A population-based epidemiologic study of adult neuromuscular disease in the Republic of Ireland
- PMID: 27927941
- DOI: 10.1212/WNL.0000000000003504
A population-based epidemiologic study of adult neuromuscular disease in the Republic of Ireland
Abstract
Objective: To estimate the prevalence rates (PRs) of acquired and inherited neuromuscular diseases (NMD) in the adult Irish population, reflecting the burden of these conditions in a single country.
Methods: This population-based study was performed in the Republic of Ireland (RoI), with a PR estimated for December 2013. Multiple case ascertainment sources were utilized. Demographic and clinical information and relevant diagnostic results were registered.
Results: A total of 2,641 adults were identified, giving a PR of 62.6/100,000 (95% confidence interval [CI] 59.95-65.24) for all NMD in RoI. Disease-specific PR include chronic inflammatory demyelinating polyradiculoneuropathy 5.87/100,000 (95% CI 5.06-6.68), Charcot-Marie-Tooth 10.52/100,000 (95% CI 9.44-11.61), hereditary neuropathy with liability to pressure palsies 0.84/100,000 (95% CI 0.54-1.15), myotonic dystrophy type I 6.75/100,000 (95% CI 5.88-7.61), Duchenne muscular dystrophy 3.0/100,000 (95% CI 2.33-3.70), Becker muscular dystrophy 2.2/100,000 (95% CI 1.64-2.88), facioscapulohumeral dystrophy 2.59/100,000 (95% CI 2.05-3.13), limb-girdle muscular dystrophy 2.88/100,000 (95% CI 2.31-3.45), periodic paralysis 1.72/100,000 (95% CI 1.28-2.15), myotonia congenita 0.32/100,000 (95% CI 0.18-0.56), paramyotonia congenita 0.15/100,000 (95% CI 0.06-0.34), Kennedy disease 0.83/100,000 (95% CI 0.40-1.27), Lambert-Eaton myasthenic syndrome 0.29/100,000 (95% CI 0.11-0.47), myasthenia gravis 15.12/100,000 (95% CI 13.82-16.42), and sporadic inclusion body myositis 11.7/100,000 (95% CI 9.82-13.58). PR for amyotrophic lateral sclerosis was established from an existing Register as 7.20/100,000 (95% CI 6.34-8.15).
Conclusions: The PR of all adult NMD in RoI is relatively high when compared with other chronic neurologic disorders, although some figures may be an underestimate of the true prevalence. The data provide a framework for international comparison and service planning.
© 2016 American Academy of Neurology.
Similar articles
-
The prevalence of hereditary neuromuscular disorders in Northern Norway.Brain Behav. 2021 Jan;11(1):e01948. doi: 10.1002/brb3.1948. Epub 2020 Nov 13. Brain Behav. 2021. PMID: 33185984 Free PMC article.
-
Methodology and design of a national epidemiological study on adult neuromuscular disease.Neuroepidemiology. 2014;43(2):123-30. doi: 10.1159/000367634. Epub 2014 Nov 5. Neuroepidemiology. 2014. PMID: 25376451
-
What we do not know about pregnancy in hereditary neuromuscular disorders.Neuromuscul Disord. 2009 Oct;19(10):675-9. doi: 10.1016/j.nmd.2009.07.004. Epub 2009 Aug 18. Neuromuscul Disord. 2009. PMID: 19692244 Review.
-
Prevalence and incidence of neuromuscular conditions in the UK between 2000 and 2019: A retrospective study using primary care data.PLoS One. 2021 Dec 31;16(12):e0261983. doi: 10.1371/journal.pone.0261983. eCollection 2021. PLoS One. 2021. PMID: 34972157 Free PMC article.
-
Population frequencies of inherited neuromuscular diseases--a world survey.Neuromuscul Disord. 1991;1(1):19-29. doi: 10.1016/0960-8966(91)90039-u. Neuromuscul Disord. 1991. PMID: 1822774 Review.
Cited by
-
A Nationwide, Population-Based Prevalence Study of Genetic Muscle Disorders.Neuroepidemiology. 2019;52(3-4):128-135. doi: 10.1159/000494115. Epub 2019 Jan 18. Neuroepidemiology. 2019. PMID: 30661069 Free PMC article.
-
The prevalence of hereditary neuromuscular disorders in Northern Norway.Brain Behav. 2021 Jan;11(1):e01948. doi: 10.1002/brb3.1948. Epub 2020 Nov 13. Brain Behav. 2021. PMID: 33185984 Free PMC article.
-
Corrigendum: A Systematic Review and Meta-Analysis of the Prevalence of Congenital Myopathy.Front Neurol. 2022 Feb 14;13:857959. doi: 10.3389/fneur.2022.857959. eCollection 2022. Front Neurol. 2022. PMID: 35237233 Free PMC article.
-
Dystrophin Exon 29 Nonsense Mutations Cause a Variably Mild Phenotype.Ulster Med J. 2017 Sep;86(3):185-188. Epub 2017 Sep 12. Ulster Med J. 2017. PMID: 29581631 Free PMC article.
-
Utilizing two surgeons for neuromuscular scoliosis suggests improved operative efficiency.Spine Deform. 2023 Jul;11(4):985-992. doi: 10.1007/s43390-023-00678-x. Epub 2023 Apr 17. Spine Deform. 2023. PMID: 37067777
MeSH terms
LinkOut - more resources
Full Text Sources
Other Literature Sources
Medical
Research Materials