Disease Information for Eaton-Lambert syndrome

Synonyms:

Eaton Lamb myasthenic syndr, Eaton Lambert (etiology), Eaton Lambert (manifestation), Eaton Lambert Myasthenic Myopathic Syndrome, Eaton Lambert Myasthenic Syndrome, Eaton Lambert Myopathic Myasthenic Syndrome, Eaton Lambert Myopathic Myasthenic Syndromes, EATON LAMBERT ROOKE SYNDROME, Eaton Lambert syndrome, Eaton Lambert syndrome (disorder), Lambert Eaton (etiology), Lambert Eaton (manifestation), Lambert Eaton Myasthenic Myopathic Syndrome, Lambert Eaton Myasthenic Myopathic Syndromes, Lambert Eaton myasthenic syndr, Lambert Eaton Myasthenic Syndrome, Lambert Eaton Myopathic Myasthenic Syndrome, Lambert Eaton Myopathic Myasthenic Syndromes, Lambert Eaton Syndrome, LEMS Lambert Eaton myasthenic syndrome, LEMS Lambrt Eatn myasth synd, MYASTHENIC MYOPATHIC SYNDROME LAMBERT EATON, Myasthenic Myopathic Syndrome of Eaton Lambert, Myasthenic Myopathic Syndrome of Lambert Eaton, Myasthenic syndrome, Myasthenic Syndrome Eaton Lambert, Myasthenic Syndrome Lambert Eaton, myasthenic syndrome of Lambert Eaton, Myopathic Myasthenic Syndrome of Eaton Lambert, MYOPATHIC MYASTHENIC SYNDROME OF LAMBERT EATON, Syndrome Eaton Lambert, Syndrome Eaton Lambert Myasthenic, Syndrome Lambert Eaton, Syndrome Lambert Eaton Myasthenic

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Eaton Lamb myasthenic syndr, Eaton Lambert (etiology), Eaton Lambert (manif... (more)

Definition:

An autoimmune disease characterized by weakness and fatigability of proximal muscles, particularly of the pelvic girdle, lower extremities, trunk, and shoulder girdle; There is relative sparing of extraocular and bulbar muscles; CARCINOMA, SMALL CELL of the lung is a frequently associated condition, although other malignancies and autoimmune diseases may be associated; Muscular weakness results from impaired impulse transmission at the NEUROMUSCULAR JUNCTION; Presynaptic calcium channel dysfunction leads to a reduced amount of acetylcholine being released in response to stimulation of the nerve; (From Adams et al, Principles of Neurology, 6th ed, pp 1471)---------------------------------------The Eaton-Lambert syndrome is an immune-mediated, myasthenia-like syndrome with weakness usually affecting the limbs and sparing ocular and bulbar muscles; It is presynaptic, resulting from impaired release of acetylcholine from nerve terminals; An IgG antibody is involved; The syndrome can precede, occur with, or develop after the diagnosis of cancer; It occurs most commonly in men with intrathoracic tumors (70% have small or oat cell lung carcinoma); Symptoms and signs include fatigability, weakness, sometimes

pain in proximal limb muscles, peripheral paresthesias, dry mouth, impotence, and ptosis; Deep tendon reflexes are reduced or lost; The diagnosis is confirmed by finding an incremental response to repetitive nerve stimulation ; Amplitude of the compound muscle action potential increases > 200% at rates > 10 Hz; Treatment is first directed at the underlying malignancy and sometimes induces remission; Guanidine (initially 125 mg po qid, gradually increased to a maximum of 35 mg/kg), which facilitates acetylcholine release, often lessens symptoms but may depress bone marrow and liver function; Corticosteroids and plasmapheresis benefit some patients; [Adapted from Merck Manual 17th] ----------------------------

Lambert-Eaton Myasthenic Syndrome, Eaton-Lambert Syndrome,

Lambert-Eaton Syndrome, Myasthenic Syndrome of Lambert-Eaton; Lambert-Eaton Myasthenic Syndrome (LEMS) is a rare autoimmune disorder characterized by the gradual onset of muscle weakness, especially of the pelvic and thigh muscles; Approximately 60 percent of LEMS cases are associated with a small cell lung cancer, and the onset of LEMS symptoms often precedes the detection of the cancer [nord 2005]------------------------------------------------

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