Abstract | BACKGROUND:
Lambert-Eaton myasthenic syndrome, a presynaptic neuromuscular junction autoimmune disorder, rarely occurs in children. Patients typically present with proximal lower extremity weakness with areflexia. METHODS: RESULTS: These patients were identified as having Lambert-Eaton myasthenic syndrome during their evaluation for proximal weakness. Low-amplitude compound muscle action potentials classically facilitating >100% with voluntary exercise and/or 50 Hz stimulation were essential to diagnosis. Three of the 12 children had associated malignancies, two of them had lymphoproliferative disorders with onset of symptoms more rapid than the rest, and the third had neuroblastoma. The nine nonparaneoplastic Lambert-Eaton myasthenic syndrome patients responded to immunomodulatory therapy with close return to their baseline function. Complete remission no longer necessitating medication was reported in two patients. Follow-up up to 17 years was available on two patients previously reported. CONCLUSION:
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Authors | Mirna Hajjar, Jennifer Markowitz, Basil T Darras, John T Kissel, Jayashri Srinivasan, H Royden Jones |
Journal | Pediatric neurology
(Pediatr Neurol)
Vol. 50
Issue 1
Pg. 11-7
(Jan 2014)
ISSN: 1873-5150 [Electronic] United States |
PMID | 24138947
(Publication Type: Case Reports, Journal Article, Research Support, N.I.H., Extramural, Review)
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Copyright | Copyright © 2014 Elsevier Inc. All rights reserved. |
Topics |
- Child
- Electromyography
- Female
- Follow-Up Studies
- Humans
- Lambert-Eaton Myasthenic Syndrome
(complications, diagnosis, therapy)
- Male
- Neuromuscular Diseases
(etiology)
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