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Motor neuron disease

MedGen UID:
38785
•Concept ID:
C0085084
•
Disease or Syndrome
Synonyms: Degenerative motor system disease; Motor neuron disorder
SNOMED CT: MND - motor neuron disease (37340000); Motor neuron disease (37340000)
 
Monarch Initiative: MONDO:0020128
Orphanet: ORPHA98503

Definition

A group of progressive, degenerative neurological disorders characterized by motor impairments. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMotor neuron disease
Follow this link to review classifications for Motor neuron disease in Orphanet.

Professional guidelines

PubMed

Spinal Muscular Atrophy: The Past, Present, and Future of Diagnosis and Treatment.
Nishio H, Niba ETE, Saito T, Okamoto K, Takeshima Y, Awano H
Int J Mol Sci 2023 Jul 26;24(15) doi: 10.3390/ijms241511939. PMID: 37569314Free PMC Article
Motor Neuron Disease: Pathophysiology, Diagnosis, and Management.
Foster LA, Salajegheh MK
Am J Med 2019 Jan;132(1):32-37. Epub 2018 Aug 1 doi: 10.1016/j.amjmed.2018.07.012. PMID: 30075105
Neuromuscular diseases: Diagnosis and management.
Mary P, Servais L, Vialle R
Orthop Traumatol Surg Res 2018 Feb;104(1S):S89-S95. Epub 2017 Nov 28 doi: 10.1016/j.otsr.2017.04.019. PMID: 29196274

Recent clinical studies

Etiology

Multisystem proteinopathy: Where myopathy and motor neuron disease converge.
Korb MK, Kimonis VE, Mozaffar T
Muscle Nerve 2021 Apr;63(4):442-454. Epub 2020 Nov 3 doi: 10.1002/mus.27097. PMID: 33145792
Atypical Motor Neuron Disease variants: Still a diagnostic challenge in Neurology.
Pinto WBVR, Debona R, Nunes PP, Assis ACD, Lopes CG, Bortholin T, Dias RB, Naylor FGM, Chieia MAT, Souza PVS, Oliveira ASB
Rev Neurol (Paris) 2019 Apr;175(4):221-232. Epub 2019 Mar 4 doi: 10.1016/j.neurol.2018.04.016. PMID: 30846210
Motor neuron disease in inherited neurometabolic disorders.
de Souza PVS, Bortholin T, Naylor FGM, Chieia MAT, de Rezende Pinto WBV, Oliveira ASB
Rev Neurol (Paris) 2018 Mar;174(3):115-124. Epub 2017 Nov 8 doi: 10.1016/j.neurol.2017.06.020. PMID: 29128155
Nocebo in motor neuron disease: systematic review and meta-analysis of placebo-controlled clinical trials.
Shafiq F, Mitsikostas DD, Zis P
Amyotroph Lateral Scler Frontotemporal Degener 2017 Nov;18(7-8):576-582. Epub 2017 Jun 8 doi: 10.1080/21678421.2017.1335325. PMID: 28593798
Motor neuron diversity in development and disease.
Kanning KC, Kaplan A, Henderson CE
Annu Rev Neurosci 2010;33:409-40. doi: 10.1146/annurev.neuro.051508.135722. PMID: 20367447

Diagnosis

Amyotrophic lateral sclerosis mimics.
Kwan J, Vullaganti M
Muscle Nerve 2022 Sep;66(3):240-252. Epub 2022 May 24 doi: 10.1002/mus.27567. PMID: 35607838
MND plus.
Turner MR
Pract Neurol 2019 Oct;19(5):376-377. Epub 2019 May 21 doi: 10.1136/practneurol-2019-002213. PMID: 31113829
Atypical Motor Neuron Disease variants: Still a diagnostic challenge in Neurology.
Pinto WBVR, Debona R, Nunes PP, Assis ACD, Lopes CG, Bortholin T, Dias RB, Naylor FGM, Chieia MAT, Souza PVS, Oliveira ASB
Rev Neurol (Paris) 2019 Apr;175(4):221-232. Epub 2019 Mar 4 doi: 10.1016/j.neurol.2018.04.016. PMID: 30846210
Motor Neuron Disease: Pathophysiology, Diagnosis, and Management.
Foster LA, Salajegheh MK
Am J Med 2019 Jan;132(1):32-37. Epub 2018 Aug 1 doi: 10.1016/j.amjmed.2018.07.012. PMID: 30075105
Progressive Muscular Atrophy.
Liewluck T, Saperstein DS
Neurol Clin 2015 Nov;33(4):761-73. doi: 10.1016/j.ncl.2015.07.005. PMID: 26515620

Therapy

Magnesium for skeletal muscle cramps.
Garrison SR, Korownyk CS, Kolber MR, Allan GM, Musini VM, Sekhon RK, Dugré N
Cochrane Database Syst Rev 2020 Sep 21;9(9):CD009402. doi: 10.1002/14651858.CD009402.pub3. PMID: 32956536Free PMC Article
Primary lateral sclerosis: consensus diagnostic criteria.
Turner MR, Barohn RJ, Corcia P, Fink JK, Harms MB, Kiernan MC, Ravits J, Silani V, Simmons Z, Statland J, van den Berg LH; Delegates of the 2nd International PLS Conference, Mitsumoto H
J Neurol Neurosurg Psychiatry 2020 Apr;91(4):373-377. Epub 2020 Feb 6 doi: 10.1136/jnnp-2019-322541. PMID: 32029539Free PMC Article
Treatment of fatigue in amyotrophic lateral sclerosis/motor neuron disease.
Gibbons C, Pagnini F, Friede T, Young CA
Cochrane Database Syst Rev 2018 Jan 2;1(1):CD011005. doi: 10.1002/14651858.CD011005.pub2. PMID: 29293261Free PMC Article
Symptomatic treatments for amyotrophic lateral sclerosis/motor neuron disease.
Ng L, Khan F, Young CA, Galea M
Cochrane Database Syst Rev 2017 Jan 10;1(1):CD011776. doi: 10.1002/14651858.CD011776.pub2. PMID: 28072907Free PMC Article
Progressive Muscular Atrophy.
Liewluck T, Saperstein DS
Neurol Clin 2015 Nov;33(4):761-73. doi: 10.1016/j.ncl.2015.07.005. PMID: 26515620

Prognosis

New knowledge on anti-IgLON5 disease.
Gaig C, Sabater L
Curr Opin Neurol 2024 Jun 1;37(3):316-321. Epub 2024 Apr 1 doi: 10.1097/WCO.0000000000001271. PMID: 38563128Free PMC Article
Amyotrophic lateral sclerosis mimics.
Kwan J, Vullaganti M
Muscle Nerve 2022 Sep;66(3):240-252. Epub 2022 May 24 doi: 10.1002/mus.27567. PMID: 35607838
Motor Neuron Disease: Pathophysiology, Diagnosis, and Management.
Foster LA, Salajegheh MK
Am J Med 2019 Jan;132(1):32-37. Epub 2018 Aug 1 doi: 10.1016/j.amjmed.2018.07.012. PMID: 30075105
Prognosis for patients with amyotrophic lateral sclerosis: development and validation of a personalised prediction model.
Westeneng HJ, Debray TPA, Visser AE, van Eijk RPA, Rooney JPK, Calvo A, Martin S, McDermott CJ, Thompson AG, Pinto S, Kobeleva X, Rosenbohm A, Stubendorff B, Sommer H, Middelkoop BM, Dekker AM, van Vugt JJFA, van Rheenen W, Vajda A, Heverin M, Kazoka M, Hollinger H, Gromicho M, Körner S, Ringer TM, Rödiger A, Gunkel A, Shaw CE, Bredenoord AL, van Es MA, Corcia P, Couratier P, Weber M, Grosskreutz J, Ludolph AC, Petri S, de Carvalho M, Van Damme P, Talbot K, Turner MR, Shaw PJ, Al-Chalabi A, Chiò A, Hardiman O, Moons KGM, Veldink JH, van den Berg LH
Lancet Neurol 2018 May;17(5):423-433. Epub 2018 Mar 26 doi: 10.1016/S1474-4422(18)30089-9. PMID: 29598923
A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/Riluzole Study Group.
Bensimon G, Lacomblez L, Meininger V
N Engl J Med 1994 Mar 3;330(9):585-91. doi: 10.1056/NEJM199403033300901. PMID: 8302340

Clinical prediction guides

Review: Microglia in motor neuron disease.
Ashford BA, Boche D, Cooper-Knock J, Heath PR, Simpson JE, Highley JR
Neuropathol Appl Neurobiol 2021 Feb;47(2):179-197. Epub 2020 Jul 21 doi: 10.1111/nan.12640. PMID: 32594542
Atypical Motor Neuron Disease variants: Still a diagnostic challenge in Neurology.
Pinto WBVR, Debona R, Nunes PP, Assis ACD, Lopes CG, Bortholin T, Dias RB, Naylor FGM, Chieia MAT, Souza PVS, Oliveira ASB
Rev Neurol (Paris) 2019 Apr;175(4):221-232. Epub 2019 Mar 4 doi: 10.1016/j.neurol.2018.04.016. PMID: 30846210
Establishment and 12-month progress of the New Zealand Motor Neurone Disease Registry.
Walker KL, Rodrigues MJ, Watson B, Reilly C, Scotter EL, Brunton H, Turnbull J, Roxburgh RH
J Clin Neurosci 2019 Feb;60:7-11. Epub 2018 Nov 22 doi: 10.1016/j.jocn.2018.11.034. PMID: 30473407
Neurons other than motor neurons in motor neuron disease.
Ruffoli R, Biagioni F, Busceti CL, Gaglione A, Ryskalin L, Gambardella S, Frati A, Fornai F
Histol Histopathol 2017 Nov;32(11):1115-1123. Epub 2017 Apr 11 doi: 10.14670/HH-11-895. PMID: 28397197
The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III).
Cedarbaum JM, Stambler N, Malta E, Fuller C, Hilt D, Thurmond B, Nakanishi A
J Neurol Sci 1999 Oct 31;169(1-2):13-21. doi: 10.1016/s0022-510x(99)00210-5. PMID: 10540002

Recent systematic reviews

Treatment for sialorrhea (excessive saliva) in people with motor neuron disease/amyotrophic lateral sclerosis.
James E, Ellis C, Brassington R, Sathasivam S, Young CA
Cochrane Database Syst Rev 2022 May 20;5(5):CD006981. doi: 10.1002/14651858.CD006981.pub3. PMID: 35593746Free PMC Article
Magnesium for skeletal muscle cramps.
Garrison SR, Korownyk CS, Kolber MR, Allan GM, Musini VM, Sekhon RK, Dugré N
Cochrane Database Syst Rev 2020 Sep 21;9(9):CD009402. doi: 10.1002/14651858.CD009402.pub3. PMID: 32956536Free PMC Article
Treatment of fatigue in amyotrophic lateral sclerosis/motor neuron disease.
Gibbons C, Pagnini F, Friede T, Young CA
Cochrane Database Syst Rev 2018 Jan 2;1(1):CD011005. doi: 10.1002/14651858.CD011005.pub2. PMID: 29293261Free PMC Article
Symptomatic treatments for amyotrophic lateral sclerosis/motor neuron disease.
Ng L, Khan F, Young CA, Galea M
Cochrane Database Syst Rev 2017 Jan 10;1(1):CD011776. doi: 10.1002/14651858.CD011776.pub2. PMID: 28072907Free PMC Article
Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND).
Miller RG, Mitchell JD, Moore DH
Cochrane Database Syst Rev 2012 Mar 14;2012(3):CD001447. doi: 10.1002/14651858.CD001447.pub3. PMID: 22419278Free PMC Article

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