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Brachial amyotrophic diplegia

MedGen UID:
1617621
•Concept ID:
C4522181
•
Disease or Syndrome
Synonyms: Brachial Amyotrophic Diplegia; brachial amyotrophic diplegia; FAS; Flail Arm Syndrome; flail arm syndrome; Man-in-barrel Syndrome; man-in-barrel syndrome
 
Monarch Initiative: MONDO:0020708

Definition

A neurodegenerative condition characterized by asymmetric weakness in the upper extremities resulting from segmental lower motor neuron dysfunction. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBrachial amyotrophic diplegia

Recent clinical studies

Etiology

Clinical and serological insights into paraneoplastic brachial amyotrophic diplegia.
Kherbek H, Itoh CY, Daley C, Eggers SD, Hinson S, Sarker P, Staff NP, Pittock SJ, Dubey D
J Neurol 2024 Jul;271(7):4620-4627. Epub 2024 May 22 doi: 10.1007/s00415-024-12425-x. PMID: 38772930
ALS Regional Variants (Brachial Amyotrophic Diplegia and Amyotrophic Leg Diplegia): Still A Diagnostic Challenge in Neurology.
Koh YH, Pang YH, Lim EW
Acta Neurol Taiwan 2023 Mar 30;32(1):42-47. PMID: 36474455
Pattern difference of dissociated hand muscle atrophy in amyotrophic lateral sclerosis and variants.
Kim JE, Hong YH, Lee JH, Ahn SW, Kim SM, Park KS, Sung JJ, Lee KW, Seong SY
Muscle Nerve 2015 Mar;51(3):333-7. Epub 2015 Jan 29 doi: 10.1002/mus.24323. PMID: 24958627
Leg amyotrophic diplegia: prevalence and pattern of weakness at US neuromuscular centers.
Dimachkie MM, Muzyka IM, Katz JS, Jackson C, Wang Y, McVey AL, Dick A, Pasnoor M, Mozaffar MT, Xiao-Song Z, Kissel JT, Ensrud E, Rosenfeld J, Barohn RJ
J Clin Neuromuscul Dis 2013 Sep;15(1):7-12. doi: 10.1097/CND.0b013e31829e22d1. PMID: 23965403Free PMC Article
Natural history and clinical features of the flail arm and flail leg ALS variants.
Wijesekera LC, Mathers S, Talman P, Galtrey C, Parkinson MH, Ganesalingam J, Willey E, Ampong MA, Ellis CM, Shaw CE, Al-Chalabi A, Leigh PN
Neurology 2009 Mar 24;72(12):1087-94. doi: 10.1212/01.wnl.0000345041.83406.a2. PMID: 19307543Free PMC Article

Diagnosis

Clinical and serological insights into paraneoplastic brachial amyotrophic diplegia.
Kherbek H, Itoh CY, Daley C, Eggers SD, Hinson S, Sarker P, Staff NP, Pittock SJ, Dubey D
J Neurol 2024 Jul;271(7):4620-4627. Epub 2024 May 22 doi: 10.1007/s00415-024-12425-x. PMID: 38772930
ALS Regional Variants (Brachial Amyotrophic Diplegia and Amyotrophic Leg Diplegia): Still A Diagnostic Challenge in Neurology.
Koh YH, Pang YH, Lim EW
Acta Neurol Taiwan 2023 Mar 30;32(1):42-47. PMID: 36474455
The M1311V variant of ATP7A is associated with impaired trafficking and copper homeostasis in models of motor neuron disease.
Bakkar N, Starr A, Rabichow BE, Lorenzini I, McEachin ZT, Kraft R, Chaung M, Macklin-Isquierdo S, Wingfield T, Carhart B, Zahler N, Chang WH, Bassell GJ, Betourne A, Boulis N, Alworth SV, Ichida JK, August PR, Zarnescu DC, Sattler R, Bowser R
Neurobiol Dis 2021 Feb;149:105228. Epub 2020 Dec 24 doi: 10.1016/j.nbd.2020.105228. PMID: 33359139Free PMC Article
Pattern difference of dissociated hand muscle atrophy in amyotrophic lateral sclerosis and variants.
Kim JE, Hong YH, Lee JH, Ahn SW, Kim SM, Park KS, Sung JJ, Lee KW, Seong SY
Muscle Nerve 2015 Mar;51(3):333-7. Epub 2015 Jan 29 doi: 10.1002/mus.24323. PMID: 24958627
Neuroborreliosis mimicking brachial amyotrophic diplegia.
te Lintelo MP, Koehler PJ, van Diepen TH, Beekman R
Clin Neurol Neurosurg 2012 Feb;114(2):159-60. Epub 2011 Oct 11 doi: 10.1016/j.clineuro.2011.09.008. PMID: 21996581

Therapy

The M1311V variant of ATP7A is associated with impaired trafficking and copper homeostasis in models of motor neuron disease.
Bakkar N, Starr A, Rabichow BE, Lorenzini I, McEachin ZT, Kraft R, Chaung M, Macklin-Isquierdo S, Wingfield T, Carhart B, Zahler N, Chang WH, Bassell GJ, Betourne A, Boulis N, Alworth SV, Ichida JK, August PR, Zarnescu DC, Sattler R, Bowser R
Neurobiol Dis 2021 Feb;149:105228. Epub 2020 Dec 24 doi: 10.1016/j.nbd.2020.105228. PMID: 33359139Free PMC Article
Brachial amyotrophic diplegia in the setting of complete HIV viral load suppression.
Cachia D, Izzy S, Ionete C, Salameh J
BMJ Case Rep 2012 Dec 6;2012 doi: 10.1136/bcr-2012-007632. PMID: 23220836Free PMC Article
Neuroborreliosis mimicking brachial amyotrophic diplegia.
te Lintelo MP, Koehler PJ, van Diepen TH, Beekman R
Clin Neurol Neurosurg 2012 Feb;114(2):159-60. Epub 2011 Oct 11 doi: 10.1016/j.clineuro.2011.09.008. PMID: 21996581

Prognosis

ALS Regional Variants (Brachial Amyotrophic Diplegia and Amyotrophic Leg Diplegia): Still A Diagnostic Challenge in Neurology.
Koh YH, Pang YH, Lim EW
Acta Neurol Taiwan 2023 Mar 30;32(1):42-47. PMID: 36474455
Amyotrophic Lateral Sclerosis Regional Variants (Brachial Amyotrophic Diplegia, Leg Amyotrophic Diplegia, and Isolated Bulbar Amyotrophic Lateral Sclerosis).
Jawdat O, Statland JM, Barohn RJ, Katz JS, Dimachkie MM
Neurol Clin 2015 Nov;33(4):775-85. Epub 2015 Sep 8 doi: 10.1016/j.ncl.2015.07.003. PMID: 26515621Free PMC Article
Flail arm syndrome with cytoplasmic vacuoles in remaining anterior horn motor neurons: A peculiar variant of amyotrophic lateral sclerosis.
Hino S, Sasaki S
Neuropathology 2015 Dec;35(6):582-6. Epub 2015 Jul 6 doi: 10.1111/neup.12223. PMID: 26149762
Pattern difference of dissociated hand muscle atrophy in amyotrophic lateral sclerosis and variants.
Kim JE, Hong YH, Lee JH, Ahn SW, Kim SM, Park KS, Sung JJ, Lee KW, Seong SY
Muscle Nerve 2015 Mar;51(3):333-7. Epub 2015 Jan 29 doi: 10.1002/mus.24323. PMID: 24958627
Natural history and clinical features of the flail arm and flail leg ALS variants.
Wijesekera LC, Mathers S, Talman P, Galtrey C, Parkinson MH, Ganesalingam J, Willey E, Ampong MA, Ellis CM, Shaw CE, Al-Chalabi A, Leigh PN
Neurology 2009 Mar 24;72(12):1087-94. doi: 10.1212/01.wnl.0000345041.83406.a2. PMID: 19307543Free PMC Article

Clinical prediction guides

The M1311V variant of ATP7A is associated with impaired trafficking and copper homeostasis in models of motor neuron disease.
Bakkar N, Starr A, Rabichow BE, Lorenzini I, McEachin ZT, Kraft R, Chaung M, Macklin-Isquierdo S, Wingfield T, Carhart B, Zahler N, Chang WH, Bassell GJ, Betourne A, Boulis N, Alworth SV, Ichida JK, August PR, Zarnescu DC, Sattler R, Bowser R
Neurobiol Dis 2021 Feb;149:105228. Epub 2020 Dec 24 doi: 10.1016/j.nbd.2020.105228. PMID: 33359139Free PMC Article
Pattern difference of dissociated hand muscle atrophy in amyotrophic lateral sclerosis and variants.
Kim JE, Hong YH, Lee JH, Ahn SW, Kim SM, Park KS, Sung JJ, Lee KW, Seong SY
Muscle Nerve 2015 Mar;51(3):333-7. Epub 2015 Jan 29 doi: 10.1002/mus.24323. PMID: 24958627

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