C L I N I C A L A N D E X P E R I M E N TA L
CLINICAL COMMUNICATION
Vision therapy may reveal symptoms of ocular myasthenia
gravis
Clin Exp Optom 2019
Hanna Buczkowska PhD
Andrzej Michalski PhD
Marcin Stopa DSc
Department of Ophthalmology and Optometry,
Heliodor Swiecicki University Hospital, Poznan
University of Medical Sciences, Poznan, Poland
E-mail: hannabuc@ump.edu.pl
Submitted: 21 August 2018
Revised: 6 June 2019
Accepted for publication: 17 June 2019
Key words: binocular vision, diplopia, ocu-
lar myasthenia gravis, ptosis, vision therapy
Myasthenia gravis is a severe, treatable
autoimmune disorder of the neuromuscular
junction, with 80–90 per cent of cases
having detectable auto-antibodies to the α
subunit of the acetylcholine receptor.1
Myasthenia gravis is characterised by alter-
nating weakness and fatigability, primarily in
muscles innervated by the cranial nerves,
but may also involve skeletal and respira-
tory muscles.1 Ocular myasthenia gravis is
one of the subtypes of myasthenia gravis
and involves weakness of the levator
palpebrae superioris, orbicularis oculi and
extraocular muscles. Initial symptoms of
ocular myasthenia gravis typically involve
intermittent ptosis and diplopia that fluctu-
ates during the day, and does not follow
any particular pattern, except that it is usu-
ally incommitant. The pupil and ciliary mus-
cle should not be affected. However, 50–80
per cent of patients with ocular myasthenia
gravis subsequently develop a generalised
condition.2
The diagnosis of ocular myasthenia gravis
can be made according to patient history,
clinical examination, and specific diagnostic
tests.
Ocular myasthenia gravis is diagnosed
based on: (1) functional methods; (2) phar-
macological testing; and (3) laboratory
© 2019 Optometry Australia
investigations. The functional category of
methods for diagnosis contains the photo
review test, sleep test and ice-pack test. The
pharmacological testing group includes the
edrophonium test and neostigmine test.
Electrophysiological testing (single-fibre elec-
tromyography and repetitive nerve stimula-
tion), immunologic testing, and imaging
studies belong to the third category of diag-
nostic examinations.
Functional tests
Functional tests are simple but without high
specificity and sensitivity. The photo review
test is based on viewing previous pictures of
the patient, and then pictures taken during
the diagnostic period early in the morning
and late in the evening for three days. On
all pictures the lid position is checked using
a magnification lens.3 The sleep test is per-
formed when the patient sleeps for
30 minutes during the day or evening, and
is consequently evaluated for a decrease of
ptosis.3 The ice-pack test is conducted by
placing an ice pack on the ptotic eyelid for
two minutes. Patients with ocular myasthe-
nia gravis demonstrate improvement in eye-
lid position, where two or more millimetres
are considered as a positive test result.4
Pharmacological testing
To confirm ocular myasthenia gravis, a ten-
silon (edrophonium chloride) test may be
performed, which will be positive in more
than 90 per cent of patients. In this case,
10 ml of solution are injected and in a myas-
thenic patient ptosis or eye-muscle function
will immediately improve.3
Another pharmacological test is per-
formed with the use of 1.5 mg of neostig-
mine where the patient’s response is
slower; however, it lasts longer.2
Electrophysiological testing
Electrophysiological testing includes single-
fibre electromyography – a very sensitive
clinical test of neuromuscular transmission
DOI:10.1111/cxo.12946
(up to 88 per cent of patients with ocular
myasthenia gravis) – which shows an
increased activity of some muscles.3 Another
electrophysiological test is repetitive nerve
stimulation; during this examination a chosen
nerve is stimulated 6–10 times at a slow rate
with a supramaximal stimulus, and the com-
pound muscle action potential is measured,
which in myasthenia gravis is reduced.2
Laboratory testing
Immunologic testing is based on anti-
acetylcholine receptor antibodies detection,
which have been demonstrated in 30–77
per cent of patients with ocular myasthenia
gravis.2 Imaging of the thymus (computed
tomography or magnetic resonance imag-
ing) is necessary to investigate possible
presence of a thymic hyperplasia or
thymoma.2,3
In addition to ocular myasthenia gravis,
the differential diagnoses of sudden-onset
binocular diplopia should include cranial
nerve palsy, trauma, thyroid dysfunction,
myositis, decompensating phoria and rare
causes like metastases to the orbit or
migraine.5 Ptosis may be induced by severe
neurological causes like sclerosis multiplex
or be associated with isolated palpebral
conditions like chalazion or injury. Consider-
ing the differential diseases, neurological
and endocrinological diagnosis has to be
performed. Specifically, thyroid dysfunction
must be ruled out because there is a higher
prevalence of an associated autoimmune
thyroid disease among patients with ocular
myasthenia gravis, and both of them are
associated with eye or lid position
disorders,6 and with lack of convergence
(Moebius’ sign).7 Herein we describe a case
that may shed new light on identification.
Case report
A 19-year-old Caucasian female presented
as a referral after optometric examination
Clinical and Experimental Optometry 2019
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Vision therapy and ocular myasthenia gravis Buczkowska, Michalski and Stopa
to our vision therapy office to eliminate peri-
odic double vision, which intensified in the
afternoon. The patient wore soft contact
lenses: right eye −3.25 DS, left eye −2.75
DS. The patient denied any systemic condi-
tions and did not report any problems of
the visual system except for intermittent
double vision. A neurologist had been previ-
ously consulted because of the diplopia and
had ruled out multiple sclerosis. On the ini-
tial eye examination, the left eye was domi-
nant at distance and near. Eye movements
were full in the nine directions of gaze. The
cover test revealed eso-deviation at distance
and near (esophoria passed periodically into
tropia). The near point of convergence was
2 cm break and 3 cm recovery. The objec-
tive refraction (autorefraction) without
cycloplegia was right eye −3.50/−0.50 × 12;
left eye −4.00/−0.50 × 167, whereas the
subjective refraction was right eye −2.75/
−0.50 × 5; left eye −3.75/−0.50 × 175. Cyclo-
plegic refraction was right eye −2.50/
−0.50 × 7; left eye −3.50/−0.50 × 171. Her
distant and near visual acuity with the sub-
jective correction was right eye 6/6, left eye
6/6, binocular 6/6. All the following tests
were performed with correction consistent
with the subjective result. ‘Worth dot’ testing
in the dark at distance and near revealed
intermittent horizontal diplopia. Eye align-
ment (evaluated by von Graefe technique)
was 15 Δ base-out at distance and 13 Δ
base-out at near, but these results were
unstable. Smooth vergence testing was used
for assessing fusional vergence ranges and
the results were as follows: distance base-
out blur and break 2 Δ, recovery 1 Δ; base-
in break 4 Δ, recovery 4 Δ (base-out) respec-
tively. These results indicated that there was
a problem with recovery after the fusion
break. Smooth vergence at near was base-
out blur and break 30 Δ, recovery 20 Δ;
base-in blur 8 Δ, break 16 Δ, recovery 8 Δ.
These values were below Morgan’s norms.8
Gradient AC/A ratio was 3:1 (norm 4:1  2).8
Monocular estimation method retinoscopy
for assessment of accommodative response
was right eye +0.50 D, left eye +0.50 D.
Accommodative amplitude (push-up method)
was normal (right and left eye 11 D) in the
first attempt of examination, but after re-
measuring the results were worse (right and
left eye 10 D). The negative relative accom-
modation was within normal limits (+2.50 D).
In contrast, the positive relative accommoda-
tion was reduced at −0.75 D. Accommodative
facility with 2.00 D flippers, performed
monocularly at 40 cm, was normal, but
Clinical and Experimental Optometry 2019
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binocularly it was lowered (especially with
minus lenses). Stamina and dynamics of
the accommodative response decreased
over the time of the examination. Based on
the tests performed, our preliminary diag-
nosis was eso-deviation (basic esophoria
passed periodically into tropia).
The patient was offered spectacle correc-
tion right eye −2.75/−0.50 × 5, left eye
−3.75/−0.50 × 175, in-office vision therapy
once a week (for fusional vergence improve-
ment) and exercises 15 minutes a day, six
days a week at home (Brock string, Brock
string and base-in prism, eccentric rings).
After three weeks of vision therapy a con-
trol examination took place. Both the first
and the second diagnostic visits were car-
ried out in the afternoon.
Measurements of phoria and fusional
vergences were comparable to the results
obtained at the first visit. Subjectively, the
patient reported that the impression of dou-
ble vision was less intense during the day.
However, she noticed disturbing symptoms
of relapsing ptosis of the upper lid in the
dominant eye a few days before the control
visit (afternoons, mainly after performing
the vision therapy). Therefore, additional
ophthalmological and neurological consulta-
tion was immediately recommended.
Further ophthalmologic examination indi-
cated that in each eye the cornea and lens
were clear and anterior chamber depth was
3.5 mm. The optic discs were pink with
sharp margins and round cup-to-disc ratios
of 0.3. There were no lesions or pigmentary
changes in the macula or periphery. The
fovea exhibited a sharp light reflex. The vas-
culature was normal. In contrast, the left
eyelid ptosis was noted with a 3 mm differ-
ence in the height of the palpebral fissure
between the eyes. Based on these findings
and symptoms, the ophthalmologist consid-
ered ocular myasthenia gravis and referred
the patient to neurology. Results of brain
coherence tomography and magnetic reso-
nance angiography were normal. Thyroid
dysfunction was a differential diagnosis but
ruled out by normal serology results of
thyroid-stimulating hormone (2.151 nIU/ml)
and free thyroxine (1.96 ng/dl) results.
Finally ocular myasthenia gravis was
confirmed by a positive result on single-
fibre electromyography. Pyridostigmine
bromide 60 mg four times daily orally was
prescribed for treatment. Diplopia and pto-
sis subsequently subsided after pharmaco-
logical treatment and the patient was
functioning well.
Discussion
The most striking observation to emerge
from this case is that the increased physi-
cal effort of the extraocular muscles as a
result of vision therapy could have
influenced the manifestation of ocular
myasthenia gravis. However, further
research is needed to confirm this posi-
tion because apart from vision therapy,
the correction of refractive errors had also
been changed.
Furthermore, ptosis induction during
vision therapy could be a factor, which
facilitates or accelerates an ocular myas-
thenia gravis diagnosis when other symp-
toms were vague. Interestingly, in our
case, ptosis primarily affected the domi-
nant eye. Upon a literature review, the
authors did not find similar observations
regarding vision therapy for diplopia and
noted changes in ptosis. However, there
was a report of transient dysfunction of
accommodation and vergence aggravated
by vision therapy. We believe further stud-
ies need to be carried out to establish
whether dominance matters regarding
which eye will be affected by ptosis first in
patients with ocular myasthenia gravis.3
We found that the accommodative range
of the patient was slightly reduced on sub-
sequent testing, even though it was within
normal limits on the initial examination.
Similar results to our outcomes were pre-
viously noted.3,9 Cooper et al. emphasised
that accommodative insufficiency could be
an early and typical sign in ocular myas-
thenia gravis with a specific pattern –
decrease of response with subsequent
cycles.10 Therefore it is necessary to
repeat tests of accommodation and con-
vergence in patients with suspicion of ocu-
lar myasthenia gravis. In people without
ocular myasthenia gravis, we expect that
the results of binocular vision or accom-
modation will be more comparable as the
tests are repeated. It is extremely impor-
tant to monitor the functioning of the
visual system in patients with ocular
myasthenia gravis, especially at the begin-
ning of treatment (lid and pupil assess-
ment, phorias, vergences, accommodation). It
seems reasonable to perform examination
with similar conditions and tests as at the first
visit, so as to be able to monitor in a more
reliable way the potential changes in the func-
tioning of the visual system. Visits should be
performed every 6–12 months depending on
© 2019 Optometry Australia

the patient’s condition and severity of
findings.
The authors feel that ocular myasthenia
gravis should always be included in the dif-
ferential diagnosis in young patients with
diplopia. Early diagnosis is an advantage
and practitioners should keep an open
mind because diagnostically critical obser-
vations may be revealed at any subse-
quent examination. In our case, symptoms
and signs that led to the diagnosis of ocu-
lar myasthenia gravis became apparent at
follow-up.
© 2019 Optometry Australia
Vision therapy and ocular myasthenia gravis Buczkowska, Michalski and Stopa
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Clinical and Experimental Optometry 2019