Lambert-Eaton myasthenic syndrome revealing an abdominal neuroblastoma
Section snippets
Case report
A 13-month-old adolescent girl was brought by her parents to the emergency ward for abdominal pain after a long period of constipation and for right ocular paresis and somnolence present for about 1 week according to the parents. The girl slept all through the physical examination. It was difficult to wake her up during the day. She refused to stand straight and experienced an extreme weakness of the legs. She did not react during venous puncture. Her head was oriented toward the right
Discussion
Early symptoms of the presence of a neuroblastoma are usually weight loss, weakness, or fever. The clinical presentation depends on the location of the tumor. In the abdomen, the mass is retroperitoneal and is most often detected in the course of routine clinical examinations. A pelvic mass may provoke urinary retention or constipation. In the thorax, the tumor is most often discovered on chest radiographs. Occasionally, the tumor is revealed by symptoms because of the compression of the
Acknowledgment
The authors are grateful to Annette Wagnière for reviewing the English text.
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Lambert-Eaton myasthenic syndrome in a 13-year-old girl with Xp11.22-p11.23 duplication
2014, European Journal of Paediatric NeurologyCitation Excerpt :Approximately 5% of LEMS-patients are children.5 In some of these, an association with neuroblastoma is seen6,7 but most childhood cases are non-paraneoplastic.3 Little is known about associations with other systemic auto-immune diseases or genetic factors that contribute to the susceptibility of an individual to develop NP-LEMS.8–10
Lambert-eaton syndrome, an unrecognized treatable pediatric neuromuscular disorder: Three patients and literature review
2014, Pediatric NeurologyCitation Excerpt :Three patients had malignancies: leukemia,10 Burkitt's lymphoma,5 and neuroblastoma.11 Three toddlers (at ages 1, 2, and 3 years) were reported to have LEMS associated with an underlying neuroblastoma12,13; however, none fulfilled standard diagnostic criteria for LEMS,14 including CMAP amplitude facilitation or voltage-gated calcium channel antibody testing and therefore were not included in this review. Voltage-gated calcium channel antibodies were elevated in five of six (83%) children tested.2,8,9,11
Narcolepsy with cataplexy as presenting symptom of occult neuroblastoma
2013, Pediatric NeurologyCitation Excerpt :It is usually associated with localized tumors, characterized by a heavy lymphocyte infiltrate, and a good oncological prognosis. The much rarer, but also immunologically mediated Lambert-Eaton myasthenic syndrome [3], anti-Hu positive limbic encephalitis[4], and anti–NMDA-positive encephalitis[5] are also described in neuroblastoma. Narcolepsy with cataplexy has been reported in children with neuroblastoma only once before [6].
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