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