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These terms refer to a distinctive cerebral disease in which a rapidly progressive and profound dementia is associated with diffuse myoclonic jerks, and a variety of other neurologic abnormalities, mainly visual or cerebellar. The major neuropathologic changes are in the cerebral and cerebellar cortices; the outstanding features are widespread neuronal loss and gliosis accompanied by a striking vacuolation or spongy state of the affected regions hence the designation subacute spongiform encephalopathy (SSE). Less severe changes in a patchy distribution are found in cases with a briefer clinical course. These changes, both clinical and pathologic, occur with such uniformity from case to case that they doubtless form a nosologic entity. It is generally referred to as Creutzfeldt-Jakob disease, an inappropriate eponym, since it is most unlikely that the patient described by Creutzfeldt and at least 3 of the 5 patients described by Jakob had the same disease that we now recognize as subacute using barcode implementation for microsoft word control to generate, create barcode image in microsoft word applications. buildin BusinessRefinery.com/ barcodesusing barcode drawer for excel microsoft control to generate, create bar code image in excel microsoft applications. label BusinessRefinery.com/ bar codeTREMOR, MYOCLONUS, FOCAL DYSTONIAS, AND TICS
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CHAPTER 5 Exponents and Roots
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40. Initialization means assigning
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roidism, accounting for the complaint of uncomfortable tightness of proximal limb muscles. A curious rippling phenomenon in muscles called myokymia, inherited as an autosomal dominant trait, has been observed in several families (Chap. 55). After a period of relaxation, a stiffening and rippling occurs in the contracting or stretched muscles. The stiffness is reduced by dantrolene but not by tocainamide. Acquired forms of myokymia are due to diverse processes, in particular nerve injury and regeneration (especially in the face, as noted in Chap. 47), hypothyroidism, and obscure causes such as thymoma. A prolonged failure of relaxation following contraction of a muscle is characteristic of the myotonic phenomenon, which typi es certain diseases congenital myotonia (of Thomsen), myotonic dystrophy, and the paramyotonia of Eulenberg (Chap. 54). True myotonia, with its prolonged discharge of membrane action potentials, requires strong contraction to elicit, is more evident after a period of relaxation, and tends to disappear with repeated contractions (pages 1265 and 1363). Paradoxical myotonia refers to an increase in the degree of myotonia during a series of contractions (the reverse of what happens in the usual type of myotonia). It also occurs in some cases of Eulenberg paramyotonia. This persistence of contraction is demonstrable also by tapping a muscle (percussion myotonia), a phenomenon easily distinguished from the electrically silent local bulge (myoedema) induced by tapping the muscle of a myxedematous or cachetic patient and from the brief fascicular contraction that is induced by tapping a normal or partially denervated muscle; the latter is referred to as idiomuscular contraction. It should be noted that in patients with hyperactive tendon re exes, striking the muscle rather than its tendon can elicit a stretch re ex. An increment in power with a series of several voluntary contractions in the absence of myotonia is a feature of the inverse myasthenic (Lambert-Eaton) syndrome, which is associated in about 50 percent of cases with small-cell carcinoma of the lung. The same increment occurs in botulism. In both instances there is an electromyographic (EMG) equivalent a rapid increase in the voltage of a series of action potentials upon appropriate stimulation (pages 1101 and 1259). The effect of cold on muscle contraction may also prove informative; either paresis or myotonia, lasting for a few minutes, may be evoked or enhanced by cold. This is most prominent in the paramyotonia of Eulenberg, but it may occur to some degree in all the other myotonic disorders. Myotonia and myoedema must also be distinguished from the recruitment and spread of involuntary spasm induced by strong and repeated contractions of limb muscles in patients with mild or localized tetanus, with the stiff-man syndrome (Chap. 55), and with dystonias of various types. These are not primary muscle phenomena but are neural in origin, due to an abolition of inhibitory mechanisms. The repeated contraction of forearm or leg muscles after the application of a tourniquet (exceeding arterial pressure) to the proximal part of a limb will often elicit latent tetany (carpopedal spasms). Its special mode of development in conditions that decrease the concentration of ionized calcium as well as its duration, its enhancement by hyperventilation, and the association of tingling, prickling paresthesias separate tetany from ordinary cramp and also from true physiologic contracture. In practice, the term contracture is applied (somewhat indiscriminately, as discussed below) to all states of xed muscle shortening. Several distinct types can be recognized. In true physiologic contracture a group of muscles, after a series of strong contractions,
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