2. PLAN OF THE TALK
Physiology of Neuromuscular junction
Procedure, technical aspects
Interpretation
Application in various conditions
3. RNS-TECHNIQUE
RNS study is one of the most useful electro
diagnostic test for diagnosing pre- and postsynaptic
neuromuscular transmissions.
4.
5. DEFINITIONS
Quantum. A quantum is the amount of Ach
packaged in a single vesicle.
Each quantum (vesicle) 1 mV change of
postsynaptic membrane potential.
Rest, MEPP
The number of quanta released after a
nerve action potential depends on the
number of quanta in the immediately
available (primary) store and calcium stores
Normally 50-300(60) vesicles
6. DEFINITIONS
End plate potential -EPP is the potential
generated at the postsynaptic membrane
following a nerve action potential and
neuromuscular transmission.
60 mV change in the amplitude of the membrane
potential.
Safety factor. The safety factor of neuromuscular
transmission is simply defined as the difference
between the EPP and the threshold potential for
initiating an action potential.
MFAP
CMAP
7. CALCIUM AND QUANTA DYNAMICS
calcium :diffuses slowly out of the presynaptic terminal in
100–200 msec.
Ach stores: immediately available (primary) store and
secondary (or mobilization) store
Inter stimulus interval
rapid RNS (more than every 100 msec, or stimulation
rate >10 Hz), calcium influx is greatly enhanced and the
probability of release of Ach quanta increases.
8. RNS-TECHNIQUE
RNS is performed on selected motor nerves with
recording by surface electrodes.
G1-motor point,G2-tendon
Supramaximal stimulus
Initial sharp negative deflection
9. MUSCLE SELECTION
Clinically weak muscles should be selected.
Usually facial and proximal limb muscles shows greater
abnormality than distal muscles.
Cholinesterase inhibitors should be stopped 12-24 hrs
before.
11. IMPORTANT POINTS:
Immobilization and isometric electrode position.
Supramaximal stimulus
Temperature must be controlled
AchI withheld 4 hrs prior to study.
Proximal site better than distal site
Distal easy; proximal difficult.
12. STIMULUS TECHNIQUE
Best at 3-5 hz
Decrement increases with stimulus rates up to
10hz.
Higher rates cause movement artifacts and painful
Pseudo facilitation
13. LOW RATE RNS
supra maximal CMAP
3–5 stimuli to a mixed or motor nerve at a rate of
2–3 Hz.
slow enough to prevent calcium accumulation,
high enough to deplete the quanta
maximal decrease in Ach release occur after the
first four stimuli
reproducible decrement
exercises for 10 seconds to demonstrate repair
of the decrement (‘‘post-exercise facilitation’’)
No decrement-1 minute max voluntary exercise –
post exercise exhaustion
15. HIGH RATE RNS
optimal frequency is 20–50 Hz,for 2–10 seconds
brief (10-second) period of maximal voluntary
isometric exercise has,the same effect as rapid
RNS
Depletion of quanta vs calcium accumulation
Incremental response in LEMS
17. RNS IN PRE AND POST SYNAPTIC DISORDERS
Parameter Pre-synaptic Post-synaptic
CMAP amplitude Small Normal
Low rate RNS Decrement Decrement
High rate RNS Increment Decrement or
normal