Lambert-Eaton Myasthenia Syndrome

Author(s):  
Aaron E. Miller ◽  
Teresa M. DeAngelis

Lambert-Eaton myasthenia syndrome (LEMS) is an autoimmune-mediated disorder of the neuromuscular junction, which involves autoantibodies to voltage-gated calcium channels on the presynaptic membrane. In this chapter, we discuss the characteristic clinical features of LEMS, its electrophysiological distinction from MG, as well as its paraneoplastic presentation. We also review the immunotherapeutic management options in cases with severe weakness and those refractory to tumor removal.

2021 ◽  
Vol 11 (8) ◽  
pp. 1035
Author(s):  
Maria Pia Giannoccaro ◽  
Patrizia Avoni ◽  
Rocco Liguori

The neuromuscular junction (NMJ) is the target of a variety of immune-mediated disorders, usually classified as presynaptic and postsynaptic, according to the site of the antigenic target and consequently of the neuromuscular transmission alteration. Although less common than the classical autoimmune postsynaptic myasthenia gravis, presynaptic disorders are important to recognize due to the frequent association with cancer. Lambert Eaton myasthenic syndrome is due to a presynaptic failure to release acetylcholine, caused by antibodies to the presynaptic voltage-gated calcium channels. Acquired neuromyotonia is a condition characterized by nerve hyperexcitability often due to the presence of antibodies against proteins associated with voltage-gated potassium channels. This review will focus on the recent developments in the autoimmune presynaptic disorders of the NMJ.


2011 ◽  
pp. 815-823 ◽  
Author(s):  
L. F. NURULLIN ◽  
A. R. MUKHITOV ◽  
A. N. TSENTSEVYTSKY ◽  
N. V. PETROVA ◽  
D. V. SAMIGULLIN ◽  
...  

It is well known that antagonists of N-type voltage-gated calcium channels inhibit the evoked quantal release of acetylcholine in amphibian neuromuscular synapses. This, however, does not exclude the functional expression of other types of voltage-gated calcium channels in these nerve terminals. Using immunocytochemistry, we detected the expression of the 1A subunit of P/Q-type calcium channels (that is otherwise typical of mammalian motor nerve endings) in the frog neuromuscular junction. In addition, we demonstrated that the P/Q-type channel blocker ω-agatoxin IVA (20 nM) reduced the action potential-induced calcium transient and significantly decreased both spontaneous and evoked mediator release. Our data indicates the functional expression of P/Q-type calcium channels in the frog motor nerve ending which participate in acetylcholine release.


2019 ◽  
Vol 216 (5) ◽  
pp. 250-253 ◽  
Author(s):  
Paul J. Harrison ◽  
Elizabeth M. Tunbridge ◽  
Annette C. Dolphin ◽  
Jeremy Hall

SummaryWe reappraise the psychiatric potential of calcium channel blockers (CCBs). First, voltage-gated calcium channels are risk genes for several disorders. Second, use of CCBs is associated with altered psychiatric risks and outcomes. Third, research shows there is an opportunity for brain-selective CCBs, which are better suited to psychiatric indications.


2003 ◽  
Vol 117 (2) ◽  
pp. 109-115 ◽  
Author(s):  
Gary Chin ◽  
Yasir El-Sherif ◽  
Farah Jayman ◽  
Rima Estephan ◽  
Andrzej Wieraszko ◽  
...  

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