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. 2021 Jan;11(1):e01948.
doi: 10.1002/brb3.1948. Epub 2020 Nov 13.

The prevalence of hereditary neuromuscular disorders in Northern Norway

Kai Ivar Müller  1   2 , Marijke Van Ghelue  2   3 , Irene Lund  1 , Christoffer Jonsrud  3 , Kjell Arne Arntzen  1   2
Affiliations

The prevalence of hereditary neuromuscular disorders in Northern Norway

Kai Ivar Müller et al. Brain Behav. 2021 Jan.

Abstract

Aim: To investigate the point prevalence of hereditary neuromuscular disorders on January 1, 2020 in Northern Norway.

Methods: From January 1, 1999, until January 1, 2020, we screened medical and genetic hospital records in Northern Norway for hereditary neuromuscular disorders.

Results: We identified 542 patients with a hereditary neuromuscular disorder living in Northern Norway, giving a point prevalence of 111.9/100,000 on January 1, 2020. The prevalence of children (<18 years old) and adults (≥18 years old) were 57.8/100,000 and 125.1/100,000, respectively. Inherited neuropathies had a prevalence of 38.8/100,000. Charcot-Marie-Tooth and hereditary neuropathy with liability to pressure palsies had a prevalence of 29.9/100,000 and 8.3/100,000, respectively. We calculated a prevalence of 3.7/100,000 for spinal muscular atrophies and 2.4/100,000 for Kennedy disease. Inherited myopathies were found in 67.7/100,000. Among these, we registered 13.4/100,000 myotonic dystrophy type 1, 6.8/100,000 myotonic dystrophy type 2, 7.3/100,000 Duchenne muscular dystrophy, 1.6/100,000 Becker muscular dystrophy, 3.7/100,000 facioscapulohumeral muscular dystrophy, 12.8/100,000 limb-girdle muscular dystrophy, 2.5/100,000 hypokalemic periodic paralysis and 11.4/100,000 myotonia congenita.

Conclusion: Our total prevalence was higher than previously hypothesized in European population-based studies. The prevalence was especially high for myotonia congenita and limb-girdle muscular dystrophy. The prevalence of Charcot-Marie-Tooth polyneuropathy was higher than in most European studies, but lower than previously reported in epidemiological studies in other regions of Norway.

Keywords: epidemiology; hereditary; neuromuscular; prevalence.

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Conflict of interest statement

The authors declare that they have no conflict of interest.

Figures

FIGURE 1
FIGURE 1
Northern Norway in red color with the two Departments of Neurology in Northern Norway. The departments are located at 69°N in Tromsø city, and at 67°N in Bodø city
See this image and copyright information in PMC

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