Presynaptic Paraneoplastic Disorders of the Neuromuscular Junction: An Update

Giannoccaro, Maria Pia; Avoni, Patrizia; Liguori, Rocco

doi:10.3390/brainsci11081035

Cited by 7 publications

(8 citation statements)

References 154 publications

(226 reference statements)

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“…However, even though there was initial response towards IVIG, the long-term prognosis was variable. 7 This finding is similar to another case described by McKasson et al who presented with nonparaneoplastic progressive cerebellar ataxia and LEMS with elevated VGCC level. The patient reported initial significant improvement with IVIG, however later deteriorated and passed away 18 months later.…”

Section: Discussionsupporting

confidence: 90%

IVIG treatment in non-paraneoplastic Lambert- Eaton myasthenic syndrome with elevated voltage- gated calcium channels antibodies and subacute cerebellar ataxia

Chiew¹,

Ng²

2022

NeuroAsia

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Non paraneoplastic Lambert Eaton myasthenic syndrome (LEMS) is rare and only very few cases have been reported to date. Besides that, LEMS is rarely associated with cerebellar ataxia. Here, we described a case of middle age gentleman who presented with subacute onset of cerebellar ataxia and subsequently found to have remarkably elevated voltage-gated calcium channels (VGCC) antibodies serum titre and LEMS. Repeated screenings for malignancies remained negative. He improved significantly with intravenous immunoglobulin (IVIG) treatment. In conclusion, even with negative malignancy screen, investigation for LEMS and measurement of VGCC level should be considered in patients with subacute cerebellar ataxia. Our case demonstrated good initial response towards IVIG treatment and hence early and timely initiation of treatment with IVIG may lead to clinical improvement. However, more clinical trials will be required in the future to further determine the long term prognosis of non-paraneoplastic cerebellar ataxia and LEMS towards IVIG treatment.

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Section: Discussionsupporting

confidence: 90%

IVIG treatment in non-paraneoplastic Lambert- Eaton myasthenic syndrome with elevated voltage- gated calcium channels antibodies and subacute cerebellar ataxia

Chiew¹,

Ng²

2022

NeuroAsia

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“…LEMS is a presynaptic disorder characterized by the dysfunctional release of ACh vesicles at the neuromuscular junction due to the presence of antibodies to the pore-forming α1A subunit of the P/Q-type VGCC. This then impairs ACh vesicle release through a variety of mechanisms that affect the active zone of a neuromuscular junction: downregulation of presynaptic P/Q-type VGCCs, disorganization of vesicle release sites, and upregulation of alternative calcium channels 2,3 . Variable release of ACh results in failure of neuromuscular transmission, manifesting as weakness, autonomic dysfunction, or both.…”

Section: Lambert-eaton Myasthenic Syndromementioning

confidence: 99%

“…This then impairs ACh vesicle release through a variety of mechanisms that affect the active zone of a neuromuscular junction: downregulation of presynaptic P/Q-type VGCCs, disorganization of vesicle release sites, and upregulation of alternative calcium channels. 2,3 Variable release of ACh results in failure of neuromuscular transmission, manifesting as weakness, autonomic dysfunction, or both. LEMS can occur as a paraneoplastic syndrome associated with malignancy (cancer-associated LEMS) or as an autoimmune phenomenon in the absence of malignancy (nontumor LEMS).…”

Section: Pathophysiologymentioning

confidence: 99%

Lambert-Eaton Myasthenic Syndrome and Botulism

Raja¹

2022

CONTINUUM: Lifelong Learning in Neurology

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PURPOSE OF REVIEW: This article reviews the pathophysiology, epidemiology, clinical features, diagnosis, and treatment of Lambert-Eaton myasthenic syndrome (LEMS) and botulism, presynaptic disorders of neuromuscular transmission in which rapid diagnosis improves long-term outcomes.RECENT FINDINGS: Therapy for LEMS has seen significant advances in recent years due to the approval of amifampridine-based compounds. LEMS is likely still underdiagnosed, particularly when no underlying malignancy is identified. Clinicians must have a strong suspicion for LEMS in any patient presenting with proximal weakness and autonomic dysfunction. Botulism is another rare disorder of presynaptic neuromuscular transmission that is most commonly associated with improper storage or preservation of food products. Over the past 2 decades, wound botulism has been increasingly reported among users of black tar heroin. A high degree of clinical suspicion and electrodiagnostic studies can be beneficial in distinguishing botulism from other acute neurologic disorders, and early involvement of state and federal health authorities may assist in confirming the diagnosis and obtaining treatment. When botulism is suspected, electrodiagnostic studies can provide clinical evidence of disordered neuromuscular transmission in advance of serologic confirmation, and providers should not wait for confirmation of the diagnosis to initiate treatment. SUMMARY: A targeted clinical history and a thorough neurologic examination with support from serologic and electrodiagnostic studies are key to early diagnosis of LEMS and botulism. Early diagnosis of both conditions creates opportunities for therapy and improves outcomes.

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“…Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disease that targets the P/Q-type voltage-gated calcium channel (VGCC) at the motor neuron terminal of the neuromuscular junction. [1][2][3] The classical triad is proximal muscle weakness, decreased tendon reflexes and autonomic dysfunction. 4 In addition, about 10% of patients show cerebellar signs almost exclusively related to small-cell lung carcinoma (SCLC).…”

Section: Introductionmentioning

confidence: 99%

Nationwide survey of Lambert-Eaton myasthenic syndrome in Japan

et al. 2022

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BackgroundThere was no nationwide epidemiological study of Lambert-Eaton myasthenic syndrome (LEMS) in Japan; therefore, we conducted a nationwide survey.MethodsFor the first survey, we sent survey sheets to randomly selected medical departments (n=7545) to obtain the number of LEMS who visited medical departments between 1 January 2017 and 31 December 2017. For the second survey, we sent survey sheets to the corresponding medical departments to obtain clinical information on LEMS.ResultsWe received 2708 responses (recovery rate: 35.9%) to the first survey. We estimated the number of LEMS as 348 (95% CI 247 to 449). The prevalence was 2.7 (95% CI 1.9 to 3.5) in 1 000 000 population. As a result of the second survey, we obtained 30 case records of 16 men and 14 women. Fourteen patients (46.7%) had a tumour, and 10 out of 14 tumours were small-cell lung carcinoma (71.4%). There was a predominance of men in the LEMS with tumour (paraneoplastic LEMS, P-LEMS) (n=11, 78.6%) and women in the LEMS without tumour (a primary autoimmune form of LEMS, AI-LEMS) (n=11, 68.8%) (p=0.0136). The onset age (mean (SD)) for the P-LEMS was 67.1 (9.0), and that for AI-LEMS was 57.8 (11.2) years old (p=0.0103). The disease duration (median) for P-LEMS was 2 years, and for AI-LEMS was 7.5 years (p=0.0134).ConclusionsThe prevalence of LEMS in Japan is similar to that in other countries. There are predominances of men in P-LEMS and women in AI-LEMS.

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Presynaptic Paraneoplastic Disorders of the Neuromuscular Junction: An Update

Cited by 7 publications

References 154 publications

IVIG treatment in non-paraneoplastic Lambert- Eaton myasthenic syndrome with elevated voltage- gated calcium channels antibodies and subacute cerebellar ataxia

IVIG treatment in non-paraneoplastic Lambert- Eaton myasthenic syndrome with elevated voltage- gated calcium channels antibodies and subacute cerebellar ataxia

Lambert-Eaton Myasthenic Syndrome and Botulism

Nationwide survey of Lambert-Eaton myasthenic syndrome in Japan

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