19-10-10

MYATHENIS GRAVIS - 1st DRAFT

3. Diagnosis and Symptomatology (common to all types of myasthenia)

3.1. Symptomatology

1. The first muscles to be affected are normally those implicated in ocular and papebral movements.
Consequently, ocular manifestations such as Palpebral ptosis (abnormal drooping of the upper lid
that may cause diplopia – double vision) are quite common among these patients (around 85% of
patients show them). It is also remarkable that pupillary reflexes are normal, although there’re no
normal vestibular movements (movements that stabilize images on the retina during head
movement by producing an eye movement in the direction opposite to head movement).

2. The main characteristic in Myastenia gravis affected patients is a

skeletal muscular or tiredness that becomes more noticeable after some
physical activity, so it gets worse along the day and better after resting.
This normally drives to a generalized feeling of weakness.

3. Muscles implicated in respiration and deglutition (the act of

swallowing) are affected in variable degrees. In case of respiratory
muscles, a limitation of inspiratory and expiratory volumes may be
noticed. When deglutition muscles are affected, the patient may
experience difficulty swallowing, nasal regurgitation of food and a
characteristic nasal voice.

* Symptoms may get worse due to infections, intoxications, menstruation and

pregnancy.
3.2. Diagnosis mechanisms

The main objective of the following diagnosis mechanisms is to prove muscular fatigue and its
reversion during rest, cold or presence of anticholinesterases drugs.

3.2.1. Physical exploration

It consists of detecting some of the most noticeable symptoms that were previously described. For
example, during office hours we may detect:
- Palpebral ptosis
- Diplopia
- Dysarthria (poor articulated speech)
- Nasal voice
- The mouth could stay open due to the fatigue of maxillary muscles

3.2.2. Electrophysiological tests

1. Repetitive ocular movements in order to increase ptosis and ophtalmoplegia (paralysis or

weakness of one or more of the muscles that control eye movement).

2. Ice application on eyelids during 2 minutes and quantify how ptosis and ophtalmoplegia change.
Thus mathod is based on the principle that cold accelerates recovering from muscular fatigue.cal
tests

3. Single Fiber Electromyography (SFEMG): uses electrodes to stimulate repetitively muscles and
evaluate muscle function. Muscle contractions that become progressively weaker may indicate MG.
It has a 99% of sensibility. This study allow us to localize the abnormality in the neuromusular union
and also differentiate a presynaptic defect form a postsynaptic defect (see fig.).
3.2.3. Pharmacological tests

1. Endrophonium test: Edrophonium chloride prevents breakdown of the neurotransmitter

acetylcholine and acts by competitively inhibiting the enzyme acetylcholinesterase, mainly at the
neuromuscular junction): it consists on the intravenous administration of 10mg of Tensilon®, and
wait until any ocular motor response (1 min.)

2. Immunological test: determination of anti-rAch antibodies in blood serum. High levels of these
antibodies may indicate MG. It has a 75-80% of sensibility on generalized Myatenia gravis.

3.2.3. Differential diagnosis

It consists on distinguishing between diseases of similar character by comparing their signs and
symptoms.

In the case of Myasthenia gravis, there are some myateniformis syndromes (syndromes that look like
the manisfestation of Myastenia gravis).

For example, there are some drug-induced myasthenias:

- MS induced by eritromicine
- MS induced by D-penicilamina: The mechanism of anti-rACh (anti-receptors of
Aceticholinesterase) antibody induction in this case is still unknown.
- MS induced by morphine
- MS induced by quinine

3.3. Prognosis

“With treatment, most individuals with myasthenia can significantly improve their muscle weakness and lead
normal or nearly normal lives. Some cases of myasthenia gravis may go into remission—either temporarily or
permanently—and muscle weakness may disappear completely so that medications can be discontinued.
Stable, long-lasting complete remissions are the goal of thymectomy and may occur in about 50 percent of
individuals who undergo this procedure. In a few cases, the severe weakness of myasthenia gravis may cause
respiratory failure, which requires immediate emergency medical care.”
(http://www.ninds.nih.gov/disorders/myasthenia_gravis/detail_myasthenia_gravis.htm)