title: Long-term follow-up in infantile-onset lambert-eaton myasthenic syndrome.
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.
authors.affiliation: Department of Neurosciences, University of Messina, Italy Department of Clinical and Experimental Medicine, University of Messina, Italy IRCCS Centro Neurolesi "Bonino-Pulejo," Messina, Italy.
authors.forename: S
authors.initials: S
authors.lastname: Portaro
authors.affiliation: Department of Neurosciences, University of Messina, Italy.
authors.forename: D
authors.initials: D
authors.lastname: Parisi
authors.affiliation: Institute of Neurological Sciences, National Research Council, Catania, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Polizzi
authors.affiliation: Department of Educational Sciences, University of Catania, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Ruggieri
authors.affiliation: Department of Neurosciences, "C. Besta" Institute, Milan, Italy.
authors.forename: F
authors.initials: F
authors.lastname: Andreetta
authors.affiliation: Department of Neurosciences, "C. Besta" Institute, Milan, Italy.
authors.forename: P
authors.initials: P
authors.lastname: Bernasconi
authors.affiliation: Department of Neurosciences, University of Messina, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Toscano
authors.affiliation: Department of Neurosciences, University of Messina, Italy crodolico@unime.it.
authors.forename: C
authors.initials: C
authors.lastname: Rodolico
mesh_terms: D000328:Adult; D017668:Age of Onset; D002648:Child; D005500:Follow-Up Studies; D006801:Humans; D015624:Lambert-Eaton Myasthenic Syndrome / Q000188:drug therapy / Q000503:physiopathology*; D008297:Male; D018482:Muscle, Skeletal / Q000503:physiopathology; D013997:Time Factors
keywords: 3,4-diaminopyridine phosphate; Lambert-Eaton syndrome; anti-voltage-gated calcium channel antibodies; proximal muscle weakness