Lambert-Eaton myasthenic syndrome in children.
Lambert-Eaton myasthenic syndrome is a presynaptic disorder of neuromuscular transmission. It is characterized by muscle weakness, hyporeflexia, and autonomic dysfunction. It is most often associated with small cell carcinomas of the lung. Rare cases have been reported in children. We recently encountered two children with Lambert-Eaton myasthenic syndrome associated with antibodies to P/Q-type calcium channel but without evidence of neoplasms. Both patients showed prolonged and significant improvement following cyclosporin treatment. The diagnosis of Lambert-Eaton myasthenic syndrome should be considered in children with progressive weakness and a negative work-up for the usual causes. High-frequency repetitive nerve stimulation and P/Q-type calcium-channel antibodies may confirm the diagnosis.
Tsao
Chang-Yong
CY
Department of Pediatrics and Neurology, College of Medicine and Public Health, The Ohio State University, USA. ctsao@chi.osu.edu
Mendell
Jerry R
JR
Friemer
Miriam L
ML
Kissel
John T
JT
D001323:Autoantibodies / Q000097:blood; D020865:Calcium Channels, P-Type / Q000276:immunology; D020907:Calcium Channels, Q-Type / Q000276:immunology; D002648:Child; D016572:Cyclosporine / Q000627:therapeutic use; D004558:Electric Stimulation; D005260:Female; D006801:Humans; D015624:Lambert-Eaton Myasthenic Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D008297:Male; D008475:Median Nerve / Q000503:physiopathology; D009460:Neurologic Examination; D009435:Synaptic Transmission / Q000187:drug effects / Q000502:physiology; D014459:Ulnar Nerve / Q000503:physiopathology
D001323:Autoantibodies; D020865:Calcium Channels, P-Type; D020907:Calcium Channels, Q-Type; D016572:Cyclosporine