scholarly journals The Lambert-Eaton Myasthenic Syndrome — an Overview

2018 ◽  
Vol 02 (01) ◽  
pp. E40-E45
Author(s):  
Siegfried Kohler ◽  
Andreas Meisel
Keyword(s):  
Myasthenia Gravis ◽  
Case Reports ◽  
Compound Muscle Action Potential ◽  
Small Cell Lung Carcinoma ◽  
Immunosuppressive Treatment ◽  
Myasthenic Crisis ◽  
Cell Lung Carcinoma ◽  
Myasthenic Syndrome ◽  
Exercise Induced

AbstractThe Lambert Eaton myasthenic syndrome (LEMS) has a prevalence of around 5/100 0000 and is around 10–20 times rarer than myasthenia gravis (MG). Although LEMS does have a number of similarities to MG, there are important differences. The syndrome is characterized by a mostly proximally localised exercise induced muscle weakness that can lead to respiratory failure often accompanied by autonomous dysfunction. Disease symptoms are caused by autoantibodies directed against P/Q type voltage gated calcium channels (VGCC) that are expressed in the presynaptic motoric nerve terminals. The diagnosis of LEMS is based on the detection of the pathogenic anti-VGCC antibodies as well as the observation of an increment of at least 60% in the electrophysiological examination of an affected muscle. An increment is defined by an increase of the at rest reduced compound muscle action potential (CMAP) either after voluntary maximal innervation or after high frequent (≥20 Hz) stimulation. In almost one third LEMS is of paraneoplastic origin. Therefore an intensive tumor screening is necessary after diagnosis.There are some differences in the clinical presentation between paraneoplastic (pLEMS) and the exclusively autoimmune (aiLEMS) form of LEMS. With respect to this the DELTA-P-Score and the detection of SOX1-antibody are important. The most frequent tumor associated with LEMS is small cell lung carcinoma (SCLC). Therapy is based on the initial distinction between paraneoplastic and autoimmune ethiology. pLEMS necessitates therapy of underlying neoplasia. Usually, aiLEMS- as well as pLEMS patients respond well to 3,4 diaminopyridine (3,4 DAP) often augmented by pyridostigmine. Similar to treatment of myasthenia gravis long-term immunosuppressive treatment is usually required to control symptoms effectively. Myasthenic crisis in LEMS can be controlled by intensive care and immunoglobulins, plasmaphereses or immunoadsorption. Based on case reports more specific immunomodulatory treatment approaches such as the B-cell depleting therapeutic antibody rituximab should be considered in therapy refractory courses of LEMS. Long-term prognosis of autoimmune LEMS with respect to clinical stabilization with (pharmacological) remission is good, although in around 75% of patients significant reductions in quality of life remain. Prognosis of tumor-associated LEMS is largely determined by the tumor and its effective therapy. Curative treatment of the tumour as well as complete remission of pLEMS are possible.

Long‐term prognosis of Japanese Lambert–Eaton myasthenic syndrome patients with or without small‐cell lung carcinoma

2020 ◽  
Vol 11 (2) ◽  
pp. 131-134
Author(s):  
Yuta Kojima ◽  
Akiyuki Uzawa ◽  
Kazumoto Shibuya ◽  
Manato Yasuda ◽  
Yukiko Ozawa ◽  
...  
Keyword(s):  
Lung Carcinoma ◽  
Small Cell Lung Carcinoma ◽  
Small Cell ◽  
Small Cell Lung ◽  
Cell Lung Carcinoma ◽  
Myasthenic Syndrome ◽  
Long Term Prognosis

scholarly journals Serum sCD25 Protein as a Predictor of Lack of Long-Term Benefits from Immunotherapy in Non-Small Cell Lung Cancer: A Pilot Study

Cancers ◽  
2021 ◽  
Vol 13 (15) ◽  
pp. 3702
Author(s):  
Anna Siemiątkowska ◽  
Maciej Bryl ◽  
Katarzyna Kosicka-Noworzyń ◽  
Jakub Tvrdoň ◽  
Iwona Gołda-Gocka ◽  
...  
Keyword(s):  
Treatment Cycle ◽  
Small Cell Lung Carcinoma ◽  
Interleukin 2 ◽  
Serum Levels ◽  
Small Cell ◽  
Soluble Form ◽  
Small Cell Lung ◽  
Cell Lung Carcinoma ◽  
Non Invasive

Prognosis of advanced non-small cell lung carcinoma (NSCLC) is poor. Even though it can improve with anti-PD-1/PD-L1 agents, most patients do not respond to treatment. We hypothesized that the serum soluble form of the unit α of the interleukin-2 receptor (sCD25) could be used as a biomarker of successful immunotherapy in NSCLC. We recruited patients dosed with atezolizumab (n = 42) or pembrolizumab (n = 20) and collected samples at baseline and during the treatment. Levels of sCD25 were quantified with the ELISA kits. Patients with a high sCD25 at baseline (sCD25.0 ≥ 5.99 ng/mL) or/and at the end of the fourth treatment cycle (sCD25.4 ≥ 7.73 ng/mL) progressed faster and lived shorter without the disease progression and serious toxicity. None of the patients with high sCD25 at both time points continued therapy longer than 9.3 months, while almost 40% of patients with low sCD25 were treated for ≥12.3 months. There was a 6.3-times higher incidence of treatment failure (HR = 6.33, 95% CI: 2.10–19.06, p = 0.001) and a 6.5-times higher incidence of progression (HR = 6.50, 95% CI: 2.04–20.73, p = 0.002) in patients with high compared with low sCD25.0 and sCD25.4. Serum levels of sCD25 may serve as a non-invasive biomarker of long-term benefits from the anti-PD-1/PD-L1s in NSCLC.

Difficulty Climbing the Stairs

2021 ◽  
pp. 149-151
Author(s):  
Anastasia Zekeridou ◽  
Vanda A. Lennon
Keyword(s):  
Computed Tomography ◽  
Intravenous Immunoglobulin ◽  
Lung Carcinoma ◽  
Small Cell Lung Carcinoma ◽  
Concurrent Chemotherapy ◽  
Small Cell ◽  
Small Cell Lung ◽  
Cell Lung Carcinoma ◽  
History Of ◽  
Myasthenic Syndrome

A 72-year-old woman with a history of rheumatoid arthritis and chronic obstructive pulmonary sought care for a 3-month history of progressive difficulty walking on uneven terrain and climbing stairs. In the 2 preceding weeks, she also noted difficulty standing up from a seated position. She reported no sensory symptoms but recently noticed dry mouth and new-onset constipation with decreased appetite. Electromyography showed diffusely low-amplitude compound muscle action potential responses to single-nerve stimuli at rest, with normal sensory nerve action potentials. Studies of the ulnar and femoral motor nerves demonstrated a decrement to low-frequency repetitive stimulation (12%) and substantial postexercise facilitation (200%) and decrement repair. The serum was positive for cyclic citrullinated peptide antibody, rheumatoid factor, and P/Q-type voltage-gated calcium channel antibody. Computed tomography of the chest showed subcarinal and right hilar lymphadenopathy without evidence of a primary lesion, with avidity on 18F-fludeoxyglucose–positron emission tomography/computed tomography. Transbronchial fine-needle aspiration biopsy of the lymph node revealed small cell lung carcinoma. The patient was diagnosed with Lambert-Eaton myasthenic syndrome and small cell lung carcinoma. Concurrent chemotherapy and radiation were administered for the small cell lung carcinoma, with some improvement of the patient’s weakness. Symptomatic treatment for Lambert-Eaton myasthenic syndrome was initiated. Therapy with 3,4-diaminopyridine improved the patient’s weakness, but her daily activities were limited by persistent, moderate, lower extremity weakness. The weakness objectively improved with intravenous immunoglobulin therapy. Two years later, the patient was maintained on 3,4-diaminopyridine and monthly intravenous immunoglobulin, with minimal persistent weakness and no evidence of cancer recurrence. Lambert-Eaton myasthenic syndrome was first described at Mayo Clinic in 1956 as a “myasthenic syndrome associated with malignant tumors” that had characteristic electromyographic findings, later shown to be presynaptic by microelectrophysiologic testing.

SOX Antibodies in Small-Cell Lung Cancer and Lambert-Eaton Myasthenic Syndrome: Frequency and Relation With Survival

2009 ◽  
Vol 27 (26) ◽  
pp. 4260-4267 ◽  
Author(s):  
Maarten J. Titulaer ◽  
Rinse Klooster ◽  
Marko Potman ◽  
Lidia Sabater ◽  
Francesc Graus ◽  
...  
Keyword(s):  
Western Blot ◽  
High Throughput Screening ◽  
Small Cell Lung Carcinoma ◽  
Diagnostic Value ◽  
Small Cell ◽  
Enzyme Linked Immunosorbent Assay ◽  
Small Cell Lung ◽  
Serum Samples ◽  
Cell Lung Carcinoma ◽  
Myasthenic Syndrome

Purpose SOX1 antibodies are common in small-cell lung carcinoma (SCLC) with and without paraneoplastic syndrome (PNS) and can serve as serological tumor marker. Addition of other antibodies might improve its diagnostic power. We validated an enzyme-linked immunosorbent assay (ELISA) to assess the diagnostic value of serum antibodies in SCLC and Lambert-Eaton myasthenic syndrome (LEMS). Clinical outcome with respect to SOX antibodies was evaluated, as the SOX-related antitumor immune response might help to control the tumor growth. Patients and Methods We used recombinant SOX1, SOX2, SOX3, SOX21, HuC, HuD, or HelN1 proteins in an ELISA to titrate serum samples and validated the assay by western blot. We tested 136 consecutive SCLC patients, 86 LEMS patients (43 with SCLC), 14 patients with SCLC and PNS (paraneoplastic cerebellar degeneration or Hu syndrome), 62 polyneuropathy patients, and 18 healthy controls. Results Our ELISA was equally reliable as western blot. Forty-three percent of SCLC patients and 67% of SCLC-LEMS patients had antibodies to one of the SOX or Hu proteins. SOX antibodies had a sensitivity of 67% and a specificity of 95% to discriminate between LEMS with SCLC and nontumor LEMS. No difference in survival was observed between SOX positive and SOX negative SCLC patients. Conclusion SOX antibodies are specific serological markers for SCLC. Our assay is suitable for high throughput screening, detecting 43% of SCLC. SOX antibodies have diagnostic value in discriminating SCLC-LEMS from nontumor LEMS, but have no relation to survival in patients with SCLC.

scholarly journals Myasthenia Gravis following Low-Osmolality Iodinated Contrast Media

2014 ◽  
Vol 2014 ◽  
pp. 1-2 ◽  
Author(s):  
Luca Bonanni ◽  
Michele Dalla Vestra ◽  
Andrea Zancanaro ◽  
Fabio Presotto
Keyword(s):  
Contrast Media ◽  
Myasthenia Gravis ◽  
Case Reports ◽  
Orotracheal Intubation ◽  
Myasthenic Crisis ◽  
Iodinate Contrast Agent ◽  
Iodinated Contrast ◽  
History Of ◽  
Total Body ◽  
Progressive Dysphagia

We describe the case of 79-year-old man admitted to our general hospital for a 6-week history of progressive dysphagia to solids and liquids associated with weight loss. To reach a diagnosis a total body CT scan with low-osmolality iodinate contrast agent was performed. Two hours later the patient developed an acute respiratory failure requiring orotracheal intubation and mechanical ventilation. The laboratory and neurological tests allow formulating the diagnosis of myasthenia gravis. In literature, other three case reports have associated myasthenic crisis with exposure to low-osmolality contrast media. This suggests being careful in administering low-osmolality contrast media in myasthenic patients.

Plasmaexchange in the Treatment of Myasthenia Gravis Associated with Thymoma

2003 ◽  
Vol 26 (2) ◽  
pp. 170-173 ◽  
Author(s):  
L. Gogovska ◽  
R. Ljapcev ◽  
M. Polenakovic ◽  
L. Stojkovski ◽  
M. Popovska ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Cholinesterase Inhibitors ◽  
Immunosuppressive Agents ◽  
Ventilatory Support ◽  
Term Therapy ◽  
First Year ◽  
Myasthenic Crisis ◽  
History Of ◽  
Long Term Therapy

Background All patients with thymomatous Myasthenia Gravis (MG) should undergo early and total thymectomy, but controversy abounds in the choice of chronic immunosuppressive agents. The value of plasmaexchange (PE) in MG has been clearly estabilshed in preoperative preparation and treatment of myasthenic crisis. Whether PE may be used in the chronic long-term therapy of patients with thymomatous MG in addition to conventional immunosuppressive agents and cholinesterase inhibitors is yet to be answered. Case history We present a 40-year old woman with an 11 year history of MG. Thymectomy was done during the first year of the disease and the histopathologic finding was thymoma. To sustain clinical remission after thymectomy she continued with immunosuppression with methylprednisolone and cyclosporin A (or azathioprine) in addition to cholinesterase inhibitors. Despite the almost continuous immunosuppression, the disease course continued with fluctuating myasthenic weakness which few times progressed to myasthenic crisis requiring mechanical ventilation. During myasthenic crisis we performed 6–8 plasmapheresis at 2–3 day intervals in addition to conventional immunosuppressive therapy. The disease rapidly worsened in January 2000 and we started with intermittent plasmapheresis (3–6 procedures at 2–3 day intervals, every 6–8 weeks) in order to sustain remission. With this therapeutic protocol, during 20 months follow-up we managed to prevent myasthenic crisis and to avoid ventilatory support. Conclusions Plasmaexchange could be used as a successful and safe therapeutic tool in chronic long-term therapy in addition to conventional immunosuppressive agents to sustain remission in patients with MG. This is particularly important in the treatment of patients with thymomatous MG because they have an increased frequency of myasthenic crisis and often respond poorly an to immunosuppression with steroids or other immunosuppressants.

scholarly journals Long-term survival in limited-stage small cell lung carcinoma: Experience in Rochester, New York from 1975 to 1981

Cancer ◽  
1986 ◽  
Vol 58 (6) ◽  
pp. 1193-1198 ◽  
Author(s):  
T. K. George ◽  
Denis Fitzgerald ◽  
Bruce S. Brown ◽  
Christy Chuang ◽  
Robert F. Asbury ◽  
...  
Keyword(s):  
New York ◽  
Lung Carcinoma ◽  
Small Cell Lung Carcinoma ◽  
Small Cell ◽  
Small Cell Lung ◽  
Term Survival ◽  
Cell Lung Carcinoma ◽  
Long Term Survival ◽  
Limited Stage
Sign in / Sign up

Export Citation Format

Share Document