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which is improved after adequate rest. The initial   be applied intramuscularly if there are any muscarinic
          symptom  is  the  ptosis  either  symmetrically  or   cholinergic receptor-mediated side effects. For children,
          asymmetrically and diplopia which are observed      neostigmine should be reduced to 0.02-0.03 mg/kg, while
          in more than 80% of patients.  In certain cases,    total amount should not be more than 1.0 mg. Test should
                                        [2]
          alternative or bilaterial ptosis and nystagmus are also   be performed at the muscle with significant symptoms
          observed. Patients suffer also from lagophthalmos.   and referred to the clinical absolute score for MG. The
          However, pupil size remains normal.                 baseline of muscle tone should be firstly recorded, which
                                                              is repeated every 10 min for 1 h. The relative score, as the
          Due to the weakness of the muscles involved in      diagnostic value, should hence be pooled by the absolute
          swallowing, there is dysphagia, difficulty of chewing   score of the trial with most significant improvement
          and velopharyngeal insufficiency. For the symptoms   according to the following formula:
          of  vocality,  patients  of  MG  have dysarthria  and
          hypophonia, some of who may also have nasality.     Relative score = (Baseline-absolute score of each
          Weakness of facial muscle leads to hanging jaw sign,   trial)/Baseline × 100%
          shallow nasolabial fold, leaky cheek blowing, snarling
          expression when smiling and sleepy or sad expression.   There is usually less than 25% of negative diagnosis
          Weakness of cervical muscle, patients could not hold   while more than 60% for positive diagnosis, and 25-60%
                                                              are as suspicious positive cases. Further tests are needed
          their upright easily. Although it is usually not the
          primary symptoms of MG, limb movement is highly     to confirm the negative diagnosis.
          affected spreading from the proximal ends. In certain   Electromyography
          cases,  the respiratory muscle is under myasthenic
          crisis which leads to dyspnea. Assisted ventilation is   Repetitive nerve stimulation
          necessarily needed to sustain life. [3,4,5]
                                                              Repetitive nerve stimulation (RNS) is an electrical
          Classification: modified Osserman scale             stimulation to nerves, such as facial, accessory,
                                                              axillary and ulnar nerves, for MG diagnosis, with
          Class I: ptosis and diplopia without other muscle   repetitive and high-powered low frequency (2-5 Hz)
          weakness elsewhere for 2 years.                     signal.  Compound  muscle action  potential  (CMAP)
                                                              over the testing muscles will be recorded.
          Class II: generalized symptoms with more than
          one set of weak muscle: (1) mild generalized form;   The duration of stimulation is about 3 s. The
          Weakness in limbs with or without ocular signs,     decrement of CMAP of MG is measured by comparing
          but without  prominent  bulbar  signs.  Patients  could   the CMAP value of the fourth or fifth stimuli to that
          live independently; (2) moderate generalized or     of the first stimulus. Diagnosis will be concluded as
          faciopharyngeal form; symptoms as in II A but with   positive when there is more than 10% reduction. MG
          bulbar signs. Patients could live independently.
                                                              patients on acetylcholinesterase inhibitor medication
                                                              should not receive this test until 12-18 h of washing
          Class III:severe acute generalized form; acute onset
          and rapid development. Faciopharyngeal symptoms     out. For diagnosing presynaptic lesions, frequency of
                                                              RNS should be increased to 10-20 Hz. Increment with
          were observed in the  first few weeks to months
          followed by respiratory insufficiency with or without   more than 100% should be classified as abnormal.
          ocular signs. Patients could live independently.    Single fiber EMG

          Class IV:severe chronic generalized form; unapparent   Single fiber EMG (SFEMG) is to measure the variable
          signs  at  early  stage due to  the slow  pathological   latency of the single axon innervation to the muscle
          progress. All symptoms stated in Class I, II and III are   fibers, known as Jitter. The variable latency is usually
          developing within 2 years.
                                                              about 15-35 s, of which more than 55 ms will be
                                                              classified as increased variability of latency. Two or
          Class V: muscle atrophy form; severe development
          with also muscle atrophy within 6 months.           more variable latency in every 20 Jitters of a single set
                                                              of muscle will be classified as abnormal. Any block
          EXAMINATION                                         during SFEMG should also be classified as abnormal.
                                                              Despite of the  significant sensitivity, SFEMG is not
          Methyl sulfate neostigmine test                     specific for MG, which is mainly for Class I MG
                                                              and  those cases  without positive outcomes  in  RNS
          Intramuscular  administration of  1.0-1.5 mg methyl   test. Furthermore, SFEMG is not affected by any
          sulfate neostigmine for adult. Atropine (0.5 mg) could   acetylcholinesterase inhibitors.


            2                                                 Neuroimmunol Neuroinfammation | Volume 3 | Issue 1 | January 20, 2016
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