Subject Review

Ocular Motor Abnormalities in Wallenberg's

Lateral Medullary Syndrome

PAUL W. BRAZIS, M.D.,* Department ofNeurology

The ocular motor abnormalities that commonly occur in Wallenberg's lateral medullary syndrome are
often unappreciated. These abnormalities include signs of dysfunction of ocular alignment (skew
deviation, ocular tilt reaction, and environmental tilt), various types of nystagmus, smooth pursuit and
gaze-holding abnormalities (eye deviation, ipsipulsion or lateropulsion, and impaired contralateral
pursuit), and saccadic abnormalities (ipsipulsion and torsipulsion). These impairments of ocular
motor control and their proposed mechanisms are discussed.

Wallenberg's lateral medullary syndrome is most often DYSFUNCTION OF OCULAR ALIGNMENT

caused by ischemia in the territory of the posterior inferior
cerebellar artery. The characteristic clinical picture is com-
posed of the following: (I) ipsilateral facial hypalgesia,
thermanesthesia, and pain due to involvement of the trigemi-
nal spinal nucleus and tract; (2) hypalgesia of the contra-
lateral trunk and extremities and thermanesthesia attribut-
able to damage to the spinothalamic tract; (3) ipsilateral
palatal, pharyngeal, and vocal cord paralysis, dysphagia, and
dysarthria caused by involvement of the nucleus ambiguus;
(4) vertigo, nausea, and vomiting as a result of involvement
of the vestibular nuclei; (5) ipsilateral cerebellar signs and
symptoms due to involvement of the inferior cerebellar
peduncle; and (6) ipsilateral Horner's syndrome from in-
volvement of descending sympathetic fibers. Although the
occurrence of Horner's syndrome is well recognized, pa-
tients with Wallenberg's syndrome also often have other
neuro-ophthalmologic abnormalities (Table 1). Herein these
abnormalities of ocular motor control will be summarized
and discussed in light of proposed pathways for the supranu-
clear control of eye movements.
*Mayo Clinic Jacksonville, Jacksonville, Florida.
Address reprint requests to Dr. P. W. Brazis, Department of Neurol-
ogy, Mayo Clinic Jacksonville, 4500 San Pablo Road, Jacksonville,
FL 32224.
Mayo Clin Proc 67:365-368, 1992
The membranous labyrinth, which consists of the utricle, the
saccule, and the three semicircular canals, monitors angular
and linear accelerations of the head. Linear acceleration is
monitored by specialized receptors, the maculae of the
utricle and saccule; horizontal head movements stimulate the
utricle linearly, and tilting of the head activates the saccule.
The utricle projects nerve fibers predominantly to the ipsi-
lateral lateral vestibular nucleus, and the saccule projects
nerve fibers to the y-group vestibular nuclei.' Utricular
projections (Fig. I) from the vestibular nuclei probably cross
the midline and ascend in the medial longitudinal fasciculus
to contact the nuclei of the third and fourth cranial nerves and
the interstitial nucleus of Cajal. 2•3 Lesions of these otolithic
pathways may result in imbalance of otolithic inputs and
may cause skew deviation (vertical divergence of Hertwig-
Magendie), a vertical misalignment of the visual axes caused
by a disturbance of prenuclear inputs," Skew deviation may
be associated with ocular torsion and head tilt (the ocular tilt
reaction), which is thought to be a motor compensation for a
lesion-induced subjective contraversive eye-head tilt. 2,3
Lateral medullary lesions damage the otolithic vestibular
.nuclei; therefore, patients with Wallenberg's syndrome often
demonstrate skew deviation, with the hypotropia on the side
of the lesion.Y Brandt and Dieterich? labeled this disorder
type 2 skew deviation and stated that this skew results from
elevation of only the contralateral eye without vertical dis-
365
366 OCULAR ABNORMALITIES IN WALLENBERG'S SYNDROME
Table l.--ocular Motor Abnormalities
in Wallenberg's Lateral Medullary Syndrome
Dysfunction ofocular alignment
Skew deviation
Ocular tilt reaction
Environmental tilt-"floor on ceiling" phenomenon
See-saw nystagmus
Nystagmus (multiple structures or pathways involved)
Horizontal
Torsional
Mixed horizontal-torsional
Mixed horizontal-torsional-vertical
See-saw nystagmus
Eyelid nystagmus
Smooth pursuit and gaze-holding abnormalities
Ipsilateral eye deviation
Impaired contralateral smooth pursuit
Lateropulsion of pursuit
Saccadic abnormalities
Ipsipulsion (lateropulsion)
Torsipulsion
Oblique saccadic trajectories on vertical gaze attempts
placement of the ipsilateral eye. Some patients also have an
ipsilateral head tilt and a disconjugate ocular torsion, with
excyclodeviation of the ipsilateral lower eye but with little or
no incyclodeviation of the contralateral higher eye. 2.8 Thus,
patients may complain of diplopia, and images may be both
displaced vertically and tilted with respect to each other.
Some patients with Wallenberg's syndrome complain of the
unusual (and almost unbelievable) sensation of environ-
mental tilt, in which the entire room is tilted on its side or
even upside down ("floor on ceiling" phenomenonj.V''"
This syndrome is also probably caused by a disturbance of
vestibular-otolith central connections.11
Damage to otolithic central projections that mediate ocu-
lar counterroll may also contribute to the genesis of torsional
nystagmus (see subsequent discussion) in the lateral medul-
lary syndrome," Central otolithic involvement may also be
responsible for the see-saw nystagmus observed in some
patients. 13-17 See-saw nystagmus is a disjunctive, vertical-
torsional nystagmus, one half-cycle of which consists of
elevation and intorsion of one eye in conjunction with syn-
chronous depression and extorsion of the other eye; the next
half-cycle consists of reversal of these vertical and torsional
movements. This type of nystagmus is usually pendular and
noted with large, extensive suprasellar lesions that compress
or infiltrate the mesodiencephalon bilaterally. With lateral
medullary lesions, however, a jerk see-saw nystagmus may
OCCUr.8.14.16 The torsional component of this nystagmus is
conjugate with the fast component contraversive to the side
of the lesion." This finding contrasts with the jerk see-saw
nystagmus described with unilateral focal mesodiencephalic
Mayo elin Proc, April 1992, Vol 67
lesions, in which the quick phase of the torsional component
is toward the side of the lesion."
NYSTAGMUS
In the lateral medullary syndrome, nystagmus may be due to
direct damage to the vestibular nuclei or their cerebellar,
semicircular canal, or otolithic connections. Nystagmus in
this syndrome is usually positional'? and can be horizon-
tal,18.19 torsional.v'? or mixed, with torsional, vertical, and
horizontal components." Typically, horizontal nystagmus
beats away from the side of the lesion; the horizontal drift
velocity is directed toward the side of the lesion and is
influenced by different positions of the eyes and by fixation.
Drift velocity is faster on contralateral than on ipsilateral
gaze, and it slows with fixation.'? Occasionally, the nys-
tagmus may beat with the fast component ipsilaterally during
gaze toward the side of the lesion20,21 or during closure of the
eyes." A vertical nystagmus is usually upbeating."
Torsional nystagmus is common in Wallenberg's syn-
drome; the upper pole of the iris beats away from the side of
the infarction." Torsional nystagmus may alter direction as
the eyes drift about a neutral position of torsion," It has been
attributed to an imbalance of central projections from the
anterior and posterior semicircular canals and the otolithic
receptors that mediate ocular counterroll."
As mentioned in the previous section, see-saw nystag-
mus may also occur in patients with lateral medullary le-
sions.13.15-17 Gaze-evoked eyelid nystagmus associated with
ocular nystagmus has been described, in which a clinically
obvious upward jerking of the eyelids occurred synchro-
nously with the fast phase of a gaze-evoked horizontal nys-
tagmus.f This eyelid nystagmus was inhibited or totally
arrested by the near reflex.
SMOOTH PURSUIT AND
GAZE-HOLDING ABNORMALITIES
The structures and pathways in the lateral medulla are also
concerned with smooth pursuit eye movements and gaze-
holding." The cerebellar flocculus, paraflocculus, and ver-
mis climbing fibers pass through the inferior cerebellar
peduncle and are involved with these functions."
Patients with the lateral medullary syndrome may com-
plain of a sensation of their bodies being "pulled to one side"
and may attempt to counteract this "lateropulsion of the
body" by leaning toward the opposite side." Because of
gaze-holding impairment, ocular movements may be simi-
larly affected, and the eyes tend to be "pulled" toward the
involved medulla (lateropulsion or ipsipulsion of eye move-
ments).5.18.20.21.23-29 If a patient is asked to fixate straight
ahead and close the eyelids, the eyes will deviate toward the
side of the medullary lesion (as reflected by a series of small
corrective hypometric saccadic [fast] eye movements in the
Mayo CIiD Proc, April 1992, Vol 67
Interstitial Nucleus
of Cajal
......
I CN III
Nucleus I.. . .
1.-
M
I CN IV
Nucleus
L
F
Fig. 1. Diagram showing utricular pathways. eN = cranial nerve; MLF = medial longitudinal
fasciculus.
opposite direction that occur in response to fixation when the
eyes are reopened). Even blinking may induce this latero-
pulsion. These abnormalities of gaze-holding may also be
reflected in saccadic eye movement abnormalities (see sub-
sequent discussion). Smooth pursuit eye movements that
track targets moving away from the side of the lesion are also
impaired in patients with lateral medullary lesions, whereas
pursuit toward the side of the lesion is normal or nearly
SO.20,23,27
SACCADIC ABNORMALITIES
The cerebellum may be involved in modulating the ampli-
tude but not the speed of saccadic (fast) eye movements."
Interruption of cerebellar central connections that traverse
the lateral medulla is thought to account for some observed
ocular motor deficits." Damage to the juxtarestiform body,
which carries signals from the fastigial nucleus to the brain-
stem reticular formation, may be the cause of a saccadic
abnormality referred to as lateropulsion of saccadic eye
movements.":"
As noted in the previous section, gaze-holding abnormali-
ties in patients with Wallenberg's syndrome may result in
ipsipulsion of eye movements. This disorder of gaze-hold-
ing may also induce saccadic abnormalities. Horizontal
saccades away from the side of the lesion are hypometric
(undershoot the target), whereas saccades directed toward
the side of the lesion are hypermetric (overshoot the target).
Quick phases of nystagmus are similarly affected. Ipsipul-
OCULAR ABNORMALITIES IN WALLENBERG'S SYNDROME 367
Lateral &
Y-~roup
Vestibu ar Nuclei 'I Utricle I
sion in patients with lateral medullary lesions is thus oppo-
site to the contrapulsion of saccades that is associated with
lesions of the superior cerebellar peduncle.P-"
This saccade bias in patients with lateral medullary le-
sions is also reflected in vertical eye movements. Attempts
to make a purely vertical saccade will result in an oblique or
elliptic saccade directed toward the lesion (in the direction of
lateropulsion); thus, corrective saccades away from the side
of the lesion are needed to bring the eyes back toward the
intended target." Later, attempted vertical saccades may
assume S-shaped trajectories as an adaptive strategy to cor-
rect the saccadic dysmetria." Even a torsional component of
this bias may occur (torsipulsion); inappropriate torsional
fast eye movements will be induced during saccades toward
or away from the side of the medullary lesion,"
ACKNOWLEDGMENT
Drs. James J. Corbett and Frank A. Rubino reviewed a draft
of the manuscript and provided helpful comments.
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