Acquired Presynaptic Neuromuscular Junction Disorders: Infant Botulism and Lambert-Eaton Myasthenic Syndrome

Ai Sakonju, Thomas O. Crawford

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Two rare treatable acquired disorders of presynaptic neuromuscular junction are described. In infantile botulism, Clostridium botulinum toxin produced from intestinal colonization blocks docking proteins from releasing acetylcholine. Patients display bulbar signs and constipation, abnormal high-frequency repetitive nerve stimulation and positive stool toxin. However, treatment within 3 days of hospitalization with botulinum immunoglobulin results in reduced length of hospital stay by 3 weeks and ventilation by 2 weeks.Pediatric Lambert-Eaton Myasthenic Syndrome (LEMS) is a primary autoimmune disorder but less frequently paraneoplastic in less than half of patients. LEMS typically presents with leg weakness and areflexia. Supportive of LEMS diagnosis is a low amplitude compound muscle action potential facilitating >25% with 10-50. Hz stimulation or exercise. Patients respond well to 3,4-diaminopyridine as well as IVIg and immunosuppressive therapies.Although these are rare disorders, a high index of suspicion will lead to timely therapies and significant improvement in outcomes, and therefore they are well worth understanding.

Original languageEnglish (US)
Title of host publicationNeuromuscular Disorders of Infancy, Childhood, and Adolescence
Subtitle of host publicationA Clinician's Approach
PublisherElsevier Inc.
Pages445-455
Number of pages11
ISBN (Electronic)9780124171275
ISBN (Print)9780124170445
DOIs
StatePublished - Jan 1 2015

Keywords

  • Infantile botulism
  • LEMS
  • Lambert-Eaton myasthenic syndrome
  • Myasthenia
  • Myopathy
  • Neuromuscular transmission disorders

ASJC Scopus subject areas

  • Medicine(all)
  • Neuroscience(all)

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