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Hand muscle weakness

MedGen UID:
66798
Concept ID:
C0239831
Finding
SNOMED CT: Hand muscle weakness (298283006)
 
HPO: HP:0030237

Definition

Reduced strength of the musculature of the hand. [from HPO]

Conditions with this feature

Charcot-Marie-Tooth disease X-linked dominant 1
MedGen UID:
98290
Concept ID:
C0393808
Disease or Syndrome
GJB1 disorders are typically characterized by peripheral motor and sensory neuropathy with or without fixed CNS abnormalities and/or acute, self-limited episodes of transient neurologic dysfunction (especially weakness and dysarthria). Peripheral neuropathy typically manifests in affected males between ages five and 25 years. Although both men and women are affected, manifestations tend to be less severe in women, some of whom may remain asymptomatic. Less commonly, initial manifestations in some affected individuals are stroke-like episodes (acute fulminant episodes of reversible CNS dysfunction).
Hereditary liability to pressure palsies
MedGen UID:
98291
Concept ID:
C0393814
Disease or Syndrome
Hereditary neuropathy with liability to pressure palsies (HNPP) is characterized by recurrent acute sensory and motor neuropathy in a single or multiple nerves. The most common initial manifestation is the acute onset of a non-painful focal sensory and motor neuropathy in a single nerve (mononeuropathy). The first attack usually occurs in the second or third decade but earlier onset is possible. Neuropathic pain is increasingly recognized as a common manifestation. Recovery from acute neuropathy is usually complete; when recovery is not complete, the resulting disability is mild. Some affected individuals also demonstrate a mild-to-moderate peripheral neuropathy.
Charcot-Marie-Tooth disease dominant intermediate C
MedGen UID:
334023
Concept ID:
C1842237
Disease or Syndrome
A rare hereditary motor and sensory neuropathy characterized by intermediate motor median nerve conduction velocities (usually between 25 and 60 m/s). It presents with moderately severe, slowly progressive usual clinical features of Charcot-Marie-Tooth disease (muscle weakness and atrophy of the distal extremities, distal sensory loss, reduced or absent deep tendon reflexes, feet deformities, extensor digitorum brevis atrophy). Findings in nerve biopsies include age-dependent axonal degeneration, reduced number of large myelinated fibers, segmental remyelination, and no onion bulbs.
Neuronopathy, distal hereditary motor, type 7B
MedGen UID:
375157
Concept ID:
C1843315
Disease or Syndrome
The spectrum of DCTN1-related neurodegeneration includes Perry syndrome, distal hereditary motor neuronopathy type 7B (dHMN7B), frontotemporal dementia (FTD), motor neuron disease / amyotrophic lateral sclerosis (ALS), and progressive supranuclear palsy. Some individuals present with overlapping phenotypes (e.g., FTD-ALS, Perry syndrome-dHMN7B). Perry syndrome (the most common of the phenotypes associated with DCTN1) is characterized by parkinsonism, neuropsychiatric symptoms, hypoventilation, and weight loss. The mean age of onset in those with Perry syndrome is 49 years (range: 35-70 years), and the mean disease duration is five years (range: 2-14 years). In most affected persons, the reported cause/circumstance of death relates to sudden death/hypoventilation or suicide.
Neuronopathy, distal hereditary motor, type 2C
MedGen UID:
461969
Concept ID:
C3150619
Disease or Syndrome
Any neuronopathy, distal hereditary motor in which the cause of the disease is a mutation in the HSPB3 gene.
Distal myopathy, Tateyama type
MedGen UID:
482073
Concept ID:
C3280443
Disease or Syndrome
CAV3-related distal myopathy is one form of distal myopathy, a group of disorders characterized by weakness and loss of function affecting the muscles farthest from the center of the body (distal muscles), such as those of the hands and feet. People with CAV3-related distal myopathy experience wasting (atrophy) and weakness of the small muscles in the hands and feet that generally become noticeable in adulthood. A bump or other sudden impact on the muscles, especially those in the forearms, may cause them to exhibit repetitive tensing (percussion-induced rapid contraction). The rapid contractions can continue for up to 30 seconds and may be painful. Overgrowth (hypertrophy) of the calf muscles can also occur in CAV3-related distal myopathy. The muscles closer to the center of the body (proximal muscles) such as the thighs and upper arms are normal in this condition.
Charcot-Marie-Tooth disease X-linked dominant 6
MedGen UID:
813032
Concept ID:
C3806702
Disease or Syndrome
A rare genetic principally axonal peripheral sensorimotor neuropathy with an X-linked dominant inheritance pattern and the childhood-onset of slowly progressive, moderate to severe, distal muscle weakness and atrophy of the lower extremities, as well as distal, pan modal sensory abnormalities, bilateral foot deformities (pes cavus, clawed toes), absent ankle reflexes and gait abnormalities (steppage gait). Females are usually asymptomatic or only present mild manifestations (mild postural hand tremor, mild wasting of hand intrinsic muscles).
Amyotrophic lateral sclerosis type 21
MedGen UID:
813851
Concept ID:
C3807521
Disease or Syndrome
Amyotrophic lateral sclerosis-21 (ALS21) is an autosomal dominant neurodegenerative disorder affecting upper and lower motor neurons, resulting in muscle weakness and respiratory failure. Some patients may develop myopathic features or dementia (summary by Johnson et al., 2014). For a discussion of genetic heterogeneity of amyotrophic lateral sclerosis, see ALS1 (105400).
Polyglucosan body myopathy type 2
MedGen UID:
863889
Concept ID:
C4015452
Disease or Syndrome
Polyglucosan body myopathy-2 is an autosomal recessive disorder characterized by proximal muscle weakness of the lower limbs resulting in gait disturbances. Some patients also have involvement of the upper limbs and/or distal muscle weakness. The age at onset is highly variable, and the disorder is slowly progressive. Muscle biopsy shows accumulation of polyglucosan, which contains abnormally long and poorly branched glucosyl chains and is variably resistant to digestion by alpha-amylase (summary by Malfatti et al., 2014). For a discussion of genetic heterogeneity of PGBM, see PGBM1 (615895).
Charcot-Marie-Tooth disease axonal type 2U
MedGen UID:
906504
Concept ID:
C4084821
Disease or Syndrome
Charcot-Marie-Tooth disease type 2U (CMT2U) is an autosomal dominant neurologic disorder characterized by late-adult onset of distal sensory impairment resulting in distal muscle weakness and atrophy affecting the upper and lower limbs. The disorder is slowly progressive (summary by Gonzalez et al., 2013). For a phenotypic description and a discussion of genetic heterogeneity of axonal CMT, see CMT2A1 (118210).

Professional guidelines

PubMed

Coletta G, Phillips SM
Ageing Res Rev 2023 Apr;86:101883. Epub 2023 Feb 13 doi: 10.1016/j.arr.2023.101883. PMID: 36792012
Gstoettner C, Mayer JA, Rassam S, Hruby LA, Salminger S, Sturma A, Aman M, Harhaus L, Platzgummer H, Aszmann OC
J Neurol Neurosurg Psychiatry 2020 Aug;91(8):879-888. Epub 2020 Jun 2 doi: 10.1136/jnnp-2020-323164. PMID: 32487526
Didesch JT, Tang P
J Hand Surg Am 2019 Apr;44(4):321-330. Epub 2018 Oct 3 doi: 10.1016/j.jhsa.2018.08.008. PMID: 30292717

Recent clinical studies

Etiology

Senet P, Maillard H, Diot E, Lazareth I, Blaise S, Arnault JP, Pistorius MA, Boulon C, Cogrel O, Warzocha U, Rivière S, Malloizel-Delaunay J, Servettaz A, Sassolas B, Viguier M, Monfort JB, Janique S, Vicaut E; BRASS collaborators
Arthritis Rheumatol 2023 Mar;75(3):459-467. Epub 2022 Dec 14 doi: 10.1002/art.42342. PMID: 36066501
Du W, Zhou M, Zhang C, Sun Q
Dermatol Ther 2022 Jul;35(7):e15529. Epub 2022 Apr 27 doi: 10.1111/dth.15529. PMID: 35441772
Dos Santos AM, Misse RG, Borges IBP, Pereira RMR, Shinjo SK
Reumatismo 2021 Jan 18;72(4):197-206. doi: 10.4081/reumatismo.2020.1298. PMID: 33677946
Sadeh M, Dabby R
J Clin Neurosci 2018 Jan;47:111-115. Epub 2017 Oct 31 doi: 10.1016/j.jocn.2017.10.071. PMID: 29097135
Necking LE, Lundborg G, Friden J
J Hand Surg Br 2002 Dec;27(6):520-5. doi: 10.1054/jhsb.2002.0810. PMID: 12475507

Diagnosis

Du W, Zhou M, Zhang C, Sun Q
Dermatol Ther 2022 Jul;35(7):e15529. Epub 2022 Apr 27 doi: 10.1111/dth.15529. PMID: 35441772
Portaro S, Naro A, Leo A, Cimino V, Balletta T, Buda A, Accorinti M, Calabrò RS
Medicine (Baltimore) 2019 Nov;98(46):e17582. doi: 10.1097/MD.0000000000017582. PMID: 31725606Free PMC Article
Sadeh M, Dabby R
J Clin Neurosci 2018 Jan;47:111-115. Epub 2017 Oct 31 doi: 10.1016/j.jocn.2017.10.071. PMID: 29097135
Suresh M, Nicholls PG, Das L, Van Brakel WH
Lepr Rev 2008 Sep;79(3):277-94. PMID: 19009977
Kim KK
J Korean Med Sci 1996 Apr;11(2):158-64. doi: 10.3346/jkms.1996.11.2.158. PMID: 8835764Free PMC Article

Therapy

Senet P, Maillard H, Diot E, Lazareth I, Blaise S, Arnault JP, Pistorius MA, Boulon C, Cogrel O, Warzocha U, Rivière S, Malloizel-Delaunay J, Servettaz A, Sassolas B, Viguier M, Monfort JB, Janique S, Vicaut E; BRASS collaborators
Arthritis Rheumatol 2023 Mar;75(3):459-467. Epub 2022 Dec 14 doi: 10.1002/art.42342. PMID: 36066501
Du W, Zhou M, Zhang C, Sun Q
Dermatol Ther 2022 Jul;35(7):e15529. Epub 2022 Apr 27 doi: 10.1111/dth.15529. PMID: 35441772
Englert H, Joyner E, Thompson M, Garcia H, Chambers P, Horner D, Hunt C, Makaroff J, O'Connor H, Russell N, March L
Intern Med J 2004 Dec;34(12):668-76. doi: 10.1111/j.1445-5994.2004.00670.x. PMID: 15610211
Necking LE, Lundborg G, Friden J
J Hand Surg Br 2002 Dec;27(6):520-5. doi: 10.1054/jhsb.2002.0810. PMID: 12475507
Koskimies K, Pyykkö I, Starck J, Inaba R
Int Arch Occup Environ Health 1992;64(4):251-6. doi: 10.1007/BF00378282. PMID: 1468793

Prognosis

Suresh M, Nicholls PG, Das L, Van Brakel WH
Lepr Rev 2008 Sep;79(3):277-94. PMID: 19009977
Kim KK
J Korean Med Sci 1996 Apr;11(2):158-64. doi: 10.3346/jkms.1996.11.2.158. PMID: 8835764Free PMC Article
Koskimies K, Pyykkö I, Starck J, Inaba R
Int Arch Occup Environ Health 1992;64(4):251-6. doi: 10.1007/BF00378282. PMID: 1468793

Clinical prediction guides

Senet P, Maillard H, Diot E, Lazareth I, Blaise S, Arnault JP, Pistorius MA, Boulon C, Cogrel O, Warzocha U, Rivière S, Malloizel-Delaunay J, Servettaz A, Sassolas B, Viguier M, Monfort JB, Janique S, Vicaut E; BRASS collaborators
Arthritis Rheumatol 2023 Mar;75(3):459-467. Epub 2022 Dec 14 doi: 10.1002/art.42342. PMID: 36066501
Du W, Zhou M, Zhang C, Sun Q
Dermatol Ther 2022 Jul;35(7):e15529. Epub 2022 Apr 27 doi: 10.1111/dth.15529. PMID: 35441772
Dos Santos AM, Misse RG, Borges IBP, Pereira RMR, Shinjo SK
Reumatismo 2021 Jan 18;72(4):197-206. doi: 10.4081/reumatismo.2020.1298. PMID: 33677946
Necking LE, Lundborg G, Friden J
J Hand Surg Br 2002 Dec;27(6):520-5. doi: 10.1054/jhsb.2002.0810. PMID: 12475507
Kim KK
J Korean Med Sci 1996 Apr;11(2):158-64. doi: 10.3346/jkms.1996.11.2.158. PMID: 8835764Free PMC Article

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