Spreading in ALS: The relative impact of upper and lower motor neuron involvement.
Journal
Annals of clinical and translational neurology
ISSN: 2328-9503
Titre abrégé: Ann Clin Transl Neurol
Pays: United States
ID NLM: 101623278
Informations de publication
Date de publication:
07 2020
07 2020
Historique:
received:
14
04
2020
revised:
16
05
2020
accepted:
18
05
2020
pubmed:
20
6
2020
medline:
23
7
2021
entrez:
20
6
2020
Statut:
ppublish
Résumé
To investigate disease spread in amyotrophic lateral sclerosis (ALS), and determine the influence of lower (LMN) and upper motor neuron (UMN) involvement. We assessed disease spread in ALS in 1376 consecutively studied patients, from five European centers, applying an agreed proforma to assess LMN and UMN signs. We defined the pattern of disease onset and progression from predominant UMN or lower motor neuron (LMN) dysfunction in bulbar, upper limbs, lower limbs, and thoracic regions Non-linear regression analysis was applied to fit the data to a model that described the relation between two random variables, graphically represented by an inverse exponential curve. We analyzed the probability, rate of spread, and both combined (area under the curve). We found that progression was more likely and quicker to or from the region of onset to close spinal regions. When the disease had a limb onset, bulbar motor neurons were more resistant. Furthermore, in the same time frame more patients progressed from bulbar to lower limbs than vice-versa, whether predominantly UMN or LMN involvement. Patients with initial thoracic involvement had a higher probability for rapid change. The presence of predominant UMN signs was associated with a faster caudal progression. Contiguous progression was leading pattern, and predominant UMN involvement is important in shortening the time for cranial-caudal spread. Our results can best be fitted to a model of independent LMN and UMN degeneration, with regional progression of LMN degeneration mostly by contiguity. UMN lesion causes an acceleration of rostral-caudal LMN loss.
Identifiants
pubmed: 32558369
doi: 10.1002/acn3.51098
pmc: PMC7359118
doi:
Types de publication
Journal Article
Multicenter Study
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
1181-1192Informations de copyright
© 2020 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.
Références
Amyotroph Lateral Scler Frontotemporal Degener. 2018 Feb;19(1-2):21-28
pubmed: 29037065
Eur Neurol. 2016;75(5-6):244-50
pubmed: 27165107
J Neurol Neurosurg Psychiatry. 2013 Jan;84(1):116-7
pubmed: 23033352
J Neurol Neurosurg Psychiatry. 2011 Nov;82(11):1244-9
pubmed: 21921087
Neurology. 2007 May 8;68(19):1571-5
pubmed: 17485643
J Neurol Neurosurg Psychiatry. 2014 Jan;85(1):85-91
pubmed: 24027298
Amyotroph Lateral Scler Frontotemporal Degener. 2017 Nov;18(7-8):505-510
pubmed: 28705085
Can J Neurol Sci. 1991 Aug;18(3 Suppl):435-8
pubmed: 1718581
Muscle Nerve. 1992 Feb;15(2):219-24
pubmed: 1549143
Neuropathology. 2020 Mar 10;:
pubmed: 32157757
Lancet. 2011 Mar 12;377(9769):942-55
pubmed: 21296405
Eur J Neurol. 2012 Jun;19(6):834-41
pubmed: 22248150
Neurology. 2003 Apr 22;60(8):1252-8
pubmed: 12707426
Acta Neuropathol. 2014 Sep;128(3):423-37
pubmed: 24916269
Nat Rev Neurol. 2013 Dec;9(12):708-14
pubmed: 24217521
Neurology. 2009 Sep 8;73(10):805-11
pubmed: 19738176
Muscle Nerve. 1992 Feb;15(2):226-8
pubmed: 1549145
Amyotroph Lateral Scler Other Motor Neuron Disord. 2000 Dec;1(5):293-9
pubmed: 11464847
Eura Medicophys. 2007 Dec;43(4):505-9
pubmed: 18084174
Neurology. 1996 Oct;47(4 Suppl 2):S71-81; discussion S81-2
pubmed: 8858055
Science. 2019 Apr 5;364(6435):89-93
pubmed: 30948552
Eur J Neurol. 2016 Jun;23(6):1117-25
pubmed: 27016147
Amyotroph Lateral Scler. 2010;11(1-2):63-6
pubmed: 19533450
Muscle Nerve. 2011 May;43(5):636-42
pubmed: 21484822