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Fatiguable weakness of proximal limb muscles

MedGen UID:
868191
•Concept ID:
C4022583
•
Finding
Synonym: Proximal fatigable limb muscle weakness
 
HPO: HP:0030200

Definition

A type of weakness of a skeletal muscle of proximal part of a limb that occurs after a muscle group is used and lessens if the muscle group has some rest. That is, there is diminution of strength with repetitive muscle actions. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFatiguable weakness of proximal limb muscles

Recent clinical studies

Etiology

Clinical evaluation of muscle functions in neurofibromatosis type 1.
Gurler G, Altunbuker H, Cankaya O, Esen-Aydinli F, Incebay O, Sel SA, Lay I, Kerem-Gunel M, Anlar B
J Paediatr Child Health 2022 Nov;58(11):1997-2002. Epub 2022 Jul 23 doi: 10.1111/jpc.16133. PMID: 35869836
Identification of Caveolae-Associated Protein 4 Autoantibodies as a Biomarker of Immune-Mediated Rippling Muscle Disease in Adults.
Dubey D, Beecher G, Hammami MB, Knight AM, Liewluck T, Triplett J, Datta A, Dasari S, Zhang Y, Roforth MM, Jerde CR, Murphy SJ, Litchy WJ, Amato A, Lennon VA, McKeon A, Mills JR, Pittock SJ, Milone M
JAMA Neurol 2022 Aug 1;79(8):808-816. doi: 10.1001/jamaneurol.2022.1357. PMID: 35696196Free PMC Article
A study of impairments in oculopharyngeal muscular dystrophy.
Brisson JD, Gagnon C, Brais B, Côté I, Mathieu J
Muscle Nerve 2020 Aug;62(2):201-207. Epub 2020 May 22 doi: 10.1002/mus.26888. PMID: 32270505
Distal myopathy with ADSSL1 mutations in Korean patients.
Park HJ, Shin HY, Kim S, Kim SH, Lee Y, Lee JH, Hong JM, Kim SM, Park KD, Choi BO, Lee JH, Choi YC
Neuromuscul Disord 2017 May;27(5):465-472. Epub 2017 Feb 14 doi: 10.1016/j.nmd.2017.02.004. PMID: 28268051
Muscle strength in myasthenia gravis.
Cejvanovic S, Vissing J
Acta Neurol Scand 2014 Jun;129(6):367-73. Epub 2013 Oct 11 doi: 10.1111/ane.12193. PMID: 24117241

Diagnosis

Clinical evaluation of muscle functions in neurofibromatosis type 1.
Gurler G, Altunbuker H, Cankaya O, Esen-Aydinli F, Incebay O, Sel SA, Lay I, Kerem-Gunel M, Anlar B
J Paediatr Child Health 2022 Nov;58(11):1997-2002. Epub 2022 Jul 23 doi: 10.1111/jpc.16133. PMID: 35869836
Identification of Caveolae-Associated Protein 4 Autoantibodies as a Biomarker of Immune-Mediated Rippling Muscle Disease in Adults.
Dubey D, Beecher G, Hammami MB, Knight AM, Liewluck T, Triplett J, Datta A, Dasari S, Zhang Y, Roforth MM, Jerde CR, Murphy SJ, Litchy WJ, Amato A, Lennon VA, McKeon A, Mills JR, Pittock SJ, Milone M
JAMA Neurol 2022 Aug 1;79(8):808-816. doi: 10.1001/jamaneurol.2022.1357. PMID: 35696196Free PMC Article
A 60-Year-Old Man With Dyspnea, Proximal Muscle Weakness, and Pulmonary Arterial Hypertension.
Rogers E, Moffet EW, Allen N, Rivera-Zengotita M, Harden C, Ataya A
Chest 2021 Aug;160(2):e225-e231. doi: 10.1016/j.chest.2021.03.040. PMID: 34366049
Distal myopathy with ADSSL1 mutations in Korean patients.
Park HJ, Shin HY, Kim S, Kim SH, Lee Y, Lee JH, Hong JM, Kim SM, Park KD, Choi BO, Lee JH, Choi YC
Neuromuscul Disord 2017 May;27(5):465-472. Epub 2017 Feb 14 doi: 10.1016/j.nmd.2017.02.004. PMID: 28268051
INCAT disability score: a critical analysis of its measurement properties.
Breiner A, Barnett C, Bril V
Muscle Nerve 2014 Aug;50(2):164-9. Epub 2014 May 17 doi: 10.1002/mus.24207. PMID: 24723454

Therapy

Lower-Extremity Torque Capacity and Physical Function in Mobility-Limited Older Adults.
Grosicki GJ, Englund DA, Price L, Iwai M, Kashiwa M, Reid KF, Fielding RA
J Nutr Health Aging 2019;23(8):703-709. doi: 10.1007/s12603-019-1232-8. PMID: 31560027Free PMC Article
Lysosomal degradation of GMPPB is associated with limb-girdle muscular dystrophy type 2T.
Tian WT, Zhou HY, Zhan FX, Zhu ZY, Yang J, Chen SD, Luan XH, Cao L
Ann Clin Transl Neurol 2019 Jun;6(6):1062-1071. Epub 2019 May 8 doi: 10.1002/acn3.787. PMID: 31211170Free PMC Article
Diagnosis of Critical Illness Myopathy After Liver Transplantation and Muscle Condition Monitoring: A Case Report.
Jang MH, Yoon MH, Ahn SJ, Lee JW, Shin MJ
Transplant Proc 2018 Dec;50(10):4023-4027. Epub 2018 Jun 30 doi: 10.1016/j.transproceed.2018.06.026. PMID: 30577307
Clinical course and outcomes of Iranian children with juvenile dermatomyositis and polymyositis.
Malek A, Raeeskarami SR, Ziaee V, Aghighi Y, Moradinejad MH
Clin Rheumatol 2014 Aug;33(8):1113-8. Epub 2014 May 31 doi: 10.1007/s10067-014-2675-2. PMID: 24878926
Acute brachial diplegia due to Lyme disease.
Gorson KC, Kolb DA, Marks DS, Hayes MT, Baquis GD
Neurologist 2011 Jan;17(1):24-7. doi: 10.1097/NRL.0b013e31820038cd. PMID: 21192188

Prognosis

A study of impairments in oculopharyngeal muscular dystrophy.
Brisson JD, Gagnon C, Brais B, Côté I, Mathieu J
Muscle Nerve 2020 Aug;62(2):201-207. Epub 2020 May 22 doi: 10.1002/mus.26888. PMID: 32270505
Distal myopathy with ADSSL1 mutations in Korean patients.
Park HJ, Shin HY, Kim S, Kim SH, Lee Y, Lee JH, Hong JM, Kim SM, Park KD, Choi BO, Lee JH, Choi YC
Neuromuscul Disord 2017 May;27(5):465-472. Epub 2017 Feb 14 doi: 10.1016/j.nmd.2017.02.004. PMID: 28268051
Clinical course and outcomes of Iranian children with juvenile dermatomyositis and polymyositis.
Malek A, Raeeskarami SR, Ziaee V, Aghighi Y, Moradinejad MH
Clin Rheumatol 2014 Aug;33(8):1113-8. Epub 2014 May 31 doi: 10.1007/s10067-014-2675-2. PMID: 24878926
Muscle strength in myasthenia gravis.
Cejvanovic S, Vissing J
Acta Neurol Scand 2014 Jun;129(6):367-73. Epub 2013 Oct 11 doi: 10.1111/ane.12193. PMID: 24117241
Hip flexor fatigue limits walking in Charcot-Marie-Tooth disease.
Ramdharry GM, Day BL, Reilly MM, Marsden JF
Muscle Nerve 2009 Jul;40(1):103-11. doi: 10.1002/mus.21264. PMID: 19405092Free PMC Article

Clinical prediction guides

Clinical evaluation of muscle functions in neurofibromatosis type 1.
Gurler G, Altunbuker H, Cankaya O, Esen-Aydinli F, Incebay O, Sel SA, Lay I, Kerem-Gunel M, Anlar B
J Paediatr Child Health 2022 Nov;58(11):1997-2002. Epub 2022 Jul 23 doi: 10.1111/jpc.16133. PMID: 35869836
Lower-Extremity Torque Capacity and Physical Function in Mobility-Limited Older Adults.
Grosicki GJ, Englund DA, Price L, Iwai M, Kashiwa M, Reid KF, Fielding RA
J Nutr Health Aging 2019;23(8):703-709. doi: 10.1007/s12603-019-1232-8. PMID: 31560027Free PMC Article
Elicited Finger and Wrist Extension Through Transcutaneous Radial Nerve Stimulation.
Zheng Y, Hu X
IEEE Trans Neural Syst Rehabil Eng 2019 Sep;27(9):1875-1882. Epub 2019 Jul 24 doi: 10.1109/TNSRE.2019.2930669. PMID: 31352346
INCAT disability score: a critical analysis of its measurement properties.
Breiner A, Barnett C, Bril V
Muscle Nerve 2014 Aug;50(2):164-9. Epub 2014 May 17 doi: 10.1002/mus.24207. PMID: 24723454
Muscle strength in myasthenia gravis.
Cejvanovic S, Vissing J
Acta Neurol Scand 2014 Jun;129(6):367-73. Epub 2013 Oct 11 doi: 10.1111/ane.12193. PMID: 24117241

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