SURVEY OF OPHTHALMOLOGY VOLUME 47 • NUMBER 3 • MAY–JUNE 2002

CLINICAL CHALLENGES
PETER J. SAVINO AND HELEN DANESH-MEYER, EDITORS

Ahhh, That’s A Strange Eye Movement

Helen V. Danesh-Meyer, FRACO

Discipline of Ophthalmology, University of Auckland, Auckland, New Zealand

Comments by Owen B. White, MD, PhD, FRACP

(In keeping with the format of a clinical pathologic conference,

the abstract and key words appear at the end of the article.)

A video presentation that effectively illustrates the
eye movements discussed in this article has been cre-
ated. Throughout the text, readers will be instructed
to refer to the images on the video. Please log on to
Survey of Ophthalmology’s Web page at http://www.
Elsevier.com/locate/survophthal to view the video.
Case Report. A 57-year-old woman noted the onset
of horizontal diplopia after surgical repair of a rup-
tured posterior fossa aneurysm 4 years prior to pre-
sentation. The diplopia showed no diurnal variability.
Visual acuity was 20/60 OD and 20/25 OS with
no improvement with pinhole in either eye. Her
pupils were equal, reactive to light, with no relative
afferent pupillary defect. Visual fields were full to
finger counting techniques. Slit-lamp examination
was unremarkable OU. There was no proptosis. Oc-
ular motility examination revealed a left esotropia
of 50 prism diopters and a right hypertropia of 10
prism diopters in primary position. No horizontal
eye movements could be made with the right eye,
no abduction and only limited adduction was possi-
ble on the left. Vertical eye movements were full.
She had a face turn of 30 degrees when fixating
with her better left eye, and no face turn when fix-
ating with her right eye. The patient had some ver-
tical oscillations of 5 degrees with a torsional com-
ponent. She also showed marked bilateral VIIth
nerve palsies.
SHOW FIRST PART OF VIDEO HERE
Where is the site of the lesion? What is the cause
of the diplopia? Are any further investigations rec-
ommended?
Comments
Comments by Owen B. White, MD, PhD, FRACP,
Kingston Centre Oculomotor Laboratory and Mo-
nash Institute for Neurological Diseases, Southern
Health Care Network, Clayton, Australia
This 57-year-old woman was apparently neurologi-
cally intact, at least from the oculomotor point of
view, prior to the aneurysm surgery. Subsequently,
she had marked impairment of ocular motility, the
salient features of which are the following:
1. Failed Horizontal Movements of the Right Eye
and Failed Abduction of the Left Eye With Limited
Spared Adduction
If we reconstruct the ophthalmoparesis in terms
of conjugate eye movements, there is absolute gaze

263
© 2002 by Elsevier Science Inc. 0039-6257/02/$–see front matter
All rights reserved. PII S0039-6257(02)00283-7
264 Surv Ophthalmol 47 (3) May–June 2002
palsy to the left, associated with limited adduction of
the left eye and an abduction failure of the right eye.
These findings argue for a significant lesion of the
left paramedian pontine reticular formation, caus-
ing the gaze palsy, in association with a right sided
lesion affecting the fascicular abducens nerve, possi-
bly at the level of the VIIth nerve nucleus, but proba-
bly more anterior. There is some sparing of the me-
dian longitudinal fasciculus fibers originating from
the right pontine gaze center, and passing superiorly
via the MLF to the left IIIrd nerve nucleus, permit-
ting some left eye adduction.
Thus, the clinical findings so far would indicate a bi-
lateral, but asymmetric, lesion of the anterior pons at
the level of the VIIth nerve nucleus and the VIth nerve as
it traverses the pons to its exit point. This is restricted
to the anterior half of the pontine tegmentum. The pa-
thology would be more substantial on the left side.
These findings would be consistent with midline
perforator vessel injury at the time of the surgery.
2. Right Hypertropia Consistent With Cranial Nerve
IV Palsy or a Skew Deviation
A skew deviation is a relatively comitant small ver-
tical deviation that usually remains constant in all
gaze positions and is rarely greater than 10 prism di-
opters. When the vertical deviation is worse on the
right or left gaze, it may mimic a fourth nerve palsy.
However, the Bielschowsky head-tilt test is usually
negative and no cyclotorsion is measured on double
Maddox rod testing. A skew deviation is usually asso-
ciated with some other signs of brainstem injury. It
can be caused by lesions anywhere in the midbrain,
pons, or medulla. Skew deviation is probably a supra-
nuclear disorder arising from imbalance of otolith in-
puts that cross in the medulla and ascend in the me-
dial longitudinal fasiciculus.
We are not provided measurements of the ocular
misalignment except in primary position for this pa-
tient. With the limited horizontal gaze, it may not be
possible to distinguish between these two entities.
3. Full Vertical Eye Movements
The preservation of vertical eye movements would
indicate that the primary vertical ocular motor path-
ways, the rostral interstitial nucleus of the median lon-
gitudinal fasciculi (riMLF), are substantially intact.
Vertical eye movements are controlled from the
upper midbrain reticular formation, including the
riMLF and the interstitial nucleus of Cajal. Impor-
tant inputs to this area are the bilateral medial longi-
tudinal fasciculi, which carry signals from the pon-
tine gaze centers more rostrally. The presence of
some sparing of vertical eye movements does imply
that there is some sparing of these important com-
mand structures.
DANESH-MEYER
4. Rhythmic Oscillatory Movements
The patient has two distinct vertical eye move-
ment abnormalities. The rapid upward deviation of
the eyes is more difficult to define. It is possible it
represents Bell’s phenomenon in a patient with bi-
lateral facial paralysis. From the video, however, there
would appear, on occasion, to be some spared eye
closure. Eye closure or attempted eye closure is not
seen with the rapid upward movements. Moreover,
the rapid upward movements occur in rapid succes-
sion on several occasions and without apparent at-
tempts at eye closure. Under the circumstances, I am
not convinced that it represents an exaggerated
Bell’s phenomenon, although I would acknowledge
this as possible. Some of the vertical movements
seem rapid and may be saccadic, similar to the verti-
cal movements seen in reverse ocular bobbing.
Typically, ocular bobbing consists of intermittent,
usually conjugate, rapid downward movement of the
eyes followed by a slower return to the primary posi-
tion. Reverse bobbing then, is rapid deviation of the
eyes upward and a slow return to the horizontal. Re-
verse bobbing is usually observed in patients who are
unconscious with significant pontine pathology and
disruption of the reticular formation.3,6,7,9
The pathophysiology of the “bobbing/dipping”
syndromes is poorly understood. Ocular bobbing
and reverse ocular bobbing are signs of intrinsic
pontine lesions, usually hemorrhage, but they also
may occur with cerebellar lesions that compress the
pons and in metabolic or toxic encephalopathies.
The eye signs themselves are not of specific localiz-
ing value, but the fact that several types of these eye
movements can be seen in the same patient at differ-
ent times suggests they share common pathophysio-
logical mechanisms. These vertical movements are
commonly associated with loss of horizontal eye
movements, reflex, and, otherwise, implying ante-
rior pontine lesions on each side with sparing of the
vestibular nuclei and the vertical pathways, the me-
dian longitudinal fasciculi. However, in the light of
the seemingly quite variable velocity characteristics,
no definitive conclusion can be drawn.
The second spontaneous vertical eye movements
represents another entity. The next diagnostic test I
would perform to determine the cause of this motil-
ity disturbance is to inspect the patient’s palate.
SHOW SECOND PART OF VIDEO
The synchronous movement of the palate with the
eyes establishes this second movement as oculopalatal
myoclonus. In recent times, this movement has more
correctly been described as palatal tremor,1 there not
being any true evidence of myoclonus in the move-
AHHH, THAT’S A STRANGE EYE MOVEMENT
ment abnormality. For reasons of familiarity, I will
continue to refer to it as “oculopalatal myoclonus.”
Oculopalatal myoclonus presents as vertical ocu-
lar oscillations at a rate of approximately 2 Hz. This
condition usually develops several months after
brainstem or cerebellar infarction, although it may
not be recognized until years after the precipitating
event. Although the palate is most often affected,
movements of the eyes, facial muscles, pharynx,
tongue, and other structures may occur. Tardive cer-
ebellar ataxia may also occur.2 The ocular move-
ments of oculopalatal myoclonus typically consist of
pendular oscillations that are usually vertical, al-
though they may have a small horizontal or torsional
component. If the lesion is primarily unilateral, it
can be associated with torsional movements, the ipsi-
lateral eye intorting as it rises. The eye oscillations
may be disconjugate, both horizontally and verti-
cally. The nystagmus sometimes disappears with sleep,
but the palatal movements usually persist. Once es-
tablished, the condition is permanent.
Localization is a little diffuse, but it would appear
to be associated with hypertrophy of the inferior oli-
vary nucleus, as demonstrated by MRI,1 and is seem-
ingly the product of lesions either in the region of
this nucleus, the dentate nucleus of the cerebellum,
or the red nucleus. These three structures together
are referred to as the myoclonic or Mollaret’s trian-
gle. As such, it does not help to localize the lesion
precisely, but it is certainly consistent with the local-
ization outlined above.4,5,8,9
The actual mechanism of generation, likewise, is
uncertain. It has been postulated that projection fi-
bers to the inferior olivary nucleus from the con-
tralateral dentate nucleus may be interrupted in
their passage via the region of the red nucleus and
the central tegmental tract.5,8 It is tempting, how-
ever, to consider the possible role of the riMLF and the
interstitial nucleus of Cajal in the production of this
phenomenon. These nuclei and tracts are involved
in the processing of vertical eye movement signals
and otolith signals intimately involved in the vertical
stabilization of the eyes in the head, and the ocular
counter-rolling reflexes active during head tilt.4
Oculopalatal myoclonus has been reported as be-
ing associated with ocular bobbing and horizontal
diplopia. It has also been reported as occurring in
conjunction with bobbing in acute brainstem le-
sions. This finding is perhaps a little unusual as it is
generally believed that oculopalatal myoclonus is a
movement that develops concomitant with olivary
hypertrophy as a consequence of chronicity of de-
nervation.7 That is clearly not the case in an acute le-
sion and argues for an alternative explanation, prob-
ably involving loss if inhibition from cerebellar
inputs and thus instability of the neural integrator
265
responsible for maintenance of vertical eye position
in the orbit.
In summary, this patient presents with facial pare-
sis and ocular motor abnormalities, which strongly
suggest primary pontine pathology involving the
gaze centers, the medial longitudinal fasciculi on
one side, and possibly other tracts mediating vertical
eye movements. The location of the aneurysm itself
indicates this will be brainstem in site. The presence
of bilateral facial paresis argues strongly for anterior
pontine pathology at the level of the facial nerve.
Case Report (Continued)
The patient underwent strabismus surgery consist-
ing of transposition of the left superior and inferior
rectus to the left lateral rectus. The goal of the sur-
gery was to eliminate the head turn and allow her to
look straight ahead with the left eye, but not neces-
sarily the diplopia. Post-operatively, she no longer
had a face turn, but still had no horizontal versions.
Comments (Continued)
Comments of Dr. White
Vertical ocular oscillations are less frequent than
horizontal nystagmus. The more commonly encoun-
tered vertical nystagmus patterns are downbeat nys-
tagmus, upbeat nystagmus, and convergence retrac-
tion nystagmus.
SHOW THIRD PART OF VIDEO HERE
Differential Diagnosis of Vertical
Nystagmus
DOWNBEAT NYSTAGMUS
Downbeat nystagmus is usually caused by poste-
rior fossa disorders in the region of the cervicomed-
ullary junction. It is always considered to be patho-
logical and warrants thorough investigation. The
differential diagnosis includes Arnold–Chiari mal-
formation, cerebellar degeneration, Wernicke’s en-
cephalopathy, multiple sclerosis, lithium intoxication,
and paraneoplastic causes, among others. Downbeat
nystagmus may also be congenital or associated with
the inherited spinocerebellar disorders. Magnetic
resonance imaging with midsagittal views to best vi-
sualize the cervicomedullary junction abnormalities
is the first test to perform. Features of downbeat nys-
tagmus include the following:
1. Increase on lateral and oblique downward gaze
2. May obey Alexander’s law (nystagmus amplitude
increases in the direction of the fast phase, and
decreases in the direction opposite the fast
phase) although at times it does not
3. Skew deviation occasionally produces diplopia
266 Surv Ophthalmol 47 (3) May–June 2002 DANESH-MEYER

UPBEAT NYSTAGMUS retraction into the orbits on attempted voluntary

Lesions of the cerebellum, medulla, and pon-
tomesencephalic junction may cause upbeat nystag-
mus. The common causes are infarction, degenera-
tion syndromes, tumors, encephalitis, and Wernicke’s
encephalopathy. Upbeat nystagmus is pathologic
when present in primary position.
Features of upbeat nystagmus include the following:
1. Does not increase on lateral gaze
2. Generally obeys Alexander’s law
3. Convergence has variable effect: may increase,
decrease, or even reverse direction of
nystagmus
CONVERGENCE RETRACTION NYSTAGMUS
Convergence retraction nystagmus is character-
ized by bursts of converging fast phase movement
brought about by attempted upgaze or convergence.
This is reliably elicited by viewing a downward rotat-
ing optokinetic drum.
Convergence retraction nystagmus occurs with le-
sions of the dorsal midbrain (Parinaud’s syndrome)
that involve the posterior commissure, such as tu-
mors in the pineal region, infarction, demyelination,
or infection. In its full expression, Parinaud’s syn-
drome includes the following:
1. Pupillary light-near dissociation
2. Upper eyelid retraction at times even on
downward gaze
3. Palsy of upgaze with ocular convergence and
upgaze
4. Accommodative spasm or paresis
5. Convergence spasm or paresis
6. Diplopia due to cranial nerve III or IV paresis
or skew deviation
References
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2. Eggenberger E, Cornblath W, Stewart DH: Oculopalatal
tremor with tardive ataxia. J Neuroophthalmol 21:83–6, 2001
3. Goyal M, Versnick E, Tuite P, et al: Hypertrophic olivary de-
generation: metaanalysis of the temporal evolution of MR
findings. AJNR Am J Neuroradiol 21:1073–7, 2000
4. Gresty MA, Ell JJ, Findley LJ: Acquired pendular nystagmus:
its characteristics, localising value and pathophysiology. J
Neurol Neurosurg Psychiatry 45:431–9, 1982
5. Guillain G, Mollaret P: Deux cas myoclonies synchrones et
rhythmees velo-pharyngo-laryngo-oculodiaphragmatiques: le
probleme anatomique et physiolopathologique de ce syn-
drome. Rev Neurol (Paris) 2:545–66, 1931
6. Lapresle J: Rhythmic palatal myoclonus and the dentato-oli-
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7. Matsuo F, Ajax ET: Palatal myoclonus and denervation su-
persensitivity in the central nervous system. Ann Neurol 5:
72–8, 1979
8. Nakada T, Kwee IL: Oculopalatal myoclonus. Brain 109:
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The authors have no proprietary or commercial interest in any
product or concept discussed in this article.
Reprints of this article are not available.

Abstract. A 57-year-old woman presents with sudden onset of horizontal diplopia following surgical
repair of a ruptured posterior fossa aneurysm. Neuro-ophthalmic examination revealed a left gaze
palsy, right abducens palsy, bilateral facial nerve palsy, reverse ocular bobbing and oculopalatal myoclo-
nus. These findings can be localized to the anterior pons caused by damage to the midline perforator
vessels resulting in anterior pontine pathology. A video demonstration of the oculopalatal myoclonus
and other types of vertical nystagmus is provided. The etiology and characteristics of these forms of nys-
tagmus is discussed. (Surv Ophthalmol 47:263–266. © 2002 by Elsevier Science Inc. All rights
reserved.)

Key words. abducens palsy • gaze palsy • ocular bobbing • oculopalatal

myoclonus • oculopalatal tremor • reverse ocular bobbing • vertical nystagmus