Ophthalmologic Manifestations of

Myasthenia Gravis

Author: Shady Awwad, MD; Chief Editor: Hampton Roy Sr, MD more...

Overview
Ninety percent of patients with myasthenia gravis (MG) develop ophthalmologic manifestations
of the disease, a disorder of neuromuscular transmission characterized by weakness and
fatigability of skeletal muscles. The basic pathology of MG is a reduced number of acetylcholine
receptors (AChR) at the postsynaptic muscle membrane brought about by an acquired
autoimmune reaction producing anti-AChR antibodies.
Two major clinical forms of MG are distinguished: ocular MG, in which the patient has strictly
ocular symptoms, and generalized MG, in which the patient develops generalized weakness. One
study found that, over a mean follow-up period of 17 years, approximately 15-17% of patients
with MG had strictly ocular symptoms.
Some evidence shows that steroids or azathioprine might prevent the conversion to generalized
MG in 75% of patients with ocular MG.[1]
Bever and coworkers reported that 82% of patients who later developed generalized weakness
did so in the first 2 years after diagnosis.[2] Hence, patients who keep having strictly ocular
symptoms for 3 or more years are unlikely to revert to the generalized aspect of the disease.
The juvenile form of the disease usually has a much more favorable prognosis than the adult
form, with a better chance of spontaneous remission.

Patient History
Ophthalmic symptoms

Among patients with myasthenia gravis (MG), 75% initially complain of ocular disturbance,
mainly ptosis and diplopia. Eventually, 90% of patients with MG develop ocular symptoms.
About 50% of patients will present solely with ocular symptoms, and about 50-60% of these
patients will progress to develop generalized disease. Ptosis may be unilateral or bilateral, and it
may shift from eye to eye.
Nonophthalmic symptoms

Oropharyngeal muscle disturbances come second in presentation, with 15% of patients first
experiencing difficulty in swallowing, talking, and chewing. Limb and trunk weakness is the

initial complaint of 10% of patients, and 85% of patients with MG develop a generalized
weakness also affecting the limb muscles.
MG can involve the respiratory muscles and lead to respiratory failure. This can sometimes be
the first presentation of the disease. Qureshi et al showed that out of 51 patients with MG and
respiratory failure, 7 (14%) had no previous diagnosis of MG.[3]

hysical Examination
Ptosis

As previously stated, ptosis can be unilateral or bilateral. The ptosis is usually most prominent on
sustained upward gaze, as shown in the image below, or on repeated eyelid closure. A subtle lid
flutter is occasionally encountered.

Increasing left ptosis developing upon sustained upward

gaze in a patient with myasthenia gravis (A through F). Note the limited elevation of
the left eye denoting superior rectus palsy (A). A initially, C after around 20 seconds,
F after 1 minute.

In cases of unilateral ptosis, the contralateral lid may assume a ptotic position upon occluding the
eye with the ptosis or lifting the ptotic lid with a finger (Herring phenomenon).
The lid twitch sign described by Cogan can be elicited by having the patient change gaze from
the downward position to the primary position.[4] The lid will be seen to overshoot in a twitch
before gaining its initial ptotic position; this is shown in the image below. A study, however,
questioned the validity of such a sign, as its sensitivity and specificity were shown to be
relatively low.[5]

Cogan sign. The patient changes gaze from the

downward position (A) to the primary position (B). Both lids are seen to overshoot in
a twitch (B) before gaining their initial ptotic position (D). In this case, the Cogan
sign is seen more obviously on the right, whereas the left lid is more ptotic.

Extraocular muscle involvement

Extraocular muscle involvement does not follow a certain pattern. However, the superior rectus
and/or the medial rectus are commonly involved. Isolated inferior rectus palsy is rare, but when
it is present, it raises the suspicion of myasthenia gravis (MG), since it is extremely rare to be
encountered otherwise.
The spectrum of extraocular muscle conditions stretches from isolated muscle palsies to total
external ophthalmoplegia. Any acquired ocular motility disturbance, with or without ptosis but
with normally reacting pupils, should raise the clinical suspicion of MG.
Orbicularis involvement

Orbicularis involvement consistently is present when ocular symptoms are reported. Weakness in
forceful closure of the eyes against resistance is present.
Pupils and ciliary muscles

Traditionally, pupils and ciliary muscles were believed not to be involved, although studies now
suggest the contrary, notably in a case analysis by Cooper and coworkers.[6]

Treatment of Ophthalmic Manifestations

A wide armamentarium of medical therapy exists for patients with myasthenia gravis (MG). No
general consensus exists about which to start first or which to combine. Medical treatment is
administered best by a neurologist. The role of the ophthalmologist is to follow up on the
ophthalmic symptoms, notably diplopia and ptosis. The neurologist orchestrates the
pharmacologic treatment.
Ptosis, when it becomes bothersome, is treated best by lid crutches, since surgery is
contraindicated because of the fluctuation of the disease, producing lagophthalmus with all of its
complications. Diplopia is eliminated by Fresnel prisms unless the deviation is large and
variable, in which case an opaque lens in front of the involved eye is preferred.
Strabismus surgery

Successful muscle surgery for selected patients with a stable course of MG and persistent
diplopia has been reported.[7, 8]
Blepharoptosis surgery

Ptosis surgery for patients with a stable ptosis that has failed to respond to medical therapy for
MG has gained popularity. The surgical technique can include external levator advancement,
frontalis suspension sling, or tarsomyectomy.[9, 10]
Consultations and follow-up

Consult neurologists after diagnosing patients with ocular MG. Treatment is initiated best by
them rather than by the ophthalmologist. Follow-up care of the patient with MG is conducted

mainly by the neurologist, who will orchestrate the treatment. The ophthalmologist is consulted
accordingly.