IFN in MS patients will have to be determined. As any
beneficial effect of the induced cortisol would be expected to be short-lived, it seems unlikely that it would
have much effect in a protocol in which IFN doses are
given infrequently 147, but it might be of significance
when IFN is given daily 13, 111. Similarly, it would be
difficult to attribute any persistent beneficial effect of
IFN to cortisol induction 147. Monitoring of cortisol
levels in MS patients receiving IFN should clarify
these issues.
Preliminary results presented at the 112th Annual Meeting of the
American Neurological Association, San Francisco, CA, Oct 19,
1987.
We are indebted to the nursing service at the Clinical Center, NIH,
for rheir help in carrying out these protocols; to the Clinical Laboratory of the Clinical Center, NIH, for performing the cortisol determinations; to Elizabeth Mingioli, Laura Muehl, and Michael Covington for technical assistance; and to Sara Brewer for preparing the
manuscript.
Dr Bever is a recipient of a Career Development Award from the
Veterans Administration.

References
1. Johnson RT. The possible viral etiology of multiple sclerosis.
Adv Neurol 1975;13:1-46
2. Neighbour AP, Bloom BH. The interferon-natural killer cell
system in multiple sclerosis. Clin Immunol Allergy 1982;2:
279-295
3. Knobler RL, Panitch HS, Braheny SL, et al. Systemic alphainterferon therapy of multiple sclerosis. Neurology 1984;34:
1273-1279
4. Jacobs L, Herndon R, Freeman A, et al. Multicenter doubleblind study of effect of intrathecally administered natural human
fibroblast interferon on exacerbations of multiple sclerosis. Lancet 1986;2:1411- 1413
5. Roosth J, Pollard RB, Brown SL, Meyer WJ. Cortisol stimulation by recombinant interferon-alpha-2.J Neuroimmunol 1986;
12:311-316
6. Bever CT,Salazar AM, Neely E, et al. Preliminary trial of poly
ICLC in chronic progressive multiple sclerosis. Neurology
1986;36:494-498
7. Rose AS, Ellison GW, Myers LW, Tourtellotte WW. Criterion
for the clinical diagnosis of multiple sclerosis. Neurology
1968;18(~~ppl):1-59
8. Bever CT, McFarlin DE, Levy HB. A comparison of interferon
responses to poly ICLC in males and females. J Interferon Res
1985;5:423-428
9. Salazar AM, Gibbs CJ, Gadjusek DC, et al. Clinical use of
interferons: central nervous system disorders. In: Came P, Carter WA, eds. Handbook of experimental pharmacology. Berlin:
Springer-Verlag, 1983:472-497
10. Nugent CA, MacDiarmid WD, Nelson AR, et al. Rate of adrenal cortisol production in response to maximal stimulation with
ACTH. J Clin Endocrinol Metab 1963;23:684-693
11. Camenga DL, Johnson KP,Alter M, et al. Systemic recombinant a-2 interferon therapy in relapsing multiple sclerosis. Arch
Neurol 1986:43:1239-1246

Acquired Ocular Motor
Apraxia Due to Bilateral
Frontoparietal Infarcts
Charles Pierrot-Deseilligny, MD,
Jean-Claude Gautier, MD, and Philippe Loron, MD

A case of acquired ocular motor apraxia in a 78-year-old
man is reported. Disordered saccades and pursuit movements in the horizontal and vertical planes were recorded electrosculographically. Magnetic resonance
imaging showed bilateral border-zone infarcts, involving the frontal lobes and the superior part of the inferior
parietal lobules.
Pierrot-Deseilligny C, Gautier J-C, Loron P.
Acquired ocular motor apraxia due to
bilateral frontoparietal infarcts.
Ann Neurol 1988;23:199-202

Acquired “ocular motor apraxia” is characterized by
great difficulty in initiating saccades to command and
to visual targets, usually in all directions 117. This rare
syndrome results from bilateral frontoparietal lesions
and must be distinguished from various, and at times
severe, saccade abnormalities present in several degenerative diseases involving the basal ganglia: It is also
different from the congenital form of ocular motor
apraxia, in which saccade disturbance is restricted to
the horizontal plane and usually decreases with age 127.
We report a case of acquired ocular motor apraxia
which may add to our knowledge of the control of
purposive saccades and pursuit movements by the cerebral cortex.
Case Report
A 78-year-old righthanded man was admitted for right hand
weakness that began suddenly a few hours before. He had a
history of peripheral arterial insufficiency in the legs, first
degree atrioventricular block, and untreated hypertension.
The day after admission, he had paresis of both arms, predominantly distal and more severe on the right side, and
complex oculomotor deficits (see text following). Pupils and
visual fields were normal. There was no visual extinction
and no obvious visual neglect: the patient saw and easily
named objects placed in any part of his peripheral visual field
From the Service d‘Urgences C6r6brovasculaires and Unit6 INSERM 289, Hbpital de la SalpCtrihre, Paris,France
Received June 4, 1987, and in revised form Aug 11. Accepted for
publication Aug 13, 1987.
Address correspondence to Dr Ch. Pierrot-Deseilligny, HBpital de
la Salpftrihre, 47 boulevard de I’Hbpital, 75651 Paris Cedex 13,
France.

Copyright 0 1988 by the American Neurological Association

1 9

C
Fig 1 . T2-weighted magnetic resonance imaging. Coronal sections through the posterior part of the frontal lobes (A), the anteriorpart ofthe parietal lobes (B), the posterior part of the
parietal lobes (C), and near the parietooccipital junction (0).
Lesions (arrows) involved the jzrnctional tewitories of the middle
and anterior cerebral arteries.

without glancing at them. General physical examination was
normal except for the presence of atrial fibrillation. Doppler
studies showed mainly an occlusion of the right internal
carotid artery with reversed flow through the right
ophthalmic artery and tight stenosis of the left internal
carotid artery at its origin, with left ophthalmic artery flow in
the normal direction.
Three weeks after his admission, a T1-weighted magnetic
resonance image (MRI) was normal, but aT2-weighted MRI
showed bilateral watershed infarcts in the junctional territories of the middle and anterior cerebral arteries. The
lesions involved the posterior part of the left middle frontal
gyms (Fig lA), which includes the frontal eye fields (FEF)
[3}. O n the right side, only the white matter underlying the

200 Annals of Neurology Vol 23 No 2 February 1988

D
right FEF appeared to be involved. The lesions extended
posteriorly in the paramedian region of both hemispheres
(Fig lB), involving the superior part of the inferior parietal
lobule (IPL) and the lateral bank of the adjacent intraparietal
sulcus bilaterally (Fig 1C). They ended near the parietooccipital junction (Fig ID).

Eye Movements
Eye movements were recorded in darkness with directcurrent electrooculography. Calibration was carried out using
those rare visually guided saccades that were performed with
the head immobilized. Saccades were tested to command,
then to visual targets (located 25 degrees away from the
center), first with the head immobilized, then with the head
free. Foveal smooth pursuit was tested by asking the patient
to follow movement of the target light as it was driven in
triangular waveforms at several constant velocities (starting at
5 degreedsecond). Optokinetic nystagmus ( O m ) was stimulated with alternate black and white stripes moving on a
screen at several constant velocities (starting at 10 degrees/

n

M

v

L

s

v

s

A
R

t
a................................

............. M
c _

s

b . . . . . .1. . . . . . . . . . .
.

B

-

_.

v

250 m s

&p-

R

. . . . . .M
L

s

I300

1s

b

*

4111

t

Illb

M

M

R
M

E

L

-b-a
F

Fig 2. Lateral eye mwements. (A)Saccds to command, rightward (a) and leftward (b), precechd by a head movement with a
vestibular ocular movement in the opposite direction. (B) Visually
guided saccade: rightward, without a head movement (a); leftward, preceded by a head movement (b) (vertical awws indicate
the onset of the visual target). (C) Fovealsmooth pursuit: stimulation (a) and eye mwement (b); no movement. (0)Optokinetic
nystagmus: horizontal a m s indicate the stimubtion direction
and the vertical a w w the reversal of stimulation; no movement.
(Ej Oculocephalic movement: present. (F) Vestibular nystagmus,
induced by caloric stimulation of the left ear (a) and of the right
ear (b). L = left; M = midline; R = right; s = saccade; v =
vestibular ocular reflex.

second) and covering 80 degrees of the visual field. Oculocephalic movements were tested by passive movements of
the patient’s head performed by the examiner. The head
movement was not recorded. All these eye movements were
stimulated successively in the horizontal and vertical planes.
Horizontal vestibular nystagmus was elicited by successive
caloric stimulation of each ear with several milliliters of ice
water. Lastly, convergence was stimulated by slowly moving
a small object toward the patient’s nose and then toward each
eye.
The first eye movement recording was carried out 1 week
after admission. The patient was alert and cooperative
throughout the eye movement examination. At rest, he

looked straight ahead (without strabismus or diplopia), and
his eyes were permanently immobile without slow deviation
or spontaneous saccades (even in darkness). Blinks were rare
and usually absent during eye movements or attempted eye
movements.
All eye movement disorders were similar in the lateral and
vertical planes. Consequently, only the lateral eye movements will be described in detail (Fig 2). With the head
immobilized, rightward and leftward saccades to command
were absent; the eyes remained on the midline. With the
head free, disorders were similar rightward and leftward.
There was first a head movement in the appropriate direction, inducing a slow vestibular eye movement in the opposite direction (Fig 2A, ab). Then a saccade was performed in
the appropriate direction, allowing the eyes to return to the
midline. This final eye movement usually consisted of one
large saccade, although several small hypometric saccades
were occasionally observed. When there was only one saccade, velocity was measured and was about 50% less than
that of saccades with identical amplitudes performed by normal subjects in our laboratory.
Visually guided saccades with the head immobilized were
obtained only five times (2 rightward and 3 leftward) in 30
attempts in each direction. One of the two rightward visually
guided saccades is shown in Figure 2B, a. Latency of these
saccades was long, between 1,100 and 1,700 msec (i.e., five
to eight times the norm). Their velocity was reduced by the
same amount as that of saccades to command. With the head
free, the same sequence of movements as that described in
saccades to command was observed either rightward or leftward (Fig 2B, b).
Foveal smooth pursuit and OKN were absent in all directions, even at the lowest stimulation velocities (Fig 2C,D).
Oculocephalic movements were present, laterally (Fig 2E)
and vertically. Caloric stimulation of the left ear induced a
right-beating vestibular nystagmus, and caloric stimulation of
the right ear induced a smaller left-beating vestibular nystagmus (Fig 2F, ab). Quick phases of vestibular nystagmus were
slow. Convergence was absent for both eyes.

Course
Six weeks after the onset, paralysis of the right hand persisted. The motor deficit of the left upper limb had completely recovered. Eye movements were recorded again. All
saccades (to command and to visual targets, in the horizontal
as well as in the vertical plane) were normal, even with the
55 ms; left =
head immobilized. Latency (right = 262
256 2 62 ms) and velocity of lateral visually guided saccades
were normal. Pursuit movements, however, were still absent
in all directions. Foveal pursuit was saccadic, without any
smooth movement, and OKN could nor be elicited, the eyes
remaining immobile during stimulation. Convergence was
still absent. The patient refused to undergo MRI a second
time.

*

Discussion
This severe disturbance of the initiation of purposive
saccades in all directions, without actual paralysis, may
be considered ocular motor apraxia in its acquired
form [l]. At least two parallel excitatory suprareticular

Brief Communication: Pierrot-Deseilligny et al: Acquired Ocular Motor Apraxia

201

tracts could be involved in saccade initiation 14, 51.
They originate from the FEF and the superior colliculus, respectively, and project directly onto the premotor reticular formations in the brainstem. Such an
organization is supported by lesion experiments in the
monkey {b]: bilateral damage to the FEF alone, or to
the superior colliculus alone, induced only temporary
disturbance of saccades, whereas bilateral damage to
both structures resulted in the severe and long-lasting
impairment of saccades. It has recently been suggested
that the superior colliculus could in fact be a relay in an
excitatory pathway arising from the posterior portion
of the lateral bank of the intraparietal sulcus (located in
the IPL) 15, 71.
It is likely that the actions of the frontal and parietal
areas overlap somewhat in the normal subject because
only bilateral frontoparietal lesions severely impair saccade initiation in the hemispheric cerebral pathology.
In 3 clinicopathological patients with such lesions, saccades and pursuit were described as being absent. In 2
of them, lesions were metastatic or vascular and affected extensive areas of both the frontal and parietal
lobes bilaterally 18, 91. ln the third case, the lesions
were much smaller, ischemic in origin, and located, as
in the case of our patient, at the junction of the territories of the middle and anterior cerebral arteries in
both hemispheres [lo]. They involved the FEF and the
superior part of the IPL. Although the abnormal image
visible on MRJ in the present case involved only the
subcortical white matter in the right frontal lobe, this
lesion could interrupt the efferent connections of the
right FEF. These data in humans, as well as recent
experiments with analogous lesions in the monkey
[111, confirm that the control of purposive saccades is
mainly distributed across the FEF and the IPL in the
cerebral cortex. In the present case, the facilitation of
saccades by the combined head and vestibular ocular
movements could originate from a simple vestibular
mechanism or from the action of undamaged cortical
areas (such as the supplementary motor area). This
case also confirms that cortical lesions can influence
saccade velocity [41.
Lastly, foveal smooth pursuit and full-field pursuit
were absent in all directions. Bilateral damage to the
IPL (visible on MRI) could account for this abnormality because, as has been recently reported, such
lesions may result in severe impairment of pursuit
movements 171. Unlike saccades, pursuit movements
showed no improvement after 6 weeks. This suggests
not only that no significant recovery occurred in the
damaged parietal areas but also that there were no
adaptive mechanisms from other oculomotor areas, at
least within such a period of time.
The authors thank Dr E. Cabanis for the MRI study and Mrs S.
Rivaud for technical assistance.

References
1. Leigh RJ, Zee DS. The neuiology of eye movements. Philadelphia: Davis, 1983
2. Cogan DG. A type of congenital ocular motor apraxia presenting
jerky head movements. Am J Ophthalmol 1953;36:433441
3. Fox FT,Fox JM, Raichle ME, Burde RM.The role of cerebral
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11. Lynch JC: The interaction of prefrontal and parietooccipital cortex in the control of purposive eye movements in rhesus monkeys. Developments in oculomotor research. A satellite symposium of the XXX International Congress of Physiol0gic:al
Sciences. Proceedings, 1986:37

Hearing Loss
in Myotonic Dystrophy
Robert B. Wright, MD,* Russell H. Glantt, M.D,"
and Jaynee Butcher, PhDt
Seventeen of 25 patients with myotonic dystrophy had
moderate to severe hearing loss, usually sensorineural,
that was identified by routine audiometric screening
and was treatable in some patients. Further testing
failed to reveal a single pathophysiological process.
Wright lU3, Glantz RH, Butcher J.
Hearing loss in myotonic dystrophy.
Ann Neurol 1988;23:202-203
Myotonic dystrophy (dystrophia myotonica, DM) involves multiple systems. We noticed a surprisingly
From the *Department of Neurology and the Section of Communicative Disorders, Rush Medical College, Chicago, IL.
Received Apr 20, 1987, and in revised form July 10 and Aug 5.
Accepted for publication Aug 5 , 1987.
Address correspondence to Dr Wright, Rush-Presbyterian St Luke's
Medical Center, 1653 West Congress Parkway, Chicago, 1L 606123864.

202 Copyright 0 1988 by the American Neurological Association