Disease Directory Multi-Minicore Disease
Neuromuscular

Multi-Minicore Disease

Also known as: MmD, minicore myopathy, multi-minicore myopathy, SELENON-related myopathy

Prevalence

Less than 1 in 100,000

Onset

Congenital or early childhood

Type

Autosomal recessive

Gene

SELENON, RYR1

About Multi-Minicore Disease

Multi-Minicore Disease is a congenital myopathy characterised by multiple small areas of reduced oxidative enzyme activity (minicores) on muscle biopsy, caused most commonly by bi-allelic mutations in SELENON (formerly SEPN1) or RYR1. The SELENON form has a distinctive phenotype of rigid spine, early respiratory failure disproportionate to limb weakness, and scoliosis; the RYR1 form is more variable and may overlap with Central Core Disease. Respiratory failure is the major cause of morbidity.

Common Clinical Features

Neonatal or infantile hypotonia Rigid spine syndrome (limited neck and spine flexion) Respiratory insufficiency disproportionate to limb weakness Scoliosis Proximal limb weakness Feeding difficulties in infancy Malignant hyperthermia susceptibility (RYR1 form)

Clinical Trial Eligibility Tips

What to know before applying to Multi-Minicore Disease trials.

Genetic subtype (SELENON vs RYR1) is required as trials target distinct mechanisms; bi-allelic mutation confirmation via sequencing is standard

Respiratory assessments including upright and supine FVC, overnight oximetry, and sleep study are primary eligibility criteria given the prominence of respiratory involvement

MRI patterns of muscle involvement differ between SELENON and RYR1 forms and are increasingly used as imaging biomarkers — a whole-body muscle MRI before trial application is highly beneficial

Patient Resources

Patient Organization

Cure CMD

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Natural History Registry

CMDIR

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Orphanet

European reference resource for rare diseases (ORPHA:178145)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

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Find recruiting Multi-Minicore Disease trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Multi-Minicore Disease. Updated daily.

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