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Peripheral motor disorders- neuromuscular disorders
Descripción
paeds-neurology Mapa Mental sobre Peripheral motor disorders- neuromuscular disorders, creado por v.djabatey el 12/01/2014.
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paeds-neurology
Mapa Mental por
v.djabatey
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v.djabatey
hace más de 10 años
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Resumen del Recurso
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
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