Peripheral motor disorders- neuromuscular disorders

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paeds-neurology Mind Map on Peripheral motor disorders- neuromuscular disorders, created by v.djabatey on 12/01/2014.
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Mind Map by v.djabatey, updated more than 1 year ago
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Created by v.djabatey over 10 years ago
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Resource summary

Peripheral motor disorders- neuromuscular disorders
  1. any part of the lower motor pathway can be affected by a neuromuscular disorder
    1. ant horn cell disorders
      1. peripheral neuropathies
        1. disorders of neuromuscular transmission
          1. primary muscle disease
          2. clinical features
            1. key feature
              1. weakness
                1. progressive or static
              2. floppiness
                1. delayed motor milestones
                  1. muscle weakness
                    1. distal wasting & weakness
                      1. esp in presence of pes anserinus
                        1. suggests hereditary motor sensory neuropathy
                    2. unsteady/abnormal gait
                      1. fatiguability
                        1. increasing fatiguability throughout the day
                          1. often w/ ophthalmoplegia & ptosis
                            1. suggests depletion at the motor end plate
                              1. i.e. myasthenia gravis
                        2. muscle cramps
                          1. suggesting a metabolic myopahty
                          2. Gower's sign
                            1. suggestive of proximal mm weakness
                              1. = the need to turn prone to rise from supine to standing
                                1. kids climb up the legs with the hands
                                2. normal til 3 years old
                              2. ant horn cell
                                1. signs of denervation
                                  1. weakness
                                    1. fasciculation
                                      1. wasting
                                      2. neuropathy
                                        1. often distal nerves affected
                                          1. motor ->
                                            1. weakness
                                              1. w/ loss of reflexes
                                            2. sensory ->
                                              1. impaired perception of pain, temp, or touch, w/ loss of reflexes
                                            3. neuromuscular junction
                                              1. as end-plate ACh stores become depleted, there is diurnal worsening through the day-> fatigualbility
                                              2. Ix
                                                1. myopathy
                                                  1. serum creatine phosphatase
                                                    1. markedly raised in Duchenne & Becker muscular dystrophy
                                                    2. muscle biopsy, needle or open
                                                      1. modern histochem techniques often a definitive diag
                                                      2. DNA testing
                                                        1. ID abnormal genes
                                                        2. ultrasound & MRI of mm
                                                          1. used in specialist centre to diag & monitor progress
                                                        3. neuropathy
                                                          1. nerve conduction
                                                            1. ID delayed motor & sensory conduction velocities
                                                            2. DNA testing
                                                              1. for abnormal genes
                                                              2. EMG (electromyography)
                                                                1. to differentiate myopathic from neuropathic disordes
                                                                  1. e.g. fatiguability on repetitive nerve stimulation in myasthenia
                                                                  2. use selectively in kds
                                                                    1. use fine needle electrodes
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