Long-term follow-up in infantile-onset Lambert-Eaton myasthenic syndrome

S. Portaro, D. Parisi, A. Polizzi, M. Ruggieri, F. Andreetta, P. Bernasconi, A. Toscano, C. Rodolico

Research output: Contribution to journalArticlepeer-review

Abstract

Lambert-Eaton myasthenic syndrome is a neuromuscular junction disorder characterized by proximal limb muscle weakness, fatigability, decreased deep-tendon reflexes, and autonomic symptoms. There are 2 forms of Lambert-Eaton myasthenic syndrome: one most frequently associated with small-cell lung cancer (P-Lambert-Eaton myasthenic syndrome) and the other that is a pure autoimmune form (NP-Lambert-Eaton myasthenic syndrome). Lambert-Eaton myasthenic syndrome is a very rare disorder in children younger than age 12 years. Herein, we report a 25-year-old man with NP-Lambert-Eaton myasthenic syndrome, which onset was at the age of 10 years. To date, this is the most long-term follow-up of NP-Lambert-Eaton myasthenic syndrome in childhood. In our patient, the only symptomatic treatment with 3,4-diaminopyridine phosphate has been sufficient to guarantee him a good quality of life. Our data remind physicians to keep in mind the diagnosis of Lambert-Eaton myasthenic syndrome in children with a proximal myopathic pattern and they confirm the specificity of compound muscle action potential incremental pattern after brief maximal effort in Lambert-Eaton myasthenic syndrome.

Original languageEnglish
Pages (from-to)NP58-NP61
JournalJournal of Child Neurology
Volume29
Issue number9
DOIs
Publication statusPublished - 2014

Keywords

  • 3 4-diaminopyridine phosphate
  • Anti-voltage-gated calcium channel antibodies
  • Lambert-Eaton syndrome
  • Proximal muscle weakness

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Medicine(all)

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