283rd ENMC international workshop: Establishing expert care recommendations for LAMA2-RD: A prototype for the development of congenital muscular dystrophy subtype-specific care guidelines. Hoofddorp, The Netherlands, January 17th-19th 2025.

LAMA2-related dystrophies (LAMA2-RDs) are among the most frequent congenital muscular dystrophies, caused by pathogenic variants in the LAMA2 gene. They typically present in infancy with severe muscle weakness but span a wide clinical spectrum, from neonatal onset to milder, later-onset forms. Complications include respiratory insufficiency, nutritional difficulties, joint contractures, scoliosis, and central nervous system involvement. No specific internationally agreed standards of care (SoC) exist, leading to variability in diagnosis, monitoring, and access to multidisciplinary management and care. The 283rd ENMC International Workshop gathered international stakeholders to review current evidence, suggest expert care recommendations and reach an agreement on the best methods to develop diagnostic and care guidelines for LAMA2-RD. Key outcomes included consensus care recommendations across major clinical domains, strategies for dissemination through a dynamic, open-access resource, and a framework to guide development of SoC for other genetic forms of congenital muscular dystrophies.

Publication Details

Journal
Open Access CRIS of the University of Bern
Published
2025-10-01
DOI
https://doi.org/10.48620/92039
Primary Topic
Muscle Physiology and Disorders
Type
article
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article

283rd ENMC international workshop: Establishing expert care recommendations for LAMA2-RD: A prototype for the development of congenital muscular dystrophy subtype-specific care guidelines. Hoofddorp, The Netherlands, January 17th-19th 2025.

Open Access CRIS of the University of Bern
Muscle Physiology and Disorders
article

283rd ENMC international workshop: Establishing expert care recommendations for LAMA2-RD: A prototype for the development of congenital muscular dystrophy subtype-specific care guidelines. Hoofddorp, The Netherlands, January 17th-19th 2025.

article en

Abstract

LAMA2-related dystrophies (LAMA2-RDs) are among the most frequent congenital muscular dystrophies, caused by pathogenic variants in the LAMA2 gene. They typically present in infancy with severe muscle weakness but span a wide clinical spectrum, from neonatal onset to milder, later-onset forms. Complications include respiratory insufficiency, nutritional difficulties, joint contractures, scoliosis, and central nervous system involvement. No specific internationally agreed standards of care (SoC) exist, leading to variability in diagnosis, monitoring, and access to multidisciplinary management and care. The 283rd ENMC International Workshop gathered international stakeholders to review current evidence, suggest expert care recommendations and reach an agreement on the best methods to develop diagnostic and care guidelines for LAMA2-RD. Key outcomes included consensus care recommendations across major clinical domains, strategies for dissemination through a dynamic, open-access resource, and a framework to guide development of SoC for other genetic forms of congenital muscular dystrophies.

Open Access CRIS of the University of Bern
Openalex Percentile: Top 44%
Muscle Physiology and Disorders
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283rd ENMC international workshop: Establishing expert care recommendations for LAMA2-RD: A prototype for the development of congenital muscular dystrophy subtype-specific care guidelines. Hoofddorp, The Netherlands, January 17th-19th 2025. · Open Access CRIS of the University of Bern (2025) | TGRS Research Map | TGRS