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Interosseus muscle atrophy

MedGen UID:
339618
Concept ID:
C1846829
Finding
Synonym: Interosseous muscular atrophy
 
HPO: HP:0007181

Definition

Atrophy of the interosseus muscles (including the palmar interossei that lie on the anterior aspect of the metacarpals, the dorsal interosseus muscles of the hand, which lie between the intercarpals, the plantar interosseus muscles, which lie underneath the metatarsal bones, and the dorsal interossei, which are located between the metatarsal bones. [from HPO]

Conditions with this feature

Distal spinal muscular atrophy type 3
MedGen UID:
337659
Concept ID:
C1846823
Disease or Syndrome
Autosomal recessive distal hereditary motor neuronopathy-3 (HMNR3), also known as distal spinal muscular atrophy (DSMA) and distal hereditary motor neuronopathy (dHMN or HMN), is characterized by distal muscle weakness and wasting without significant sensory involvement. For a discussion of genetic heterogeneity of autosomal recessive HMN, see HMNR1 (604320). Harding (1993) classified autosomal recessive distal hereditary motor neuronopathy as dHMN IV (HMN4) and dHMN III (HMN3). Both have juvenile onset and differ only by less severe involvement in HMN3. However, Viollet et al. (2004) reported an extended Lebanese kindred in which both HMN III and HMN IV occurred, suggesting that the same gene was involved in both phenotypes (see Irobi et al., 2006).
Monomelic amyotrophy
MedGen UID:
356265
Concept ID:
C1865384
Disease or Syndrome
Monomelic amyotrophy, also known as Hirayama disease, is characterized by insidious onset of weakness and wasting of the muscles of the hand and forearm. It is usually unilateral, but can be bilateral. It occurs most commonly as a sporadic condition, is most common in young men, and follows a relatively benign course after a few years of progression (Nalini et al., 2004; Misra et al., 2005).
Autosomal recessive limb-girdle muscular dystrophy type 2Y
MedGen UID:
1385152
Concept ID:
C4511482
Disease or Syndrome
Autosomal recessive myopathy with rigid spine and distal joint contractures (MRRSDC) is characterized by onset of slowly progressive muscle weakness in the first or second decades of life. There is initial involvement of the proximal lower limbs, followed by distal upper and lower limb muscle weakness and atrophy. Other features include joint contractures, rigid spine, and restricted pulmonary function; some patients may have mild cardiac involvement (summary by Kayman-Kurekci et al., 2014).
Charcot-Marie-Tooth disease, axonal, mitochondrial form, 1
MedGen UID:
1731194
Concept ID:
C5435765
Disease or Syndrome
Mitochondrial form of axonal Charcot-Marie-Tooth disease-1 (CMTMA1) is inherited only through the maternal line. The disorder is characterized by onset of distal muscle weakness and atrophy mainly affecting the lower limbs and resulting in difficulty walking in the second decade of life, although both earlier and later onset can occur. Upper limb involvement often develops with time, and affected individuals have weakness and atrophy of the intrinsic hand muscles. Other features may include distal sensory impairment, foot deformities, scoliosis, hypo- or hyperreflexia, spastic paraparesis, and neurogenic bladder. Electrophysiologic studies are compatible with an axonal sensorimotor peripheral neuropathy, and muscle and nerve biopsy show evidence of mitochondrial dysfunction with decreased activities of respiratory complexes, mtDNA deletions, and mitochondrial hyperplasia (summary by Fay et al., 2020).
Peripheral motor neuropathy, childhood-onset, biotin-responsive
MedGen UID:
1809728
Concept ID:
C5676997
Disease or Syndrome
Childhood-onset biotin-responsive peripheral motor neuropathy (COMNB) is an autosomal recessive disorder characterized predominantly by the onset of distal muscle weakness and atrophy late in the first decade of life. The disorder predominantly affects the upper limbs and hands, resulting in difficulties with fine motor skills. Some patients may have lower limb involvement, resulting in gait difficulties. Electrophysiologic studies and muscle biopsy are consistent with chronic denervation with axonal and demyelinating features. Rare patients may have additional neurologic signs, including spasticity, ataxia, and cerebellar signs. Sensation is intact, and patients have normal cognitive development. Treatment with biotin, pantothenic acid, and lipoic acid may result in clinical improvement (Holling et al., 2022).

Recent clinical studies

Etiology

Bersch I, Fridén J
EBioMedicine 2021 Dec;74:103737. Epub 2021 Dec 9 doi: 10.1016/j.ebiom.2021.103737. PMID: 34896792Free PMC Article
Gawel M, Jamrozik Z, Szmidt-Salkowska E, Slawek J, Rowinska-Marcinska K
J Neurol Sci 2012 Aug 15;319(1-2):81-5. Epub 2012 May 28 doi: 10.1016/j.jns.2012.05.011. PMID: 22647584
Löscher WN, Stampfer-Kountchev M, Sawires M, Seppi K, Mueller J, Szubski C, Hirnsperger K, Brenneis C, Poewe W, Wenning GK
Mov Disord 2007 Jan 15;22(2):174-8. doi: 10.1002/mds.21242. PMID: 17133517
Auer-Grumbach M, Löscher WN, Wagner K, Petek E, Körner E, Offenbacher H, Hartung HP
Brain 2000 Aug;123 ( Pt 8):1612-23. doi: 10.1093/brain/123.8.1612. PMID: 10908191
Reiners K, Herdmann J, Freund HJ
Muscle Nerve 1989 Aug;12(8):647-59. doi: 10.1002/mus.880120806. PMID: 2674708

Diagnosis

Tankisi H, Pia H, Strunge K, Howells J, Cengiz B, Samusyte G, Koltzenburg M, Fuglsang-Frederiksen A, Bostock H
Amyotroph Lateral Scler Frontotemporal Degener 2023 Feb;24(1-2):139-147. Epub 2022 Jul 27 doi: 10.1080/21678421.2022.2101926. PMID: 35899374
Jokela ME, Jääskeläinen SK, Sandell S, Palmio J, Penttilä S, Saukkonen A, Soikkeli R, Udd B
J Neurol Sci 2015 Aug 15;355(1-2):143-6. Epub 2015 Jun 3 doi: 10.1016/j.jns.2015.06.002. PMID: 26059445
Gawel M, Jamrozik Z, Szmidt-Salkowska E, Slawek J, Rowinska-Marcinska K
J Neurol Sci 2012 Aug 15;319(1-2):81-5. Epub 2012 May 28 doi: 10.1016/j.jns.2012.05.011. PMID: 22647584
Andreisek G, Kilgus M, Burg D, Saupe N, Crook DW, Meyer V, Marincek B, Weishaupt D
AJR Am J Roentgenol 2005 Oct;185(4):930-9. doi: 10.2214/AJR.04.1644. PMID: 16177411
Auer-Grumbach M, Löscher WN, Wagner K, Petek E, Körner E, Offenbacher H, Hartung HP
Brain 2000 Aug;123 ( Pt 8):1612-23. doi: 10.1093/brain/123.8.1612. PMID: 10908191

Prognosis

Mohseny B, Nijhuis TH, Hundepool CA, Janssen WG, Selles RW, Coert JH
Arch Phys Med Rehabil 2015 May;96(5):845-53. Epub 2014 Dec 4 doi: 10.1016/j.apmr.2014.11.014. PMID: 25482051

Clinical prediction guides

Jokela ME, Jääskeläinen SK, Sandell S, Palmio J, Penttilä S, Saukkonen A, Soikkeli R, Udd B
J Neurol Sci 2015 Aug 15;355(1-2):143-6. Epub 2015 Jun 3 doi: 10.1016/j.jns.2015.06.002. PMID: 26059445
Mohseny B, Nijhuis TH, Hundepool CA, Janssen WG, Selles RW, Coert JH
Arch Phys Med Rehabil 2015 May;96(5):845-53. Epub 2014 Dec 4 doi: 10.1016/j.apmr.2014.11.014. PMID: 25482051
Gawel M, Jamrozik Z, Szmidt-Salkowska E, Slawek J, Rowinska-Marcinska K
J Neurol Sci 2012 Aug 15;319(1-2):81-5. Epub 2012 May 28 doi: 10.1016/j.jns.2012.05.011. PMID: 22647584
Andreisek G, Kilgus M, Burg D, Saupe N, Crook DW, Meyer V, Marincek B, Weishaupt D
AJR Am J Roentgenol 2005 Oct;185(4):930-9. doi: 10.2214/AJR.04.1644. PMID: 16177411
Auer-Grumbach M, Löscher WN, Wagner K, Petek E, Körner E, Offenbacher H, Hartung HP
Brain 2000 Aug;123 ( Pt 8):1612-23. doi: 10.1093/brain/123.8.1612. PMID: 10908191

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