zxing read barcode example java 2 DIAGNOSTIC YIELD OF TESTS COMMONLY USED IN THE DIAGNOSIS OF MYASTHENIA GRAVIS in Objective-C

Creator Data Matrix in Objective-C 2 DIAGNOSTIC YIELD OF TESTS COMMONLY USED IN THE DIAGNOSIS OF MYASTHENIA GRAVIS

TABLE 23 2 DIAGNOSTIC YIELD OF TESTS COMMONLY USED IN THE DIAGNOSIS OF MYASTHENIA GRAVIS
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AChR Binding (%) Blocking (%) 19 30 52 66 52 Modulating (%) 75 72 89 91 86
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Distal Rep Stim (%)
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Prox Rep Stim (%)
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SFEMG (%)
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Remission Ocular Mild generalized Moderate severe Generalized All
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81 71 88 93 86
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45 50 76 96 99 62 77
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59 63 91 99 86 92
AChR, antiacetylcholine receptor; SFEMG, single ber electromyography With permission from Dumitru D, Amato AA Neuromuscular junction disorders In: Electrodiagnostic Medicine, 2nd edn, 25 Philadelphia: Hanley & Belfus, 2002, p 1165
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SECTION II
SPECIFIC DISORDERS
CONGENITAL MYASTHENIC SYNDROMES
The evaluation of a patient with a suspected CMS can be a specialized undertaking that exceeds the capabilities of most institutions AChR antibodies, anti-voltage-gated calcium channel antibodies, and potentially other serological tests to address and exclude autoimmune DNMT should be considered Demonstrating a decremental response to slow repetitive stimulation will support the existence of a DNMT It will not, however, distinguish CMS from other NMT disorders, nor will the absence of a decrement exclude a CMS from consideration Identi cation of an afterdischarge(s) as described in 2 (Fig 23 4) is not speci c for AChE de ciency or the slow channel syndrome but is very suggestive in the appropriate clinical context MUAP variability may be dif cult to demonstrate in an uncooperative child and has the same limitations as a slow repetitive stimulation SFEMG, a sensitive but nonspeci c test, is usually reserved for patients in whom a decremental response cannot be demonstrated In children, SFEMG is usually performed by the stimulated rather than the voluntary technique Ultimately, the identi cation of the existence and likely subtype of CMS may depend on a very specialized testing, which includes in vitro electrophysiological analysis such as patch-clamp experiments on single AChR channels, ultrastructural and immunocytochemical synaptic imaging, and potential genetic analysis in research laboratories
onds, are small, and can be easily overlooked These afterdischarges may decrement or disappear with repetitive stimulation delivered at the rates of 02 and 2 Hz A decremental response may occur at both slow (eg, 2 Hz) and fast (eg, 40 Hz) rates of stimulation The administration of edrophonium does not alter the decrement Routine needle electromyographic examination demonstrates MUAP instability Abnormal spontaneous activity is absent
AChR De ciency
Routine conduction studies are normal322 327 Repetitive stimulation at 2 3 Hz reveals a decremental response There is no abnormal spontaneous activity SFEMG analysis demonstrates increased jitter and blocking
Primary Kinetic Defect with Slow Channel Syndrome
The magnitude of the CMAP may be normal or slightly reduced, depending on the severity of the disease Afterdischarges as in AChE de ciency may occur 165 167 Following a brief voluntary contraction, this afterdischarge disappears only to return following a brief rest period A decrementing response to slow repetitive stimulation is usually observed only in clinically weak muscles Routine needle electromyographic analysis reveals MUAPs with variable amplitudes, polyphasia, and decreased durations, which may suggest a myopathic disorder Rarely, positive sharp waves, brillation potentials, and fasciculation potentials can be observed
Electrophysiology Choline Acetyltransferase De ciency
A decremental response to slow repetitive stimulation may be demonstrated in proximal muscles and is more likely to occur following serial trains of stimuli delivered after 1 minute or more of exercise Motor unit variability may be seen on needle electromyography No abnormal spontaneous activity is expected
Primary Kinetic Defect with Fast Channel Syndrome
Repetitive stimulation demonstrates a mild decrement with 3 Hz stimulation
Dok-7
A decremental response to slow repetitive stimulation is seen in the majority of cases188 The anticipated ndings in familial limb-girdle MG are also essentially identical to autoimmune MG Sensory and motor nerve conductions are normal, decremental responses are common in response to slow repetitive stimulation in clinically weak muscles, small and unstable MUAPs are demonstrable on needle EMG, and increased jitter values and blocking are found on SFEMG125 128,130,131 Fibrillation potentials and positive waves have not been reported
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