scholarly journals Lambert-Eaton Myasthenic Syndrome; Pathogenesis, Diagnosis, and Therapy

2011 ◽  
Vol 2011 ◽  
pp. 1-5 ◽  
Author(s):  
Nils Erik Gilhus
Keyword(s):  
Motor Nerve ◽  
Small Cell Lung Carcinoma ◽  
Small Cell ◽  
Motor Nerve Terminal ◽  
Symptomatic Therapy ◽  
Small Cell Lung ◽  
Cell Lung Carcinoma ◽  
Voltage Gated Calcium Channels ◽  
Myasthenic Syndrome ◽  
Very High

Lambert-Eaton Myasthenic Syndrome (LEMS) is a rare disease with a well-characterized pathogenesis. In 50% of the patients, LEMS is a paraneoplastic manifestation and caused by a small cell lung carcinoma (SCLC). Both LEMS patients with SCLC and those without this tumour have in 85% of cases pathogenetic antibodies of very high LEMS specificity against voltage-gated calcium channels (VGCCs) in the cell membrane of the presynaptic motor nerve terminal. Better understanding of LEMS pathogenesis has lead to targeted symptomatic therapy aimed at the neuromuscular junction and to semispecific immuno-suppression. For SCLC LEMS, tumour therapy is essential.

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.

Anesthesia for an Unsuspected Lambert-Eaton Myasthenic Syndrome with Autoantibodies and Occult Small Cell Lung Carcinoma

1992 ◽  
Vol 76 (1) ◽  
pp. 142-144 ◽  
Author(s):  
STEPHEN SMALL ◽  
HASSAN H. ALI ◽  
VANDA A. LENNON ◽  
ROBERT H. BROWN ◽  
DANIEL B. CARR ◽  
...  
Keyword(s):  
Lung Carcinoma ◽  
Small Cell Lung Carcinoma ◽  
Small Cell ◽  
Small Cell Lung ◽  
Cell Lung Carcinoma ◽  
Myasthenic Syndrome

scholarly journals A Case of Small Cell Lung Carcinoma with SIADH and Lambert-Eaton Myasthenic Syndrome.

Haigan ◽  
1993 ◽  
Vol 33 (3) ◽  
pp. 429-435
Author(s):  
Takashi Shibusa ◽  
Yuji Inoue ◽  
Mitsuo Asakawa ◽  
Chiaki Hamamatsu ◽  
Masakatsu Ando
Keyword(s):  
Lung Carcinoma ◽  
Small Cell Lung Carcinoma ◽  
Small Cell ◽  
Small Cell Lung ◽  
Cell Lung Carcinoma ◽  
Myasthenic Syndrome
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