Med Pregl. 2005 Sep-Oct; 58(9-10): 495-7
Zivaljević M, Popović S, Vujkov T

INTRODUCTION: Paraneoplastic neurological syndromes are rare non-metastatic complications of cancer that have an immune-mediated etiology. The Lambert-Eaton myasthenic syndrome (LEMS) is a neuromuscular disorder, often associated with small cell lung carcinoma (SCLC), which is characterized by reduced quantal release of acetylcholine from the motor nerve terminals. LAMBERT-EATON MYASTHENIC SYNDROME: The Lambert-Eaton Myasthenic Syndrome (LEMS) is characterized by proximal muscle weakness initially affecting gait, autonomic symptoms (dry mouth, constipation, erectile failure) and augmentation of strength during initial voluntary activation. Symptomatic treatment of the junctional disorder is based on cholinergic drugs, immunosuppression, immunomodulation and physical therapy useful in case of unsuccessful antineoplastic therapy. CASE REPORT: A rare case of ovarian cancer with Eaton-Lambert syndrome is reported. A 50-year-old woman was admitted to the gynecologic department, complaining of weakness and pain in her arms and shoulders. Physical therapy resulted in partial improvement. Treatment of paraneoplastic syndrome markedly improves the quality of life of cancer patients. Patients presenting with this syndrome should undergo a careful evaluation for the presence of an occult malignancy.

Rom J Intern Med. 2004; 42(4): 725-35
Tănăsescu R, Ticmeanu M, Nicolau A

In the practice of neurology, the type of clinical involvement suggests the site of the lesion and the mechanism beneath. Sometimes, the symptoms can be delusive, turning the diagnostic approach to a wrong path and raising the necessity of an algorithm considering the less probable entities. Dysimmunity as mechanism of neurological disease involving both the neuromuscular junction and peripheral nerves is frequently insidious and difficult to suspect on clinical basis alone. We report the case of a 67-year-old male with atypical Eaton-Lambert syndrome and mononeuropathy probably in relation with lupus-like entity. The patient has also high titers of anticardiolipin antibodies and lupus anticoagulant. We are considering the diagnostic algorithm before an isolated and atypical neurological presentation and reviewing the main neurological manifestations in lupus-like and autoimmune systemic disease. We raise the difficulty to classify an inflammatory connective tissue disease in the absence of other pathologic features than autoantibodies and isolated neurological symptoms and discussing the main therapeutic issues.

Ann N Y Acad Sci. 2003 Sep; 998: 11-7
Nudler S, Piriz J, Urbano FJ, Rosato-Siri MD, Renteria ES, Uchitel OD

Different types of voltage-activated Ca(2+) channels have been established based on their molecular structure and pharmacological and biophysical properties. One of them, the P/Q-type, is the main channel involved in nerve-evoked neurotransmitter release at neuromuscular junctions and the immunological target in Eaton-Lambert Syndrome. At adult neuromuscular junctions, L- and N-type Ca(2+) channels become involved in transmitter release only under certain experimental or pathological conditions. In contrast, at neonatal rat neuromuscular junctions, nerve-evoked synaptic transmission depends jointly on both N- and P/Q-type channels. Synaptic transmission at neuromuscular junctions of the ataxic P/Q-type Ca(2+) channel knockout mice is also dependent on two different types of channels, N- and R-type. At both neonatal and P/Q knockout junctions, the K(+)-evoked increase in miniature endplate potential frequency was not affected by N-type channel blockers, but strongly reduced by both P/Q- and R-type channel blockers. These differences could be accounted for by a differential location of the channels at the release site, being either P/Q- or R-type Ca(2+) channels located closer to the release site than N-type Ca(2+) channels. Thus, Ca(2+) channels may be recruited to mediate neurotransmitter release where P/Q-type channels seem to be the most suited type of Ca(2+) channel to mediate exocytosis at neuromuscular junctions.

Rev Neurol. 2000 Dec 16-31; 31(12): 1225-8
Rosich-Estrago M

INTRODUCTION: Some forms of myasthenia gravis and many cases of Eaton-Lambert syndrome are disorders of neuromuscular transmission possibly of paraneoplastic origin. Paraneoplastic necrotizing myopathy and some cases of inflammatory myopathy (polymyositis-dermatomyositis) are paraneoplastic myopathies. DEVELOPMENT: These are all autoimmune processes. The diagnosis is clinical, based mainly on the characteristics of the muscle weakness and motor disorders, confirmed by serological methods (demonstration of antibodies) pharmacological and electromyographic tests, by imaging studies and in some cases biopsy. Treatment includes specific symptomatic measures (aimed at correcting the defect in neuromuscular transmission in the myasthenic syndromes, reducing axonal auto-excitability in the neuromuscular hyperexcitability syndromes, etc.) and pathogenic treatment (basically immunosuppression). A search for the hidden tumour should also be part of the diagnostic investigation. The paraneoplastic disease may remit if the associated tumour is treated.

Genet Couns. 2000; 11(2): 153-6
Cohen J, Schanen NC

The features of Goldenhar complex have been well-described and classically include branchial arch abnormalities, epibulbar dermoid and vertebral abnormalities. We have identified an infant with these features in association with complex congenital heart disease and intrahepatic biliary atresia. Although Lambert described an autosomal recessive disorder with an association of biliary atresia and branchial arch abnormalities, none of those cases had epibulbar dermoid. Diagnostic considerations in this case include inclusion of biliary atresia as a new feature in the expanding spectrum of the Goldenhar complex, versus Lambert syndrome with epibulbar dermoid.

South Med J. 2000 May; 93(5): 514-5
Murray CK, Wortmann GW

Fluoroquinolones have been associated with peripheral sensory disorders and weakness, especially in patients with underlying myasthenia gravis or myasthenia-like Eaton-Lambert syndrome. Trovafloxacin is a relatively new quinolone for which these side effects have not been described. We report a case of diffuse weakness due to a demyelinating polyneuropathy that began after initiation of trovafloxacin in a patient without an underlying neurologic disorder.

Med Arh. 1999; 53(3 Suppl 1): 27-31
Zutić H

The incidence of lung cancer has exploded in this century. The disease is most commonly diagnosed in the 65-75 year age group but can occur as young as 30 years. Cigarette smoking is usually related to lung cancer in men and the risk of developing lung cancer increases with duration of smoking and the number of cigarettes smoked per day. The risk of developing lung cancer decreased to almost that of non smokers after 10-15 years of non smoking. People exposed to asbestos have an 80 fold increase in lung cancer if they smoke. Some increased risk is associated with exposure to radioactive materials--(uranium when mined underground), arsenic and chromium. Lung cancer is more common in city than in rural dwellers. Cell Types: Squamous 34%, Large Cell 28%, Adenocarcinoma and Bronchoalveolar 20%, Small Cell 17%, Mixed 1%. Clinical Presentations: Patients with lung cancer present with several symptoms, signs, and laboratory findings, depending upon the location, growth characteristics, and metabolic activity of the tumour. Asymptomatic: usually patients with solitary pulmonary nodules. Non-specific systemic symptoms: fatigue, weight loss and loss of appetite. Respiratory Symptoms: Haemoptysis, symptoms (and signs) of intrathoracic spread of tumour (hoarseness, pleuritis or pleural effusion, paralysed diaphragm, dysphagia, superior vena cava obstruction, Pancoast s syndrome. Symptoms from extrathoracic metastases: (mediastinal supraclavicular and neck), liver, brain, adrenals, bone and contralateral lung, in approximately that order. Systemic syndromes unassociated with metastatic spread of the primary tumour, often seen with the undifferentiated cell-types: Endocrinopathies (1. hypercalcemia, 2. Cushing s syndrome, 3. inappropriate secretion of antidiuretic hormone (SIADH), 4. gynecomastia, 5. carcinoid syndrome (rare); Neuromuscular disorders (1. myasthenic (Eaton-Lambert) syndrome, 2. peripheral neuropathy, 3. cerebellar degeneration); Connective-tissue syndromes (1. clubbing of fingers/toes (common), 2. hypertrophic pulmonary osteoarthropathy (rare), 3. dermatomyositis, 4. acanthosis nigricans, 5. non specific arthralgia). Diagnosis-The three requirements for diagnosis of lung cancer are: locating the tumour precisely, determining its specific histological cell type, assessing its extent (staging). The next diagnostic procedures are useful: sputum cytology, Pleural aspiration and biopsy, fibre optic bronchoscopy, needle aspiration biopsy. Surgery is the treatment of choice in NSCLC. Chemotherapy: it is the treatment of choice in those patients with small cell carcinoma with limited disease. The combination of chemotherapy with concurrent radiotherapy is now used. Prognosis for patients with lung cancer depends upon cell type, tumour size, location, spread, coexisting disease, and the patient s age. Numerous regimes of palliative radiotherapy and chemotherapy have been investigated as alternatives or adjuncts to surgery; results are often encouraging in the short term, poor in the long term. A great deal of clinical research continues in this area.

Masui. 1999 Mar; 48(3): 260-4
Onozawa H, Tanaka T, Kagaya S, Kumagai M, Asano T, Tanifuji Y

A 66-year-old male with colon cancer was scheduled for left hemicolectomy. He had a past history of respiratory failure due to chronic obstructive pulmonary disease (COPD). Anesthesia method chosen was general anesthesia with sevo-flurane combined with epidural anesthesia. Respiration was managed with assisted ventilation using laryngeal mask airway and muscle relaxation was obtained with suxamethonium chloride given intermittently. After the operation, he did not seem to have COPD because of the relation between arterial PCO2 and bicarbonate in the perioperative period. Therefore, after obtaining informed consent from this patient, we determined the relation between arterial and spinal fluid acid-base balance under acetazolamide administration. He was more sensitive to central respiratory response because his respiration increased following the decrease of spinal fluid bicarbonate. We further examined and diagnosed him as Eaton-Lambert syndrome by evoked electromyography and by Ca2+ channel antibody.

Oncol Rep. 1999 May-Jun; 6(3): 665-8
Sherer Y, Shoenfeld Y

Although some autoimmune diseases are known to be associated with a higher incidence of cancers, they are not usually considered as paraneoplastic diseases, but rather as autoimmune diseases that may be neoplasia-associated, since currently it is not known whether the tumor leads to their development, or alternatively another underlying condition turns the patients prone to both cancer and autoimmunity. We review the association of cancer with pemphigus, paraneoplastic pemphigus, myasthenia gravis and Eaton-Lambert syndrome, and discuss the importance of looking for an occult malignancy in a patient with a newly-diagnosed autoimmune disease that is known to be associated with cancer.

Acta Med Croatica. 1997; 51(4-5): 225-8
Kes P

Neurologic diseases are one of the most common indications for therapeutic plasma exchange (TPE). In autoimmune neurologic diseases like Guillain-Barre syndrome, myasthenia gravis, chronic inflammatory demyelinating polyneuropathy, and paraprotein-associated polyneuropathy, TPE has been found to be beneficial. In some others (e.g., multiple sclerosis and Eaton-Lambert syndrome), TPE cannot be considered a generally accepted treatment option.

Jpn J Clin Oncol. 1996 Jun; 26(3): 128-33
Sakuragi T, Oshita F, Nagashima S, Kasai T, Kurata T, Fukuda M, Yamamoto N, Ohe Y, Tamura T, Eguchi K, Shinkai T, Saijo N

To assess the feasibility of treatments for patients with small cell lung cancer (SCLC) showing a poor performance status (PS, Eastern Cooperative Oncology Group; ECOG 3 or 4), we retrospectively reviewed the outcome for 13 SCLC patients showing poor PS treated at the National Cancer Center Hospital between January 1984 and May 1994. The main factors which contributed to poor prognosis were superior vena cava (SVC) syndrome, massive pleural effusion, tracheal stenosis due to lymph node swelling, pericardial effusion and pulmonary fibrosis (causing dyspnea in combination), brain metastasis resulting in neurological disturbance, cachexia, Eaton-Lambert syndrome causing muscle weakness, retroperitoneal lymph node metastasis causing abdominal pain, peritoneal effusion due to abdominal lymph node swelling, vertebral metastasis causing paraplegia, and dermatomyositis/polymyositis (DM/PM) causing muscle weakness. All of the patients received chemotherapy with or without radiotherapy. The PS of 8 patients improved with treatment, but no improvement was seen in 5. We analyzed these 13 patients and considered the treatments for those with poor PS. Chemo-radiotherapy was tolerable in SCLC patients showing PS 3, and improved their PS if severe conditions or combined disease did not arise concurrently. It was further suggested that PS 4 patients with severe conditions or combined disease should not be given the treatments.

Scand J Urol Nephrol. 1995 Sep; 29(3): 351-3
Agarawal SK, Birch BR, Abercrombie GF

Eaton-Lambert myasthenic syndrome is rare. We report the first case of adenocarcinoma of prostate associated with this syndrome. Subsequent treatment by orchidectomy caused tumour regression and remission of the syndrome. The patient remains well 9 months after the treatment.