Juvenile myasthenia gravis in Norway: A nationwide epidemiological study

T H Popperud1, M I Boldingh2, C Brunborg3

  • 1Department of Neurology, Oslo University Hospital, Oslo, Norway; Institute of Clinical Medicine, University of Oslo, Oslo, Norway.

Abstract

Insights

Juvenile myasthenia gravis (JMG) is rare in Norway, with a stable incidence rate over 25 years. This study found JMG is more common in females than males.

Area of Science:

  • Neurology
  • Pediatrics
  • Epidemiology

Background:

  • Autoimmune myasthenia gravis (MG) can affect children, termed juvenile MG (JMG).
  • Understanding the epidemiology of JMG is crucial for healthcare planning.

Purpose of the Study:

  • To determine the incidence and prevalence of juvenile myasthenia gravis (JMG) in Norway.
  • To analyze trends in JMG occurrence over time.

Main Methods:

  • A retrospective, population-based study in Norway (2012-2013).
  • Identified JMG cases (<18 years) from hospital records (1989-2013), antibody databases, and MG registries.
  • Verified diagnoses and age at onset through medical records.

Main Results:

  • Identified 63 unique JMG cases, with an average annual incidence of 1.6 per million.
  • Prevalence ranged from 3.6 to 13.8 per million.
  • Females represented the majority (55 vs. 8 males), with higher JMG risk in both prepubertal and postpubertal females.

Conclusions:

  • Juvenile myasthenia gravis (JMG) is confirmed as a rare condition in Norway.
  • The incidence rate of JMG has remained stable over the past 25 years.
  • JMG is significantly less common in males compared to females.

Related Concept Videos

Myasthenia Gravis: Overview and Treatment01:20

Myasthenia Gravis: Overview and Treatment

Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which leads...
Myasthenia Gravis: Diagnostic Tests01:15

Myasthenia Gravis: Diagnostic Tests

Myasthenia gravis is an autoimmune condition affecting neuromuscular transmission, causing generalized weakness in skeletal muscles. Initial diagnoses rely on patients' signs, symptoms, and medical history. The challenge lies in distinguishing myasthenia from other muscular dystrophies. An important diagnostic feature is the significant improvement of symptoms after administering anticholinesterase inhibitors.
The edrophonium test is a diagnostic tool for myasthenia gravis. It involves...
Disorders of the Skeletal Muscle01:28

Disorders of the Skeletal Muscle

The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
Musculoskeletal disorders
Musculoskeletal disorders involve injuries and conditions affecting the skeletal muscles and associated connective tissues. These disorders can arise from acute biomechanical stresses or chronic overuse and can occur across different age groups. Common injuries include sprains, fractures, and muscular strains, often resulting from...
Chronic Obstructive Pulmonary Disease-IV: Assessement and Diagnostic Studies01:27

Chronic Obstructive Pulmonary Disease-IV: Assessement and Diagnostic Studies

Assessing and diagnosing Chronic Obstructive Pulmonary Disease (COPD) involves a detailed approach that includes a comprehensive review of medical history, physical examination, and a variety of diagnostic tests. This thorough evaluation is essential to ensure an accurate diagnosis and guide effective management strategies.
Medical History
Myasthenia Gravis ll: Pathophysiology01:22

Myasthenia Gravis ll: Pathophysiology

The disease process of myasthenia gravis begins at the neuromuscular junction, where antibodies attack key proteins needed for muscle activation. This immune reaction weakens signal transmission, leading to the characteristic muscle fatigue and weakness that define the condition.Immune-Mediated DamageIn most individuals, antibodies target acetylcholine receptors (AChRs) on the postsynaptic membrane of muscle cells. By blocking acetylcholine binding, these antibodies prevent the nerve signal...
Asthma III: Clinical Manifestations01:13

Asthma III: Clinical Manifestations

Asthma presents with a characteristic pattern of episodic respiratory symptoms that reflect underlying airway inflammation, bronchoconstriction, and mucus hypersecretion. Although severity varies among individuals, certain clinical manifestations are considered hallmarks of the disorder and often guide diagnosis and assessment.Respiratory SymptomsA persistent cough is one of the most common early features of asthma. It is frequently dry and tends to worsen at night or in the early morning,...