generate barcode in asp.net using c# THE PERIODIC PARALYSES AND HEREDITARY, NONDYSTROPHIC MYOTONIAS in Microsoft Office

Drawing QR Code in Microsoft Office THE PERIODIC PARALYSES AND HEREDITARY, NONDYSTROPHIC MYOTONIAS

THE PERIODIC PARALYSES AND HEREDITARY, NONDYSTROPHIC MYOTONIAS
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THOMASEN E: Myotonia, Thomsen s Disease, Paramyotonia, Dystrophia Myotonica Aarhus, Denmark, Universitetsforlaget i Aarhus, 1948 THOMSEN J: Tonische Krampfe in willkurlich beweglichen Muskeln in Folge von ererbter psychischer disposition (Ataxia muscularis ) Arch Psychiatr Nervenkr 6:706, 1876 TRUDELL RG, KAISER KK, GRIGGS RC: Acetazolamide-responsive myotonia congenita Neurology 37:488, 1987
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TYLER FH, STEPHENS FE, GUNN FD, PERKOFF GT: Studies on disorders of muscle: VII Clinical manifestations and inheritance of a type of periodic paralysis without hypopotassemia J Clin Invest 30:492, 1951 YAZAKI K, KURIBAYASHI T, YAMAMURA Y, et al: Hypokalemic myopathy associated with a 17 -hydroxylase de ciency: A case report Neurology 32:94, 1982
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DISORDERS OF MUSCLE CHARACTERIZED BY CRAMP, SPASM, PAIN, AND LOCALIZED MASSES
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Quite apart from spasticity and rigidity, which are due to a disinhibition of spinal motor mechanisms, there are forms of muscular stiffness and spasm that can be traced to abnormalities of the lower motor neuron and its spinal inhibitory mechanisms or to the sarcolemma of the muscle ber and its intrinsic conducting apparatus Thus, muscles may go into spasm because of an unstable depolarization of motor axons, which sends volleys of impulses across neuromuscular junctions as occurs in myokymia, hypocalcemic tetany, and pseudohypoparathyroidism In other states, discussed in the preceding chapter, the innervation of muscle may be normal but contraction persists despite attempts at relaxation (myotonia) Or, after one or a series of contractions, the muscle may be slow in decontracting, as occurs in paradoxical myotonia and hypothyroidism; in the contracture of McArdle phosphorylase de ciency and phosphofructokinase de ciency, muscle, once contracted, may lack the energy to relax Each of these conditions evokes the complaint of cramp or spasm, which is variably painful and interferes with free and effective voluntary activity Each condition has its own identifying clinical and electromyographic (EMG) characteristics, and most of them respond favorably to therapy A premium is therefore attached to the clinical differentiation of these phenomena
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Muscle Cramp
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This subject has already been introduced in Chap 48, where it was pointed out that everyone at some time or other experiences muscle cramps Usually they occur during the night, after a day of unusually strenuous activity; less often they occur during the day, either during a period of relaxation or occasionally after a strong voluntary contraction or postural adjustment A random restless or stretching movement will induce a hard contraction of a single muscle (most frequently of the foot or leg) that cannot be voluntarily relaxed The muscle is visibly and palpably taut and painful, and the condition is readily distinguished from an illusory cramp, in which the sensation of cramp is experienced with little or no contraction of muscle The latter phenomenon may occur in normal persons as well as in those with certain peripheral nerve diseases Massage and vigorous stretch of the cramped muscle will cause the spasm to yield, though for a time the muscle remains excitable and subject to recurrent cramps Visible fasciculations may precede and follow the cramp, indicating an excessive excitability of the terminal branches of motor neurons supplying the muscle Sometimes the cramp is so strong that the muscle appears to have been injured; it remains sore to touch and painful upon use for a day or longer Cramps of precordial chest muscles or diaphragm may arouse fear of heart or lung disease In the EMG, the cramp is marked by bursts of high-frequency, high-voltage action potentials, and the precramp
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phase by runs of activity in motor units Why cramps should be painful is not known; probably the demands of the overactive muscle exceed metabolic supply, causing a relative ischemia and accumulation of metabolites Overwork of muscle with or without impairment of circulation is also painful Between cramps, the muscles are normal clinically and electromyographically Cramps are known to increase in frequency under certain conditions and with certain diseases They are frequent during pregnancy for reasons not fully understood Dehydration and sweating predispose to cramping and are a constant threat to athletes, who try to prevent them by ingesting electrolyte solutions Exertional cramps are frequent in motor system disease and hypothyroidism and less so in chronic polyneuropathies Patients undergoing hemodialysis are subject to cramps, which can be suppressed by intravenous hypertonic saline or hypertonic glucose Rapid rehydration after dehydration is another provocative factor Focal cramping occurs after partial nerve or root injury and with diseases involving anterior horn cells In one such condition, the calf muscle on one side is subject to severe recurrent cramps after decompression of the S1 root in lumbar disc disease; in extreme cases, the muscle hypertrophies The mechanism of muscle cramping is obscure In a number of cases with exercise-induced stiffness and muscle pain, sometimes progressing to cramp, below-normal levels of myoadenylate deaminase have been found The signi cance of this observation is uncertain This enzyme, which is present in high concentration in muscle, is thought to function primarily during aerobic exercise and facilitate the regeneration of adenosine triphosphate from adenosine diphosphate through the action of adenylate kinase Others assert that low levels of this enzyme are not speci c, occurring in such unrelated disorders as hypokalemic periodic paralysis and spinal muscular atrophy (see Layzer for details) Quinine sulfate 300 mg at bedtime and repeated in 4 h if necessary, or 300 mg tid for diurnal cramping is an effective medication; diphenhydramine hydrochloride (Benadryl) 50 mg or procainamide 05 to 10 g can be substituted if quinine is not tolerated Phenytoin, carbamazepine, propoxyphene, and sometimes clonazepam are even more useful in alleviating repeated daytime cramping
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