El síndrome de Eaton Lambert: una presentación clínica inusual que suele preceder al diagnóstico oncológico / Eaton lambert syndrome: a rare clinical presentation that usually precedes oncological diagnosis

El síndrome de Eaton Lambert es un trastorno de tipo autoinmune que afecta a la unión neuromuscular. Cursa con debilidad muscular proximal y simétrica y en el 60% de los casos se asocia a una neoplasia subyacente. Dado que esta debilidad es de inicio insidioso, los pacientes suelen consultar inicialmente a su especialista de Medicina Familiar y Comunitaria. Reconocer esta entidad es fundamental para el diagnóstico precoz de la enfermedad oncológica ya que habitualmente precede en meses a los síntomas locales derivados de la enfermedad tumoral. El médico o médica de Atención Primaria tiene un papel fundamental en el adelanto diagnóstico y debe realizar una derivación precoz al medio hospitalario para completar el estudio. Presentamos un caso clínico de síndrome de Eaton Lambert paraneoplásico en el contexto de un carcinoma de pulmón de células pequeñas que trata de ilustrar esta cronología y abordar el proceso diagnóstico y terapéutico.(AU)

Eaton Lambert syndrome is an autoimmune disorder that involves the neuromuscular junction. Clinical course includes proximal muscular weakness and symmetry. In 60% of cases this is associated with underlying neoplasia. Given that this weakness is of insidious onset, patients usually initially consult their family doctor. Recognizing this condition is essential for early diagnosis of oncological disease as it usually occurs a few months prior to local symptoms arising from tumour disease. The family doctor plays a crucial role in early diagnosis and should refer early to hospital to complete the study. We report a case study of paraneoplastic Eaton Lambert syndrome in the context of a small cell lung carcinoma that tries to depict this chronology, in addition to tackling the diagnostic and therapeutic process.(AU)

Lambert-Eaton Myasthenic Syndrome Associated With Extrapulmonary Small Cell Cancer Detected by 18F-FDG PET/CT.

A 46-year-old woman suffering from lower extremity weakness, dysarthria, dysphagia, dyspnea, and dry mouth was suspected of having Lambert-Eaton myasthenic syndrome based on the characteristic electromyographic abnormalities of right ulnar nerve, which demonstrated reduced resting compound muscle action potential amplitude and greater than 100% incremental change in high-frequency repetitive nerve stimulation. An F-FDG PET/CT was performed to detect the underlying neoplasm and revealed a retroperitoneal soft tissue mass with high F-FDG avidity in the left iliac fossa. The patient's symptoms were prominently relieved after the tumor resection, and the histopathology indicated a small cell carcinoma.

Paraneoplastic Lambert-Eaton Myasthenic Syndrome With Limbic Encephalitis: Clinical Correlation With the Coexistence of Anti-VGCC and Anti-GABAB Receptor Antibodies.

OBJECTIVE: To characterize Lambert-Eaton myasthenic syndrome and limbic encephalitis with coexistent voltage-gated calcium channel (VGCC) antibody and γ-aminobutyric acid (GABA) B receptor antibody. METHODS: Case study. RESULTS: A 57-year-old man presented with 6 months of weakness, unsteadiness, and vision difficulties. Examination revealed proximal weakness and diminished reflexes. Electrodiagnostic study revealed low-amplitude motor potentials and facilitation on high-frequency stimulation. Laboratory evaluation identified P/Q-type VGCC antibody. Positron emission tomography identified a mediastinal lesion, confirmed as small-cell lung carcinoma. The patient developed confusion and seizures. Cerebrospinal fluid analysis identified antibodies to GABAB receptor. CONCLUSIONS: This case describes a patient with Lambert-Eaton myasthenic syndrome, limbic encephalitis, and autoantibodies to VGCC and GABAB receptor. Atypical presentation of paraneoplastic neurological syndromes could indicate the presence of a second antibody that may have significant impact on therapy.

Ophthalmoplegia associated with lung adenocarcinoma in a patient with the Lambert-Eaton myasthenic syndrome: A case report.

RATIONALE: The Lambert-Eaton myasthenic syndrome (LEMS) is a neuromuscular disease; its unique symptoms of LEMS include dry mouth with a metallic taste, constipation, and erectile dysfunction. As it is quite rare, isolated ocular muscle impairment associated with LEMS east to ignore. PATIENT CONCERNS: A 65-year-old man presented with alternating ptosis and diplopia. Isolated ocular muscle impairment had lasted for 6 years, and the patient was initially diagnosed with ocular myasthenia gravis (MG). Treatment with azathioprine only slightly improved symptoms over the first 2 months; long-term treatment was not effective. DIAGNOSES: Dynamic observation of chest computed tomography images revealed a slowly progressing nodule in the lower lobe of the left lung. The subsequent pathologic examination following mass resection confirmed a diagnosis of lung adenocarcinoma. INTERVENTIONS: The patient was ultimately diagnosed with the Lambert-Eaton myasthenic syndrome associated with pulmonary adenocarcinoma. OUTCOMES: Resection of the lung tumor relieved all symptoms. LESSONS: Other causes of ocular MG symptoms should be considered when standard MG therapy is ineffective, especially the Lambert-Eaton myasthenic syndrome.

Paraneoplastic neurological syndromes and the role of PET imaging.

Paraneoplastic neurological syndromes are immune-mediated effects of cancer that involve the nervous system and are associated with various anti-neuronal antibodies. They can be associated with several cancers such as lung, gynecologic, thymic, testicular and others, but the diagnosis of cancer often becomes a challenge, since paraneoplastic neurological syndromes usually precede the cancer presentation by months or even years. Furthermore, even when the cancer develops, the tumor size remains small and usually undetectable by conventional diagnostic tests and imaging. Positron emission tomography imaging may be an adjunct to timely diagnosis of the occult malignancy in order to employ the specific cancer treatment. Furthermore, positron emission tomography can assess the extent of the functional abnormality in the brain and monitor its response to treatment.

Managing non-paraneoplastic Lambert-Eaton myasthenic syndrome: clinical characteristics in 25 German patients.

In about 40% of patients LEMS is not a paraneoplastic phenomenon (NT-LEMS). Several clinical aspects important to these patients remain open, especially the question when a LEMS can definitely be diagnosed as NT-LEMS. Here we describe a series of 25 German NT-LEMS patients regarding their clinical characteristics, duration of symptoms, value of serological markers, paraneoplastic antibodies and FDG-PET/CT. Furthermore, we discuss the current diagnostic criteria of NT-LEMS.

[Association of combined small cell and large cell lung cancer with Lambert-Eaton myasthenic syndrome: case report]. / Kombine küçük hücreli ve büyük hücreli akciger kanseri ile Lambert-Eaton miyastenik sendromu birlikteligi: Olgu sunumu.

Lambert-Eaton myasthenic syndrome (LEMS) is a rare type of neuromusculer conduction disorder. This disease can be seen with lung cancer and, it is associated with otoimmunity. Among the symptoms of lung cancer LEMS can be seen, but it is very rare. In this case, LEMS symptoms were analyzed before lung cancer symptoms. The localization of the tumor was near the pulmonary artery. By the early diagnose, the patient had the chance of cure.