Definitions

Lambert

Lambert

[lam-bert]
Hitchcock, Lambert, 1795-1852, American chairmaker, b. Cheshire, Conn. In 1818 in Barkhamsted, Conn., Hitchcock established a factory whose employees came to number about 100. The village that the factory created was named Hitchcockville (changed in 1866 to Riverton). Hitchcock at first made parts of chairs and sold the parts to chairmakers. Later, he discontinued this business and manufactured complete chairs that were sold throughout the United States. The Hitchcock chair is characteristic of its time and has come to be sought by collectors. It is a factory product, typically not carved or upholstered, and is painted black over red, often with designs stenciled in colors or bronze. It is of good wood and is sturdily built. The legs and rungs are simple and well turned. The seat is of wood, cane, or rush. The name of L. Hitchcock or of a firm that he formed (Hitchcock, Alford & Company) is stenciled on the back edge of the seat.

See J. T. Kenney, The Hitchcock Chair (1971).

Lambert, Johann Heinrich, 1728-77, German-French philosopher and scientist, b. Alsace. He developed many basic concepts in mathematics, including that of the hyperbolic functions in trigonometry. In physics he achieved valuable results in work on the measurement of the intensity of light (the metric unit of brightness in the cgs system is named for him), degrees of heat, and humidity. In his philosophical work Neues Organon (1764) he pointed out the importance of beginning with experience and using the analytical method to investigate any theory of knowledge. His correspondence with Kant is of great philosophical significance. His other important books are Photometria (1760) and Pyrometrie (1779).
Lambert, John, 1619-83, English parliamentary general. He fought in the first civil war (1642-46) and assisted Henry Ireton in drawing up the Heads of the Proposals in 1647. In 1648 he commanded the Army of the North against the Scots in the second civil war and later took part in the Scottish campaigns of 1650-51 and the defeat of Charles II at Worcester. He played a leading role in drafting the Instrument of Government (1653), by which Oliver Cromwell became protector, but broke with Cromwell over the latter's acceptance of the Humble Petition and Advice. At the fall of Richard Cromwell (1659) Lambert defeated a royalist uprising in Cheshire and assisted in dissolving the Rump Parliament. When Gen. George Monck marched south to restore Parliament, Lambert attempted to join him, but his army deserted him. After the Restoration (1660), he was tried for treason and banished to the island of Guernsey.
Simnel, Lambert, c.1475-1525, imposter and pretender to the English throne. Little is known of his early life, but before 1486 he caught the attention of an Oxford priest, Richard Simon or Symonds, who trained him to impersonate Richard, duke of York, younger son of Edward IV, who is now thought to have died, or been murdered, while imprisoned in the Tower of London. The plan was changed, however, and in 1486 Simon took Simnel to Ireland, claiming that he was Edward, earl of Warwick, another Yorkist claimant to the throne. A number of Yorkist adherents rallied to his cause, and in May, 1487, Simnel and his supporters, led by John de la Pole, earl of Lincoln (see under Pole, family), crossed to England and were defeated by the forces of Henry VII at the battle of Stoke (June, 1487). Simnel was taken prisoner but pardoned and supposedly was employed thereafter as a scullion in the royal kitchen, as a mark of Henry VII's lenience.

(born Feb. 22, 1796, Ghent, Belg.—died Feb. 17, 1874, Brussels) Belgian statistician, sociologist, and astronomer. He is known for his application of statistics and the theory of probability to social phenomena. He collected and analyzed government statistics on crime, mortality, and other subjects and devised improvements in census taking. In Sur l'homme (1835) and L'Anthropométrie (1871) he developed the notion of the homme moyen, the statistically “average man.” A founder of quantitative social science, he was nonetheless widely criticized for the crudeness of his methodology.

Learn more about Quetelet, (Lambert) Adolphe (Jacques) with a free trial on Britannica.com.

Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder which affects calcium channels of the nerve-muscle (neuromuscular) junction. The etiology of LEMS may resemble myasthenia gravis, but there are substantial differences between the clinical presentation and pathogenetic features of the two disorders.

While children and young adults may be affected, the disease is usually observed in middle aged and older individuals. The incidence of the disease is difficult to determine due to its low frequency.

History

Anderson was the first person to mention a case with possible clinical findings of LEMS in 1953, but Lambert, Eaton and Rooke were the first physicians to substantially describe the clinical and electrophysiological findings of the disease in 1966.

It is usually associated with auto-immune self antibodies to the pre-synaptic voltage gated calcium channels, which leads to neuromuscular block.

Causes

While LEMS may be found as a solitary disease, 50% of cases have an associated malignancy. Malignancies that may be found with LEMS may include small-cell lung cancer, lymphoma, non-Hodgkin's lymphoma, T-cell leukemia, non-small cell lung cancer, prostate cancer, and transitional cell carcinoma of the bladder. The myasthenic syndrome associated with thymoma is actually true myasthenia gravis, where weakness worsens with repeated activity (as opposed to LEMS, where weakness improves with repeated activity).

Whether solitary or cancer-associated, the disease is believed to be of autoimmune origin. In 1989, the previously anticipated antibodies were demonstrated to be directed against presynaptic calcium channels, which are located in neuromuscular junction (see synapse) and are responsible for the efficient release of acetylcholine. The calcium channel antibodies prevent the opening of calcium channels and thus prevent the release of acetylcholine.

There are some patients that do not carry these antibodies in their serum samples and the exact cause of disease in these cases still remains to be determined. In cases with both LEMS and lung cancer (usually small cell type), the antibodies are suggested to be aimed at cancer cells and to bind and affect the antigens in neuromuscular junction accidentally.

Clinical findings

The major clinical finding is progressive weakness that does not usually involve the respiratory muscles and the muscles of face. In patients with affected ocular and respiratory muscles, the involvement is not as severe as myasthenia gravis. The proximal parts of the legs and arms are predominantly affected. Many patients have autonomic symptoms like dry mouth or impotence. Reflexes are usually reduced or absent.

Diagnosis

The diagnosis is established by clinical and laboratory findings (chest x-ray for a possible lung malignancy, antibodies to calcium channels, incremental response in repetitive nerve stimulation). Incremental response is an increased response of muscle fibers to very high frequencies of electrical stimulation. Observed increase in the response of muscle fibers proves that there is a difficulty with the release of acetylcholine and this difficulty can be overwhelmed by intensive stimulation.

Treatment

Corticosteroids, azathioprine and 3,4-diaminopyridine are used in treatment of LEMS with limited success. In some cases with a progressive and intractable course, plasma exchange or intravenous immunoglobulin can be tried.

3,4 diaminopyridine work by blocking K+ channel efflux in nerve terminal so that action potential duration is increased. Ca2+ channels can then be open for longer time and allow greater acetylcholine release to stimulate muscle at end plate.

References


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