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Related Concept Videos

Myasthenia Gravis: Overview and Treatment01:20

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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.
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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.
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Disorders of the Skeletal Muscle01:28

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The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
Musculoskeletal disorders
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Accessory Structures of the Eye01:17

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Optical perception, or vision, is an extraordinary sense dependent on converting light signals received via the ocular organs. These organs, known as eyes, are securely positioned within the bony cavities of the skull, called orbits. The orbits serve a dual purpose: a protective shield for the ocular globes and a stable attachment point for the soft ocular tissues. The eye's external protective mechanisms include the eyelids, which are edged with lashes that act as a barrier against foreign...
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The muscles of the eye are sophisticated structures that control eye movement and focus, allowing for the precise and rapid adjustments necessary for vision. The human eye is controlled by ten muscles — six extraocular muscles, three intraocular muscles, and one primary eyelid retractor muscle.
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Direct-acting cholinergic agonists have many therapeutic uses in various medical fields. Choline esters, including acetylcholine, have limited clinical utility due to their non-selectivity and short duration of action. Still, acetylcholine and carbachol are applied topically during ophthalmologic surgery to induce miosis. Pilocarpine, a muscarinic and ganglionic stimulator, effectively treats open-angle glaucoma and alleviates xerostomia and dry mouth caused by radiotherapy or Sjögren...
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[Pediatric myasthenia with ocular involvement].

L Prud'homme1, C Gitiaux2, C Barnerias3

  • 1Département d'ophtalmologie 2, hôpital national d'ophtalmologie des Quinze-Vingts, Paris, France; Service d'ophtalmologie, Paris-Cité University, hôpital universitaire Necker-Enfants-Malades, AP-HP, Paris, France.

Journal Francais D'Ophtalmologie
|May 9, 2024
PubMed
Summary

Pediatric myasthenia, a rare condition, often presents with ptosis and strabismus. Early diagnosis and specialized care are crucial for managing this challenging diagnosis in children.

Keywords:
MyastheniaMyasthéniePediatricsPtosisPédiatrieStrabismeStrabismus

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Area of Science:

  • Ophthalmology
  • Neurology
  • Pediatrics

Context:

  • Myasthenia is a rare neuromuscular disorder affecting children, with significant potential for severe outcomes.
  • Early diagnosis by pediatric ophthalmologists is critical due to the availability of specific treatments.

Purpose:

  • To review cases of pediatric myasthenia with ocular symptoms.
  • To understand the diagnostic challenges and clinical presentations of myasthenia in children.

Summary:

  • A retrospective review included 26 children with myasthenia and ocular symptoms.
  • Ptosis was universal; strabismus occurred in 68%. Autoimmune myasthenia gravis and congenital myasthenic syndrome were the main diagnoses.
  • Congenital forms were proportionately higher in children than adults.

Impact:

  • Highlights the ophthalmologic manifestations of pediatric myasthenia, primarily ptosis and strabismus.
  • Emphasizes diagnostic difficulties due to non-specific signs and challenges in pediatric testing.
  • Recommends specialized centers for diagnosis and management of pediatric myasthenia.