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Review
. 2018 May;36(2):379-394.
doi: 10.1016/j.ncl.2018.01.008.

Lambert-Eaton Myasthenic Syndrome

Vita G Kesner  1 , Shin J Oh  2 , Mazen M Dimachkie  3 , Richard J Barohn  3
Affiliations
Review

Lambert-Eaton Myasthenic Syndrome

Vita G Kesner et al. Neurol Clin. 2018 May.

Abstract

Lambert-Eaton myasthenic syndrome is a paraneoplastic or primary autoimmune neuromuscular junction disorder characterized by proximal weakness, autonomic dysfunction and ariflexia. The characteristic symptoms are thought to be caused by antibodies generated against the P/Q-type voltage-gated calcium channels present on presynaptic nerve terminals and by diminished release of acetylcholine. More than half of Lambert-Eaton myasthenic syndrome cases are associated with small cell lung carcinoma. Diagnosis is confirmed by serologic testing and electrophysiologic studies. 3,4-diaminopyridine is effective symptomatic treatment of LEMS.

Keywords: 3,4-Diaminopyridine; Lambert-Eaton myasthenic syndrome; Neuromuscular transmission disorder; P/Q-type voltage-gated calcium channels; Paraneoplastic syndrome.

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Figures

Fig. 1.
Fig. 1.
Spreading of symptoms in patients with NT-LEMS and SCLC-LEMS. Frequency of symptoms at 3 months (A) and 12 months (B) in patients with NT-LEMS, and frequency of symptoms at 3 months (C) and 12 months (D) in patients with SCLC-LEMS. The percentages describe the approximate proportion of patients who have that symptom within the given timeframe. (From Titulaer MJ, Lang B, Verschuuren JJ. Lambert-Eaton myasthenic syndrome: from clinical characteristics to therapeutic strategies. Lancet Neurol 2011;10(12):1099; Reprinted with permission from Elsevier (The Lancet).)
Fig. 2.
Fig. 2.
Typical repetitive nerve stimulation pattern in the abductor digiti minimi muscle in LEMS. (A) Low compound action potential amplitude at rest, marked incremental response (+400 mg) after 30 seconds of exercise. (B) Decremental response (−46%) at low-rate stimulation (3 Hz). (C) Marked incremental response (+400%) at high-rate stimulation (50 Hz). (From Oh SJ. Treatment and management of the neuromuscular junction. In: Bertorini TE, editor. Neuromuscular disorders. Treatment and management. Philadelphia: Elsevier-Saunders; 2011. p. 323; with permission.)
Fig. 3.
Fig. 3.
Flowchart of recommended screening for SCLC in patients with LEMS. CT, computed tomography; FDG, F-fluorodeoxyglucose. (From Titulaer MJ, Lang B, Verschuuren JJ. Lambert-Eaton myasthenic syndrome: from clinical characteristics to therapeutic strategies. Lancet Neurol 2011;10(12):1103; Reprinted with permission from Elsevier (The Lancet).)
Fig. 4.
Fig. 4.
Treatment scheme for LEMS. (From Titulaer MJ, Lang B, Verschuuren JJ. Lambert-Eaton myasthenic syndrome: from clinical characteristics to therapeutic strategies. Lancet Neurol 2011;10(12):1104; Reprinted with permission from Elsevier (The Lancet).)
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References

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