsiquiatr 2000; 58:808-813.
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