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initial decrement to repetitive stimulation, with a subsequent increment to the baseline CMAP at high rates of stimulation During repetitive stimulation, the afterdischarges evoked during single stimulation are absorbed into the subsequent CMAPs and are no longer observed Patients with signi cant intoxication reveal a decrement to repetitive stimulation at low rates of stimulation, while those with moderate-to-mild intoxication only reveal a decrement at rates >10 Hz With slow repetitive stimulation, edrophonium chloride administration, in contrast to MG, results in a marked worsening of the decremental response, which returns to the resting level within about 30 seconds229 Application of d-tubocurarine, an AChR-blocking agent, repairs the decrement by decreasing the number of AChRs available to the excess ACh Needle electromyographic examination does not reveal positive sharp waves or brillation potentials during the initial stages of the disease The only abnormality is a reduction in the number of voluntary MUAPs In patients who develop an axonal neuropathy as a result of delayed or chronic organophosphate exposure, a typical length-dependent EDX neuropathy pattern eventually develops In patients given nondepolarizing neuromuscular blocking agents, there is a reduced CMAP that is expected to decrement with slow repetitive stimulation The results are similar to those observed in MG The MUAP can be seen to decline in magnitude as single muscle bers belonging to a particular motor unit serially block with increasing dosages of curare415 As might be expected with SFEMG analysis, doses of curare that do not produce weakness may nevertheless produce an increase in jitter values416

pletely normal To date, there have been no convincing morphological changes of the NMJ attributed to MuSK antibodies Muscle biopsies in these latter patients have demonstrated minor simpli cation of some end plates The density and distribution of AChR in patients with MuSK are essentially normal in the studies reported to date Complement deposition or other markers of an immune-mediated disease has not been identi ed423,424

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root certificate is preinstalled onto SymbianOS-based devices by the device manufacturer The device manufacturer will also generally provide its root certificate The use of Symbian Signed is not required of manufacturers taking advantage of the SymbianOS platform; however, most do in order to take advantage of the ecosystem of signed applications The Symbian Signed program can be developed into four main categories: OpenSigned Online, Open Signed Offline, Express Signed, and Certified Signed Each of the categories enforces different requirements to obtain a signature and impose different limitations on their use Open Signed Online is the first tier of Symbian Signed Anyone can get their application signed by providing an International Mobile Equipment Identity (IMEI) number, an e-mail address, and the SIS installation package Open Signed Online can be used to grant any User and System capabilities This will be signed by a developer certificate Upon installation, the user is informed that the application was signed by a developer certificate and asked whether they would like to continue Further, the package that it is being installed on the phone specified will be validated based on the IMEI number provided during signing Open Signed Offline provides a developer certificate that can be used to sign multiple SIS installation files that can generally be used on up to 1,000 devices by IMEI number This certificate can be used to grant any User and System capabilities, although installation will still be confirmed by the end user due to the use of a developer certificate Obtaining this certificate requires one to have a Symbian Signed account as well as to have purchased a valid Publisher ID A special Open Signed Offline certificate can be requested that also grants restricted and manufacturer capabilities by sending the request to the device manufacturer Installers signed by this certificate can only be used on a phone with an IMEI number that matches one of the 1,000 recorded numbers Express Signed is the first tier intended for end-user release Installation packages signed through the Express Signed program are valid for ten years and can be installed onto any device Express Signed applications can use both User- and System-level capabilities Applications must meet the Symbian Signed Test Criteria, although independent testing house validation is not required All Express Signed applications may be audited to ensure that they meet these criteria In order to participate in the Express Signed process, one needs a Symbian Signed account, a valid Publisher ID, and the purchase of one Content ID per application submission Certified Signed is the final tier intended for end-user release Installation packages signed at this level have full access to User, System, and Restricted capabilities Applications can petition for access to Manufacturer capabilities Certified Signed applications must meet the test criteria, as confirmed through independent testing.

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The histologic changes of the CMS are, in large part, limited to those detectable by ultrastructural analysis This evaluation is a very specialized undertaking done at a limited number of centers Light microscopic analysis of muscle is not routinely performed in patients with suspected CMS In most of these disorders, routine muscle biopsy ndings are normal or nonspeci c in nature

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