TECNIQUES IN MYAESTHENIA GRAVIS

by Dr. Heba Raafat Lecturer of Clinical Neurophysiology Cairo University

AIM OF OUR TECHNIQUES

Exclude other disrders with evident decremental response

Proper diagnosis of Myaesthenia Gravis

OTHERS CONITIONS

Various Denervating Conditions:

Neuropathies MND Inflammatory myopathies Myotonic disorders

ELECTROPHYSIOLOGICAL MYAESTHENIA GRAVIS

Includes:

TECHNIQUES FOR

Motor & sensory nerve conductions RNS Excersice testing Routine EMG SF-EMG

SENSITIVITY OF ELECTROPHYSIOLOGICAL TECHNIQUES

RNS:

Generalized Myaesthenia: 50-75% positive Ocular Myaesthenia: always normal

SF-EMG: Almost 99% positive

However--------nonspecific

Negative SF-EMG in a weak muscle

excludes

the diagnosis of MG

Nerve Conduction studies

NERVE CONDUCTION STUDIES

Aim:

Exclude other conditions as neuropathies Exclude LEMS---- small amplitude responses Ensure normal studies-----expected in MG

NERVE CONDUCTION STUDIES

Rules: At least one motor and sensory nerve
In upper Lower extremeties

Reason:
Study the integrity of the nerve before RNS

Expectation:
Normal motor and sensory studies

Repetitive Nerve stimulation

REPETITIVE NERVE STIMULATION

Study:
Distal Proximal nerves Distal: Easier technically Example: ulnar nerve Proximal: However higher percentage of positive results Example: spinal accessory facial nerves

REPETITIVE NERVE STIMULATION

In cases of:
----- Myaesthenia gravis

-----Eaton Lambert
----- Botulism

REPETITIVE NERVE STIMULATION

Abnormalities in MG:

Normal amplitude CMAP by single stimulation

Decremental response at lower rates of stimulation Any decrement > 10% is abnormal

REPETITIVE NERVE STIMULATION

Abnormalities in Eaton-Lambert syndrome:

Low amplitude CMAP by single stimulation

A decremental response at lower rates of stimulation

An incremental response at higher rates of stimulation Increment of >200% is abnormal

Abnormalities in botulism are similar to those

TECHNICAL FACTORS IN RNS

Immobilization

Supramaximal
Temperature Acetylcholine

stimulation

Inhibitors should be stopped 3-4 hours before the study Nerve selection Stimulation Frequency Number of stimulations

Exercise Testing

EXERCISE TESTING IN NMJ DISORDERS

Postexersice

Exhaustion

Postexercise

Facilitation

POSTEXERCISE EXHAUSTION

When to do it?
Routinely Esp when RNS was normal

Technique:

Maximal exercise for 1 min. Slow RNS immediately,1,2,3,& 4 mins after Decrement becomes more marked after 2-4 mins

POSTEXERCISE FACILITATION
When to do it?
Routinely after Postexcercise Exhaustion If Postexcercise Exhaustion----- significant decremental response

Technique:
Maximal contraction for 10 secs Slow RNS should follow immediately

Expectation:
Repair of previous decrement in MG Increment in Eaton-Lambert

EMG

EMG

Aim:
Exclude other severe denervating disorders Diagnose Botulism---- signs of denervation

Technique:

Particular Attention to----weak muscles

Expectation in MG:
Normal (usually) Small short duration MUPs similar to myopathic

SF-EMG

SF-EMG
When

to do it?

Clinically

suspected M.G. with previously mentioned techniques all normal

After exclusion of other disorders by previously mentioned techniques

SF-EMG
Expectation:

Increased

Jitter blocking

Sometimes

Thank you