J7ournal ofNeurology, Neurosurgery, and Psychiatry 1996;60:579-581

SHORT REPORT

Monocular elevation paresis and contralateraldowngaze paresis from unilateralmesodiencephalic infarction

G Wiest, C Baumgartner, P Schnider, S Trattnig, L Deecke, C Mueller

AbstractA 26 year old woman presented withmonocular elevation paresis of the righteye, contralateral paresis of downwardgaze, and subtle bilateral ptosis. Magneticresonance imaging disclosed a unilateralembolic infarction restricted to the meso-diencephalic junction involving the leftparamedian thalamus. Preserved verticaloculocephalic movements and intactBell's phenomenon suggested a supranu-clear lesion. This rare "crossed verticalgaze paresis" results from a lesion nearthe oculomotor nucleus affecting ipsilat-eral downward gaze and contralateralupward gaze fibres, originating in the ros-tral interstitial nucleus of the medial lon-gitudinal fasciculus (riMLF).

(7 Neurol Neurosurg Psychiatry 1996;60:579-581)

bidirectional shunting of blood. There was nohistory of cerebral infarctions or other neuro-logical diseases. Apart from a right sided mildsupranuclear facial palsy, the neurologicalexamination was normal. With the exceptionof a slight increase of LDH (248 U/l) and cho-lesterol (279 U/1) all laboratory studies gavenormal results. Deep vein thrombosis wasexcluded by sonography. Duplex sonographyof the carotid and vertebral arteries andcarotid-vertebral Doppler ultrasonographywere normal.

NEURO-OPHTHALMOLOGICAL FINDINGSOn admission mild bilateral ptosis was pre-sent, more pronounced on the left side. Bothpupils were 4 mm in diameter and reactednormally to direct light and to near stimulus.Fundoscopy and visual acuity were normaland there was no history of strabism. In theprimary position no deviation or cyclotorsion

Keywords: monocular elevation paresis; supranuclearlesion; mesencephalon

Departnent ofNeurology, UniversityofVienna, AustriaG WiestC BaumgartnerP SchniderS TrattnigL DeeckeC MuellerMRI-Institute,Department ofRadiology, UniversityofVienna, AustriaS TrattnigCorrespondence to:Dr Gerald Wiest,Department of ClinicalNeurology, University Clinicof Neurology,Wahringergurtel 18-20,A-1090 Vienna, Austria.Received 1 August 1995and in final revised form18 January 1996Accepted 24 January 1996

Jampel and Fells' were the first to describe anacquired monocular elevation paresis, whichwas attributed clinically to a contralaterallesion in the pretectum. The hypothesis, thatsupranuclear fibres to the subnucleus of theoculomotor complex-which supplies theopposite superior rectus-are affected, hassubsequently been confirmed by radiology andhistopathology in a patient with similar symp-toms.2 Its supranuclear nature is shown clini-cally by the persistence of verticaloculocephalic movements and the preservedBell's phenomenon. Our patient had anunusual combination of monocular elevationparesis and contralateral monocular paresis ofdownward gaze. Both radiological and clinicalfindings suggested a supranuclear lesion. Toour knowledge, this type of vertical oculomotordysfunction has not previously been reported.

Case reportA 26 year old woman noted a sudden onset ofvertical diplopia, dizziness, and weakness ofthe right angle of the mouth. Ten months ear-lier an atrial septal defect had been diagnosed.One week before admission, the patient under-went cardiac catheterisation, which showed a

..:

*. io.}.. 0. . . ...:: E ;. :: ::. E . . ,. .: .: . ..::r r .:: .'i!. te:o :t

.::iib"S . .. . -ERi:er F 7__ bBF.-i :... : .... . 1'*.} := ... - ! - a g.:'.- >8oe ago@' | E*:: . ?! !#Efic... :. > ' .. ':!.T.' .:.... : : ':: . ..

..

a; .y.

:' .ilK !<. , i y2eai. .: .::w_r lX

,:.

.. M.Y :: wFE alt. : . ,

°°.: ..:

*:w.#w

.. :: A 9fiSi5}}ew U.. o-.R;jg '8\.it . ' , .: . : ... ff| ,8; .. ; :.::::.:

.:

*.:..:.

na E-aRe9D3 llkeesq

:}& . }> -. lR;f'_. ^.}*e

.. :- ::_.: .:. .:.. _ :}3K :' iP

.. _ Peo; !8. ,eFe.

......

i:.. ........ ........ .............. . _ .... . -... :: . .: .:. .... .:.:. ,X ......... :. B se'_B ................. ' ': . ..

:s ...

.w E . . :::'^f: ..F::.XX -..**.: ... :.> weY. :^.

."' _-l K R.#.}..., ... _D;'; i' P XP

*\F._x>: .js:- _<_

:X^: 16 :.t .

^8£o*S:f9 .

::3t. s:-.i:0

:S.. :_.;*<_!.

Yo: ...

. P.w:V ^ ,imj .,,srE¢.f b- { e.S¢ . g.,a

:. Mm.;:0

._ Y9X& r s.

4w , e:.:*: :.

s: 2.

Figure 1 Positions ofgaze. From top to bottom: forwardgaze (showing the eyes level in the primary position andsubtle, left accentuated ptosis), upward gaze (showingelevation paresis of the right eye), right gaze, left gaze, anddownward gaze (showing downgaze paresis of the left eye).

579 on M

arch 1, 2020 by guest. Protected by copyright.

http://jnnp.bmj.com

/J N

eurol Neurosurg P

sychiatry: first published as 10.1136/jnnp.60.5.579 on 1 May 1996. D

ownloaded from

Wiest, Baumgartner, Schnider, Trattnig, Deecke, Mueller

Figure 2 (A) Sagzttal Tiweighted SE image afterintravenous gadolinium-DTPA: Intense, butinhomogeneous, contrastenhancement with irregularmargzns is shownextendingfrom the lowerpart of diencephalon to theupper portion ofmesencephalon. (B)Follow up examination(Tl weighted SE sequencein coronal plane afterintravenous gadolinium-DTPA) shows a CSFfilled parenchymal defectwithout contrastenhancement.

A

....I

oO Downgaze pathways* - Upgaze pathways

Figure 3 Premotor structures of vertical gaze control and presumed site of lesion. PC =posterior commissure; riMLF = rostral interstitial nucleus of the medial longitudinalfasciculus; DMLBs = medium lead burst neurons with downward on directions; UMLBs =medium lead burst neurons with upward on directions; iC = interstitial nucleus of Cajal;CCN = central caudal nucleus; III = oculomotor nucleus (the trochlear nucleus is notshown); SR = superior rectus muscle; IR = inferior rectus muscle; dashed area indicatespresumed site of lesion.

of the globe and fundus could be seen. Ondownward gaze and upward gaze, the patientmentioned vertical diplopia. Ductions showed adeficit of the left eye in downward gaze morepronounced in left downward gaze-and of

the right eye in upward gaze equally severewith the eye abducted or adducted. This isconsistent with underaction of the left inferiorrectus and right superior rectus muscle. Theright eye moved above the horizontal planefrom 250 to 30°; the maximal amplitude ofmovement under the midline in the left eyewas 200 in downgaze, and 10° in left down-ward gaze (fig 1).

Horizontal ocular ductions were of normalamplitude and convergence was present forboth eyes. Oculocephalic movements weresymmetrically preserved in both vertical direc-tions and Bell's phenomenon was normal.

Brain CT showed a small left paramedianthalamic hypodense lesion compatible with aninfarction, involving the upper part of the leftmesencephalon.

Magnetic resonance imaging of the brain-stem disclosed an abnormal, high intensity sig-nal on T2 weighted images 11 mm in diameterin the median upper part of the left mesen-cephalon extending to the left ventromedianthalamic area. Figure 2A shows the sagittal TIweighted SE image.The patient received heparin intravenously

for nine days. Diplopia, supranuclear facialpalsy, and ptosis were greatly improved aftertwo days, whereas dizziness persisted for oneweek. Brain CT showed a hypodense zone inthe infarction area two weeks after onset.When the patient was last seen neurologicallythree weeks after admission, the elevationparesis on the right eye had almost disap-peared. Downward gaze on the left eye wasmuch improved as well, and the patient wasable to depress the left eye about 30° to 350under the midline. As the double vision waspresent only in left downward gaze, it was tol-erated well by the patient. Follow up MRI onemonth after onset showed a sharply demar-cated infarction zone of fluid like density (fig2B). Two months after onset, the patientunderwent surgery for the atrial septal defect.

DiscussionMonocular elevation paresis is regardedmainly as the so-called "double elevator

580

t.. 410 .,.i

on March 1, 2020 by guest. P

rotected by copyright.http://jnnp.bm

j.com/

J Neurol N

eurosurg Psychiatry: first published as 10.1136/jnnp.60.5.579 on 1 M

ay 1996. Dow

nloaded from

Monocular elevation paresis and contralateral downgaze paresis from unilateral mesodiencephalic infarction

palsy," describing the inability to elevate oneeye in all horizontal positions, equally severe inabduction or adduction. The acquired form ofmonocular elevation palsy was first describedby Jampel and Fells.' Because the superiorrectus muscle is innervated by the contralat-eral superior rectus subnucleus and the infe-rior oblique is innervated by the ipsilateralinferior oblique subnucleus, they postulatedthat monocular elevation paresis of central ori-gin is most likely due to a lesion of thesupranuclear pathways for upward gaze in thecontralateral pretectum. Subsequently only afew clinical cases have been reported, and thelesions were too large to localise precisely theimpaired tracts.2The critical areas involved in vertical gaze

are the rostral interstitial nucleus of the mediallongitudinal fasciculus (riMLF), the intersti-tial nucleus of Cajal, and the posterior com-missure.89 Recent neurophysiological data (fig3) showed that premotor neural medium leadburst neurons with downward on directions(DMLBs), similar to premotor neural mediumlead burst neurons with upward on directions(UMLBs), are confined to the riMLF nucleusand that both types are intermixed. It isemphasised that the UMLBs of the riMLFramify extensively within a restricted region ofthe oculomotor nucleus, corresponding to theterritories of motor neurons supplying theinferior oblique and superior rectus muscles ofboth eyes.'3 The projection of riMLF UMLBsto upward motor neuron pools was found tobe bilateral. The RiMLF DMLBs, as well,ramify extensively within the dorsal two fifthsof the rostral pole of the oculomotor nucleusand in the trochlear nucleus. By contrast withUMLBs, DMLBs have mainly ipsilateral pro-jections to the oculomotor nucleus.'4 15

If a lesion thus reaches the fibres after thedecussation and before reaching the oculomo-tor nucleus, the elevation paralysis affects thecontralateral eye. If the lesion affects theupgaze efferent fibres of the riMLF just afterthey leave the nucleus and before they decus-sate, an ipsilateral elevation palsy will result."0In our patient the limitation of elevation wasequally severe with the eye abducted oradducted, suggesting a palsy of the superiorrectus muscle, according to the predominanceof action of the superior rectus in all positionsof gaze. The lesion of the meso diencephalicjunction thus may have destroyed premotorfibres projecting on to the ipsilateral superiorrectus nucleus (fig 3).

Preserved Bell's phenomenon and oculo-cephalic movements as well as the radiologicalfindings are strong arguments for a supranu-clear lesion. The mild bilateral ptosis, beingmore severe on the left side, accounts forinvolvement of the central caudal nucleus.To our knowledge, monocular palsy of

downward gaze-as shown in our patient-has

destruction of fibres ascending from the leftvestibular nuclei to the subnucleus that inner-vates the left inferior rectus was suggested. Inthe second report,'2 as could be the case in our

patient, it was assumed that descending fibresfrom the riMLF to the ipsilateral subnucleusof the inferior rectus were destroyed. In our

patient the downgaze paresis on the left eyewas more pronounced when the eye was

abducted, which accounts for an isolated palsyof the inferior rectus.Monocular elevation paresis, contralateral

downward gaze palsy, and mild bilateral ptosiscan theoretically be caused by a lesion in one

oculomotor nucleus. Clinically, however, thiswould at least coincide with loss of Bell'sphenomenon and upward oculocephalicresponses, which was not the case in our

patient. Furthermore, it is difficult to make a

strong argument for a nuclear lesion in theabsence of any pupillary mydriasis, accomoda-tive dysfunction, medial rectus involvement,or more impressive ptosis.

These findings support the possibility of a

monocular elevation palsy and contralateraldownward gaze paresis due to a unilateralmesodiencephalic lesion, as presented in our

patient. This rare case of dysconjugate oculo-motor disorder provides further evidence forthe supranuclear organisation of vertical gaze.

1 Jampel RS, Fells P. Monocular elevation paresis caused by a

central nervous system lesion. Arch Ophthalmol 1968;80:45-57.

2 Lessell S. Supranuclear paralysis of monocular elevation.Neurology 1975;25: 1134-6.

3 Bogousslavsky J, Regli F, Ghika J, Hungerbuhler JP.Internuclear opthalmoplegia, prenuclear paresis of con-

tralateral superior rectus, and bilateral ptosis. J7 Neurol1983;230: 197-203.

4 Bogousslavsky J, Regli F. Nuclear and prenuclear syn-

dromes of the oculomotor nerve. Neuro-ophthalmology1983;3:211-6.

5 Hommel M, Bogousslavsky J. The spectrum of verticalgaze palsy following unilateral brainstem stroke.Neurology 1991;41:1229-34.

6 Hoyt CS. Acquired "double elevator" palsy and poly-cythemia vera. J7 Pediatr Ophthalmol Strabismus 1979;15:362-5.

7 Deleu D, Buisseret T, Ebinger G. Vertical one-and-a-halfsyndrome. Supranuclear downgaze paralysis withmonocular elevation palsy. Arch Neurol 1989;46:1361-3.

8 Buttner-Ennever JA, Buttner U, Cohen B, Baumgartner G.Vertical gaze paralysis and the rostral interstitial nucleusof the medial longitudinal fasciculus. Brain 1982;105:125-49.

9 Pierrot-Deseilligny CH, Chain F, Gray F, Serdaru M,Escourolle R, Lhermitte F. Parinaud's syndrome.Electro-oculographic and anatomical analyses of six vas-

cular cases with deductions about vertical gaze organiza-tion in the premotor structures. Brain 1982;105:667-96.

10 Ford CS, Schwartze GM, Weaver RG, Troost BT.Monocular elevation paresis caused by an ipsilaterallesion. Neurology 1984;34: 1264-7.

11 Meienberg 0, Rover J, Kommerell G. Prenuclear paresis ofhomolateral inferior rectus and contralateral superioroblique eye muscles. Arch Neurol 1978;35:231-3.

12 Bogousslavsky J, Regli F. Upgaze palsy and monocularparesis of downward gaze from ipsilateral thalamo-mes-encephalic infarction: a vertical "one-and-a-half" syn-drome. JNeurol 1984;231:43-5.

13 Moschovakis AK, Scudder CA, Highstein SM, Warren JD.Structure of the primate oculomotor burst generator. I.

Medium-lead burst neurons with upward on-directions.Neurophysiol 199 1;65:203-17.

14 Moschovakis AK, Scudder CA, Highstein SM, Warren JD.Structure of the primate oculomotor burst generator. II.Medium-lead burst neurons with downward on-direc-tions. Neurophysiol 199 1;65:218-29.

15 Moschovakis AK, Scudder CA, Highstein SM. A structuralbasis for Hering's law: projections to extraocular

been reported only twice." 12 In the first case" m

581

motoneurons. Science 1990;i48:1118-9.

on March 1, 2020 by guest. P

rotected by copyright.http://jnnp.bm

j.com/

J Neurol N

eurosurg Psychiatry: first published as 10.1136/jnnp.60.5.579 on 1 M

ay 1996. Dow

nloaded from