Myasthenia gravis Lambert‐Eaton overlap syndrome

Oh, Shin J.

doi:10.1002/mus.24921

Cited by 29 publications

(31 citation statements)

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“…The occurrence of MG in LEMS patients is a widely accepted phenomenon [191]. Although diagnosis of MG in LEMS individuals has mainly been based on clinical presentation, tests for serum autoantibodies against AChR and VGCC revealed a number of double seropositive cases [192–194].…”

Section: Potential Mechanisms Of Double Autoantibody Positivitymentioning

confidence: 99%

Mapping autoantigen epitopes: molecular insights into autoantibody-associated disorders of the nervous system

Sinmaz

Nguyen

Charabati

et al. 2016

J Neuroinflammation

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BackgroundOur knowledge of autoantibody-associated diseases of the central (CNS) and peripheral (PNS) nervous systems has expanded greatly over the recent years. A number of extracellular and intracellular autoantigens have been identified, and there is no doubt that this field will continue to expand as more autoantigens are discovered as a result of improved clinical awareness and methodological practice. In recent years, interest has shifted to uncover the target epitopes of these autoantibodies.Main bodyThe purpose of this review is to discuss the mapping of the epitope targets of autoantibodies in CNS and PNS antibody-mediated disorders, such as N-methyl-D-aspartate receptor (NMDAR), α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), leucine-rich glioma-inactivated protein 1 (Lgi1), contactin-associated protein-like 2 (Caspr2), myelin oligodendrocyte glycoprotein (MOG), aquaporin-4 (AQP4), 65 kDa glutamic acid decarboxylase (GAD65), acetylcholine receptor (AChR), muscle-specific kinase (MuSK), voltage-gated calcium channel (VGCC), neurofascin (NF), and contactin. We also address the methods used to analyze these epitopes, the relevance of their determination, and how this knowledge can inform studies on autoantibody pathogenicity. Furthermore, we discuss triggers of autoimmunity, such as molecular mimicry, ectopic antigen expression, epitope spreading, and potential mechanisms for the rising number of double autoantibody-positive patients.ConclusionsMolecular insights into specificity and role of autoantibodies will likely improve diagnosis and treatment of CNS and PNS neuroimmune diseases.

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Section: Potential Mechanisms Of Double Autoantibody Positivitymentioning

confidence: 99%

Mapping autoantigen epitopes: molecular insights into autoantibody-associated disorders of the nervous system

Sinmaz

Nguyen

Charabati

et al. 2016

J Neuroinflammation

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“…However, LEMS cases with isolated anti-VGCC N-type antibodies are rare. 15 Recently a similar case of dropped head syndrome with positive anti-VGCC N-type antibodies was published, suggesting an anti-VGCC N-type mediated, non-paraneoplastic LEMS. 20 In this patient neither SCLC nor anti-VGCC P/Q-type antibodies could be detected and the patient improved under immunosuppressive and symptomatic treatment.…”

Section: Discussionmentioning

confidence: 99%

“…15,18-21 These cases were characterized by ocula-bulbar symptoms, a response to edrophonium, hyporeflexia and an increment of reduced CMAP in NCS. Oh and colleagues presented a case of a young women with myasthenic symptoms as well as proximal muscle weakness, elevated anti-VGCC N-type antibodies and response to intravenous immunoglobulins.…”

Section: Discussionmentioning

confidence: 99%

“…While in LEMS 98% of the patient has decreased or absent reflexes, in overlap syndromes this was only documented in 74% of the patients. 15 Therefore, normal reflexes do not exclude the diagnosis of a potential MLOS. The detection of anti-VGCC N-type antibodies is often accompanied by a cancer disease, typically a small cell lung cancer (SCLC).…”

Section: Discussionmentioning

confidence: 99%

“…7,13,14 Recently the existence of a myasthenia gravis Lambert-Eaton overlap syndrome (MLOS) was reported. 15 …”

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confidence: 99%

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Clinical features of neuromuscular disorders in patients with N-type voltage-gated calcium channel antibodies

et al. 2016

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Neuromuscular junction disorders affect the pre- or postsynaptic nerve to muscle transmission due to autoimmune antibodies. Members of the group like myasthenia gravis and Lambert-Eaton syndrome have pathophysiologically distinct characteristics. However, in practice, distinction may be difficult. We present a series of three patients with a myasthenic syndrome, dropped-head syndrome, bulbar and respiratory muscle weakness and positive testing for anti-N-type voltage-gated calcium channel antibodies. In two cases anti-acetylcholin receptor antibodies were elevated, anti-P/Q-type voltage-gated calcium channel antibodies were negative. All patients initially responded to pyridostigmine with a non-response in the course of the disease. While one patient recovered well after treatment with intravenous immunoglobulins, 3,4-diaminopyridine, steroids and later on immunosuppression with mycophenolate mofetil, a second died after restriction of treatment due to unfavorable cancer diagnosis, the third patient declined treatment. Although new antibodies causing neuromuscular disorders were discovered, clinical distinction has not yet been made. Our patients showed features of pre- and postsynaptic myasthenic syndrome as well as severe dropped-head syndrome and bulbar and axial muscle weakness, but only anti-N-type voltage-gated calcium channel antibodies were positive. When administered, one patient benefited from 3,4-diaminopyridine. We suggest that this overlap-syndrome should be considered especially in patients with assumed seronegative myasthenia gravis and lack of improvement under standard therapy.

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