doi:10.1016/j.ejpn.2008.03.006 
Copyright © 2008 European Paediatric Neurology Society Published by Elsevier Ltd.
Case study
Lambert–Eaton myasthenic syndrome in childhood
A. Kostera-Pruszczyka, 
, 
, B. Ryniewicza, K. Rowinska-Marcinskab, M. Dutkiewiczc and A. Kamińskaa, d
aDepartment of Neurology, Medical University of Warsaw, Poland
bDepartment of Neurology, Faculty of Health Science, Medical University of Warsaw, Poland
cDepartment of Internal Medicine, Hypertension and Angiology, Medical University of Warsaw, Poland
dNeuromuscular Unit, Polish Academy of Science, Warsaw, Poland
Received 31 July 2007;
revised 18 March 2008;
accepted 30 March 2008.
Available online 30 June 2008.
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Abstract
Lambert–Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder of the neuromuscular junction. LEMS can be associated with a variety of neoplasms. Patients present with proximal muscle weakness and fatigability, often combined with areflexia. Only 5% of reported cases are children. We report a case of 11-year old boy with non-neoplastic Lambert–Eaton myasthenic syndrome. Repetitive nerve stimulation test showed 83% increment after maximal voluntary contraction, presence of antibodies against voltage-gated calcium channels confirmed the diagnosis. The boy responded well to immunosuppressive treatment with prednisone and azathioprine and remains cancer-free for 4 years.
Keywords: Voltage-gated calcium channel antibodies; Neuromuscular; Children; Repetitive nerve stimulation; Lambert–Eaton myasthenic syndrome; Myasthenia gravis
Fig. 1. Repetitive nerve stimulation test of the ulnar nerve, recording from abductor digiti minimi muscle. At diagnosis (A) 45% decrement at 3 Hz; and (B) 82% increase of CMAP amplitude immediately after 30-s maximal voluntary contraction (postexercise facilitation).
Table 1.
Repetitive nerve stimulation test results
* At diagnosis, ** a year later, *** at last examination 3 years after diagnosis
ADM – abductor digiti minimi muscle, APB – abductor pollicis brevis muscle, EDB – extensor digitorum brevis muscle.
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