Plasma exchange in polyneuropathy associated with monoclonal gammopathy of undetermined significance

Article Abstract:

Monoclonal gammopathy of undetermined significance (MGUS) is a condition in which monoclonal antibody proteins (proteins made by cells that are clones; hence, identical) appear in the blood and abnormalities characteristic of other disorders are not present. Peripheral neuropathy (disease of the peripheral nerves) is associated with MGUS; in many cases, the neuropathy progresses and may lead to serious sensory and motor disability or even death. One treatment for MGUS is plasma exchange, in which the patient's plasma (containing the monoclonal proteins) is removed and albumin is reinfused. The effectiveness of this approach was evaluated in this controlled study involving 39 patients with neuropathy and MGUS. The patients were randomly assigned to undergo plasma exchange (19 subjects) or sham exchange (20) twice weekly for three weeks. The extent of their neurologic disability and weakness was rated according to a standard scoring system. Results indicated that the average disability score improved by 2 points in the sham-exchange group and by 12 points in the plasma-exchange group, a significant difference. Muscle weakness scores also improved considerably after plasma exchange (improvement of 1 and 10 points, respectively, in the two groups). Muscle action potentials (the electrical impulses that cause muscles to contract) improved in the plasma-exchange group but grew worse (declined) in the sham-exchange group. Sensory measures did not change significantly between the groups. When the patients who had undergone sham exchange subsequently underwent plasma exchange, their neuropathy disability, muscle weakness, and muscle action potential scores improved markedly. The subjects suffered from MGUS associated with IgG, IgA, or IgM gammopathies (affecting proteins of those antibody classes): it appeared that those with IgM gammopathy had a poorer response to plasma exchange than those with IgG or IgA gammopathy. (Consumer Summary produced by Reliance Medical Information, Inc.)

Care and treatment, Usage, Plasma exchange (Therapeutics)

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3,4-Diaminopyridine in the treatment of Lambert-Eaton myasthenic syndrome

Article Abstract:

Lambert-Eaton myasthenic syndrome is a neurological disorder first described in 1956 which is associated with lung cancer. Symptoms include muscle weakness, poor reflex response, and impairment of the autonomic nervous system which controls involuntary bodily functions. The disease results from an inhibition of the release of acetylcholine by nerve fibers; nerve impulses are not transmitted effectively between nerves and between nerves and muscles. Treatment with aminopyridines increases the availability of acetylcholine and the use of these drugs has shown some therapeutic success. Recent studies in animals indicated that 3,4-diaminopyridine may be effective in treating Lambert-Eaton myasthenic syndrome without producing the side effects caused by other drug therapies. Twelve patients with Lambert-Eaton myasthenic syndrome were given daily doses of up to 100 mg of 3,4-diaminopyridine over several months. Marked improvement in these patients was measured by increased muscle strength and relief of autonomic symptoms. A return of muscle strength from 70 percent to 81 percent of normal was noted in the upper body and from 45 to 65 percent normal capacity in the legs. The few side effects observed included stomach upset, light-headedness, and fatigue. One patient had a seizure. Overall, 3,4-diaminopyridine was an effective treatment for Lambert-Eaton myasthenic syndrome and investigators recommended use of this drug alone or in conjunction with other therapies.

Drug therapy, Neuromuscular diseases, Cancer, Aminopyridines

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Prevalence of monoclonal gammopathy of undetermined significance

Article Abstract:

A study was conducted, using sensitive laboratory techniques, to ascertain the prevalence of monoclonal gammopathy of undetermined significance (MGUS), in a large population in a well-defined geographic area. It was revealed that among the subject residents of Olmsted County, Minnesota, MGUS was found in 3.2 percent of persons 50 years of age or older and 5.3 percent of persons 70 years of age or older.

Science & research, Minnesota, Research, Diagnosis

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