Journal ofNeurology, Neurosurgery, and Psychiatry 1990;53:675-680

Hemiparetic multiple sclerosis

J Cowan, I E C Ormerod, P Rudge

AbstractEight patients are described who presen-ted with hemiparesis which involved theface in seven. Six of the eight subsequen-tly developed clinically definite multiplesclerosis and in the remaining twopatients multiple sclerosis was the likelydiagnosis. Magnetic resonance imaginggave useful information about the site ofthe lesions responsible for the presentingsyndrome and provided ailditionalinformation in support of a diagnosis ofmultiple sclerosis.

Hemiparesis which involves the face isuncommon as the presenting feature of multi-ple sclerosis (MS), and may lead to diagnosticconfusion, particularly when the syndrome isprogressive or stuttering in onset. In recentyears various investigations have enabled thediagnosis of MS to be pursued in patientswho, because of their unusual clinical picture,previously might not have been suspected ofsuffering from the disease. Of these inves-tigative techniques magnetic resonance imag-ing (MRI) of the brain and spinal cord is themost sensitive. In this article patients withhemiparetic MS are described in whom MRIscanning gives useful information about theprobable sites of the lesions responsible forthe hemiparetic episodes. Other patients withan acute hemiparetic syndrome are describedin whom MS is the most likely diagnosis.

The National Hospitalfor Nervous Diseases,Queen Square,LondonJ CowanI E C OrmerodP RudgeCorrespondence to:Dr Ormerod,The National Hospitalfor Nervous Diseases,Queen Square, LondonWC1N 3BG,United KingdomReceived 20 June 1989and in revised form21 November 1989.Accepted 29 November 1989

PatientsCase 1 In 1975, when 39 years old, this righthanded woman experienced an episode of tin-gling affecting the whole of the left side of herbody and over the next three years she hadfive further episodes all precipitated by exer-

tion. The symptoms lasted for up to threeweeks and were accompanied by mild weak-ness on the left side. Between episodes shewas free of either symptoms or signs. At theage of 45 years she had another similarepisode of sensory disturbance and was admit-ted to hospital where within a few hours shedeveloped a left hemiparesis involving the faceand limbs. Computerised tomography (CT) ofthe head was normal. She recovered to near

normal over a period of six months. She thenstarted to have intermittent headaches andover the following eight months she had an

episode of weakness of the right leg followedby an episode of left hemiparesis. A repeat CTscan showed an area of low attenuation in the

right cerebral hemisphere, thought torepresent a mature infarct. Routine bloodtests, erythrocyte sedimentation rate (ESR),serology for syphilis and autoantibodies werenormal or negative. Further investigationswere unremarkable including echocar-diogram, arch aortogram, carotid ultrasoundscan and visual evoked potentials (VEPs).

In 1984 (aged 48 years) there was anotherepisode of hemiparesis involving the right faceand limbs which was associated with dys-phasia. She was admitted to the NationalHospital at which time the weakness had lar-gely resolved and the physical signs werelimited to ataxia of all limbs. A further CTscan showed two areas of low attenuation, thefirst in the white matter adjacent to the righttrigone and the second in the left centrumsemiovale. MRI at that time showed wide-spread periventricular lesions and otherlesions within the cerebral white matter withlesions bilaterally in the corona radiataimmediately superior to the internal capsule(fig 1). The cerebrospinal fluid (CSF) wasacellular but showed oligoclonal banding ofthe immunoglobulins. VEPs and somatosen-sory evoked potentials (SEPs) were normal.

Case 2 This right handed man developeda mild left hemiparesis, involving the face,when aged 34 years. This resolved completelyover a period of two weeks. He remained welluntil the age of 51 when he became impotent.At the age of 56 years he had an episode ofunsteadiness of gait with veering to the right.This lasted a week but did not resolve com-pletely and was followed by a progressiveunsteadiness of gait and dragging of the rightleg, and later he developed slurring of hisspeech and clumsiness of the hands. He wasadmitted to the National Hospital in 1985when aged 59 years. On examination he had aslurring dysarthria and there were pyramidalsigns in all limbs with extensor plantar res-ponses and ataxia of limbs and trunk. Routineblood tests including ESR and serology forsyphilis were normal; the anti-nuclear anti-body (ANF) was not sought. VEP latency wasincreased bilaterally and SEPs and brain stemauditory evoked potentials (AEPs) were bothabnormal. The CSF was acellular withnegative syphilis serology but showed oligo-clonal banding. Cranial CT was normal. MRIin 1985 showed several lesions in the whitematter of both cerebral hemispheres and inthe right internal capsule (fig 2).

Case 3 This left handed man had twoattacks of right sided weakness between theages of 13 and 15 years. When sixteen years

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Figure I MRI scans attwo levels (a, b) in patientI (SE2000160). Thereare periventricular lesionsand also discrete whitematter lesions. The lesionsinvolve the white matterimmediately above theinternal capsules.

Figure 2 MRI scan inpatient 2 (SE 2000140).There is a lesion in theright internal capsule(arrowed).

old he developed, over a period of 24 hours, a

right hemiparesis involving the face whichwas associated with a right hemisensory im-pairment, headache and intermittent diplopia.Two weeks later he was admitted to theNational Hospital. On examination he had a

slurring dysarthria. He had incomplete ab-duction of the left eye and some nystagmus on

lateral gaze to right or left. He had a severe

right hemiparesis with an extensor plantarresponse on the right. The CSF had a normalprotein content with six white cells/mm3. Aleft carotid angiogram was normal. He re-

covered within two weeks. At the age of 22years he had a left hemiparesis and hemi-sensory disturbance which recovered over

eight weeks. A few months later he had an

episode of right sided weakness with bilateralvisual impairment and intermittent diplopia.This came on within days of a bicycle accident

in which he had experienced an apparentlyminor head injury. He also noticed tinglingdown the spine and legs on neck flexion. Onexamination he had some restriction of abduc-tion of both eyes, nystagmus on upgaze andfirst degree horizontal nystagmus in bothdirections. There was sensory loss involvingall three divisions of the right trigeminalnerve, and both taste and hearing were im-paired on the right. In the limbs there wasweakness of the right leg with right sidedhyperreflexia. The CSF contained six lym-phocytes/mm3 and 0-5 g/l of protein. Bilateralvertebral and right carotid arteriograms werenormal.When he was 46 years he had an episode of

tinnitus with right hemisensory loss anddiplopia on looking to the right. These symp-toms lasted for two weeks. Two years later hedeveloped a right sided weakness over a fewhours and three days later he became dys-phasic. He was admitted to the National Hos-pital and found to have a nominal dysphasiaand a dense right hemiparesis involving theface with right hemisensory loss. The CSFcontained five lymphocytes/mm3 and a proteinof 0-69 g/l and oligoclonal bands were present;the syphilis serology was negative. Routineblood tests were normal including the ESRand autoantibody screen. The CT showedmild cerebral atrophy, cerebellar atrophy andan area of low attenuation in the left coronaradiata. The VEP was delayed on the right.MRI scanning was performed in 1985 (aged

52 years) which revealed lesions in the deepcerebral white matter of both hemispheresand in the left cerebral peduncle. There werealso lesions in the posterior limbs of bothinternal capsules (fig 3). Examination at thistime revealed a right hemiparesis and ataxia inboth arms. The plantar responses were flexor.

Case 4 In 1979, aged 23 years, this mansuddenly developed a sensation of pins andneedles down his right side followed by adense right hemiparesis. These symptomspassed off within minutes but he had nine

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Figure 3 MRI scans inpatient 3. The SE 2000/40 (a) image shows lesionsin both internal capsules(arrowed) confirmed onthe IR 2000/500 image(b).

similar episodes over the next 24 hours eachlasting about 10 minutes. He was transferredto the National Hospital the following day andhad another three similar episodes affectingthe right side the longest of which lasted fortwo hours and during which examination re-vealed a right hemiparesis involving the facewith hyperreflexia, a right extensor plantarresponse, and right hemisensory disturbance.He was left with a mild residual weaknesswhich cleared over the next two months.Routine blood tests were normal or negativeincluding ESR, ANF and serology forsyphilis. A CT scan showed an area of lowattenuation in the region of the internal cap-sule bilaterally. Four vessel cerebral angio-graphy was normal. He subsequently devel-oped impaired visual acuity bilaterally withright optic atrophy and bilaterally delayedVEPs. Oligoclonal bands were found in theCSF. Cranial MRI in 1985 showed periven-tricular changes around the margins of thelateral ventricles and discrete lesions within

the cerebral white matter. In particular therewere lesions in the posterior limb of the inter-nal capsule on the right and there was a lesioninvolving the posterior aspect of the leftlentiform nucleus and pallidum which in-volved the posterior limb of the internal cap-sule on the left (fig 4).

Case 5 At the age of 33 years this mandeveloped numbness and weakness in theright leg, followed some months later bysimilar symptoms in the right hand. He wasadmitted to hospital the following year wherehe was found to have first degree nystagmusto the right and a mild right hemiparesis,involving the leg more than the arm, andsparing the face. Myelography revealed nosignificant abnormality and a CT head scanwas normal. Examination of the CSF and theVEPs were normal.When he was 38 years of age he complained

of nocturnal jerking of the right and ofintermittent constipation. He noticed that hislegs were stiff and this was exacerbated by

Figure 4 MRI scans inpatient 4. The SE 2000/60 (a) image demonstrateslesions in both internalcapsules. The anatomicalposition of the lesions isshown more accurately onthe IR 2000/500 (b).

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constipation. On examination he was found tohave a spastic paraparesis. The following yearhe complained of impotence and four yearslater he developed transient numbness of theleft hand followed by intermittent pain in hislimbs which lasted two weeks and was exacer-bated by having a hot bath. Examination atthat time revealed a pale left optic disc and aright hemiparesis with a right extensor plantarresponse. The weakness of the right sideprogressed over the next five years. Bloodtests were normal including the ESR andsyphilis serology; an autoantibody screen wasnot performed. MRI examination at that timeshowed bilateral periventricular lesionsaround the margins of the lateral ventricles.

Case 6 At the age of 32 years this womanbegan to drop objects from her left hand andover the following six months she noticed aprogressive weakness of the left hand. A yearlater her left leg began to drag and shedeveloped weakness of the left side of the face.She was admitted to hospital for investigationin 1984 and at that time the CT head scan andthe VEPs were normal. The CSF had a nor-mal cell count with negative serology forsyphilis but contained oligoclonal bands. Nodefinite diagnosis was reached. Following herdischarge she had several episodes, each last-ing a few hours, of blurred vision affecting theleft eye. She also noticed a temporary increasein her left sided weakness following a hotbath. She was re-admitted to hospital formyelography which showed a swelling in thecervical spinal cord, and the weakness im-proved a little following a course of ACTHinjections. Later that year there was aprogressive decline in the power of her leftside and her speech became slurred. She wasadmitted to the National Hospital for furtherevaluation. On examination there was bilateraloptic atrophy and a left hemiparesis with a leftextensor plantar response. The tone in theright arm was increased and all limb reflexeswere brisk. The ESR was normal and the

syphilis serology was negative; an autoanti-body screen was not performed. MRI of thebrain was performed during this admission,two years after the first symptom. Thisshowed bilateral periventricular lesions and alesion in the posterior limb of the right inter-nal capsule (fig 5).

Case 7 This 26 year old woman awoke withweakness in the right arm and leg and a mildslurring of speech. There was some improve-ment over the next few hours but the followingday the weakness was more marked. She wasadmitted to the National Hospital. On examin-ation there was a right hemiparesis whichinvolved the face and over the next week theweakness progressed. The routine blood testswere normal or negative including ESR, auto-antibody screen and the syphilis serology.Evoked potentials, CT head scan and leftcarotid angiography were normal. The CSFprotein and cell count were normal but the IgGwas oligoclonal. MRI examination of the brainwas performed during her admission and thisshowed a lesion in the left internal capsule andthere were two other lesions seen in the cerebralwhite matter (fig 6).

Case 8 This 33 year old woman developedsudden blurring of vision in the right visualfield of each eye followed by complete loss ofvision in the same fields. This was associatedwith some pain, mainly over the forehead. Fivedays later she developed a worsening of theheadache and some unsteadiness of gait. Shewas admitted to the National Hospital. Thevisual acuities were 6/9 bilaterally and therewas a right homonymous hemianopia with amild right hemiparesis. Routine blood testswere normal or negative and these included theESR and ANF. The CSF contained eightlymphocytes/mm3 with a protein of055 g/l andoligoclonal bands but negative syphilisserology. A CT head scan was normal but MRIat that time revealed a lesion in the posteriorlimb of the right internal capsule and peri-ventricular lesions around the margins of the

Figure 5 MRI scans(SE 2000140) in patient6. There is a lesion in theright internal capsule (a)which also involves thecerebral peduncle (b).

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Figure 6 MRI scans inpatient 7. There is a lesionin the left internal capsuleseen on SE 2000/40 (a)and IR 2000/500 (b)images. There are alsoother lesions (arrowed)within the cerebral whitematter (c) seen on the IR2000/500 image.

lateral ventricles of both hemispheres (fig 7).

DiscussionThe veracity of the diagnosis of MS in theabove cases must be considered. Since none ofthe patients has died post mortem informationis not available. The most recent criteria for thediagnosis and categorisation of MS includeCSF analysis for oligoclonal bands, evokedpotentials and information obtained fromimaging techniques.' Using these criteriacases'1 can be classified as clinically definiteMS. Although it is most likely that cases 7 and 8presented with a first attack ofMS they cannotbe classified as MS at this time despite thepresence of oligoclonal bands in both. In eachof these latter cases there was only one clinicalattack with appropriate physical signs but theparaclinical evidence of additional lesions(MRI) was obtained during the initial episodewithout adequate temporal separation.The MRI scans showed the lesion probably

responsible for the hemiparesis in all but onecase and also showed additional clinicallyunsuspected lesions in all cases. In some of thecases the interval between the initial presenta-tion and the MRI examination was prolongedand this raises the question of the significanceof these MRI-demonstrated lesions. It cannotbe stated with absolute certainty that the MRI-demonstrated lesions caused the clinical syn-drome in question but the visualisation oflesions in appropriate sites is very striking. Itis not known how long lesions may remainvisible on MRI after their initial appearance.Although some MRI-visible lesions mayresolve there is no doubt that other lesions inpatients with MS have remained visible andstable over a period of years. The character anddistribution of the other brain lesions in our-cases corresponds to that previously describedin MS.2 The lesions thought to be responsiblefor hemiplegia occurred most commonly in theregion of the posterior limb of the internalcapsule, a site containing many large, myelin-ated fibres, through which runs the pyramidaltract.3

In case 5, the only patient in whom the facewas spared, the hemiparetic syndrome mightbe explained clinically by a spinal cord or brainstem lesion; not all patients with clinicalevidence of a brain stem or spinal cord syn-drome have lesions demonstrable by MRI.45The dysphasia observed in cases 1 and 3 is noteasily explained on the basis of the lesionsshown; there did not appear to be appropriatelysited cortical or subcortical lesions in thesepatients. Dysphasia has been described pre-viously in patiens with MS but it is uncommonand there has been discussion concerning thesites of the lesions responsible.67

It is unclear why hemiplegia involving theface is relatively uncommon in MS especiallywhen the corticospinal tracts are heavilymyelinated and have a long course. In a largeMRI study of patients with clinically definiteMS, lesions within the internal capsule wereseen in 11%Q of patients.2 To be clinicallyapparent a lesion must disrupt the function of

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Figure 7 MRI scans inpatient 8. There are lesionsin the left internal capsuleand around the righttrigone (arrows). Theanatomical site of thelesions is seen more clearlyon the IR 2000/40 image(b).

an adequate number of axons in a given path-way and this may explain in part the apparentdisparity. Lesions affecting the pyramidaltracts at a more caudal level are probably morecommon, particularly in the cervical spinalcord,8 and such lesions are frequently sym-metrical in the cord giving a tetraparetic ratherthan a hemiparetic presentation.The nature of the hemiparetic episodes is

also interesting. Symptoms have sometimeslasted for only a few minutes while others havelasted for months or have been permanent.Some episodes of marked weakness have lastedfor several days and then have completelyremitted, only to return later on both the sameand the opposite sides of the body. Why apatient should have a recurrence of such a raresymptom as hemiparesis, particularly withbilateral non-synchronous involvement, isunknown. Transient episodes, especially thoseonly lasting an hour or two are typical ofMS, asis the worsening of symptoms after a hot bath.9This may merely be physiological highlightingof an already existing lesion but hemiparesislasting longer presumably indicates the occur-rence of a new lesion.Hemiparetic MS has been discussed pre-

viously but only rarely as a isolated topic.McAlpine noted hemiplegia as an unusual firstsymptom in MS and commented that theseepisodes may be brief and recurrent.'0 He alsostated that the sudden onset of hemiparesis inMS may cause confusion with a haemorrhagicor thrombotic event;" angiography was oftenperformed in our patients and a diagnosis ofmigraine suggested in several. Kelly'2 notesthat the onset of hemiplegia in MS is mostcommonly subacute and Matthews" commentsthat acute hemiparesis in MS is rare and alsoappears more commonly in men.When a hemiparetic episode occurs as a first

neurological manifestation a vascular event,tumour, vasculitis, syphilis and encephalitis areamong the diagnoses considered and investiga-tions are performed to ascertain the cause andguide the clinician on appropriate man-agement. MRI may be useful in these patientsin identifying the causative lesion and alsodemonstrating other clinically unsuspectedlesions. These findings may obviate the needfor more invasive investigations such ascerebral angiography.

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2 Ormerod IEC, Miller DH, McDonald WI, et al. The role ofNMR imaging in the assessment of multiple sclerosis andisolated neurological lesions. Brain 1987;1 10:1579-616.

3 Hannaway J, Young RR. Localisation of the pyramidal tractin the internal capsule in man. JNeurol Sci 1977;34:63-70.

4 Ormerod IEC, Bronstein A, Rudge P, et al. Magneticresonance imaging in clinically isolated lesions ofthe brainstem. J Neurol Neurosurg Psychiatry 1986;49:737-43.

5 Miller DH, McDonald WI, Blumhardt LD, et al. Magneticresonance imagining in isolated non-compressive spinalcord syndromes. Ann Neurol 1987;22:714-23.

6 Olmos-Lau N, Ginsberg MD, Geller JB. Aphasia in multi-ple sclerosis. Neurology 1977;27:623-6.

7 Friedman JH, Brem H, Mayeux R. Global aphasia inmultiple sclerosis. Ann Neurol 1983;13:222-3.

8 Oppenheimer DR. The cervical cord in multiple sclerosis.Neuropathol Exp Neurobiol 1978;4:169-82.

9 Halliday AM, McDonald WI. The pathophysiology ofdemyelinating disease. Br Med Bull 1977;33:21-7.

10 McAlpine D, Lumsden CE, Acheson ED, eds. Multiplesclerosis: a reappraisal. Edinburgh 1972: ChurchillLivingstone, 129.

11 McAlpine D, Lumsden CE, Acheson ED, eds. Multiplesclerosis: a reappraisal. Edinburgh 1972: ChurchillLivingstone, 1972:172-5.

12 Kelly R. Clinical aspects ofmultiple sclerosis. In: Vinken PJ,Bruyn GW, eds. Handbook of clinical neurology vol 9.Amsterdam: North Holland, 1970.

13 Matthews WB, Acheson ED, Batchelor JR, Weller RO, eds.McAlpine's multiple sclerosis. Edinburgh: ChurchillLtvingstone, 1985:153.

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