Acquired Presynaptic Neuromuscular Junction Disorders

Infant Botulism and Lambert-Eaton Myasthenic Syndrome

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: A Clinician's Approach
PublisherElsevier Inc.
Pages445-455
Number of pages11
ISBN (Print)9780124171275, 9780124170445
DOIs
StatePublished - Dec 3 2014

Fingerprint

Neuromuscular Junction Diseases
Lambert-Eaton Myasthenic Syndrome
Botulism
Length of Stay
Botulinum Toxins
Constipation
Immunosuppressive Agents
Action Potentials
Acetylcholine
Ventilation
Immunoglobulins
Leg
Hospitalization
Therapeutics
Exercise
Pediatrics
Muscles
Proteins

Keywords

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

ASJC Scopus subject areas

  • Medicine(all)
  • Neuroscience(all)

Cite this

Sakonju, A., & Crawford, T. O. (2014). Acquired Presynaptic Neuromuscular Junction Disorders: Infant Botulism and Lambert-Eaton Myasthenic Syndrome. In Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician's Approach (pp. 445-455). Elsevier Inc.. https://doi.org/10.1016/B978-0-12-417044-5.00025-1

Acquired Presynaptic Neuromuscular Junction Disorders : Infant Botulism and Lambert-Eaton Myasthenic Syndrome. / Sakonju, Ai; Crawford, Thomas Owen.

Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician's Approach. Elsevier Inc., 2014. p. 445-455.

Research output: Chapter in Book/Report/Conference proceedingChapter

Sakonju, A & Crawford, TO 2014, Acquired Presynaptic Neuromuscular Junction Disorders: Infant Botulism and Lambert-Eaton Myasthenic Syndrome. in Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician's Approach. Elsevier Inc., pp. 445-455. https://doi.org/10.1016/B978-0-12-417044-5.00025-1
Sakonju A, Crawford TO. Acquired Presynaptic Neuromuscular Junction Disorders: Infant Botulism and Lambert-Eaton Myasthenic Syndrome. In Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician's Approach. Elsevier Inc. 2014. p. 445-455 https://doi.org/10.1016/B978-0-12-417044-5.00025-1
Sakonju, Ai ; Crawford, Thomas Owen. / Acquired Presynaptic Neuromuscular Junction Disorders : Infant Botulism and Lambert-Eaton Myasthenic Syndrome. Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician's Approach. Elsevier Inc., 2014. pp. 445-455
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