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J. Neurol. Neurosutrg. Psychiat., 1965, 28, 48

Homolateral ataxia and crural paresis:A vascular syndrome1

C. M. FISHER AND MONROE COLE

From the Neurology Service of the Massachusetts General Hospital and the Department of Neurology, HarvardMedical School, U.S.A.

In the past few years, at the Massachusetts GeneralHospital, we have observed 14 patients havingsuffered strokes with an unusual neurological deficitin which the arm and leg on the same side showed a

combination of severe cerebellar-like ataxia andpyramidal signs. The cases so strongly resembledone another that the clinical picture may be regardedas a cerebrovascular syndrome.

In a typical case weakness of the lower limb,especially the ankle and toes, and a Babinski signwere associated with a striking dysmetria of the armand leg on the same side. The neurological examina-tion was otherwise almost normal. The only avail-able neuropathological examination was indecisivebecause of the great number and complexity of thelesions which had accumulated by the time the patientdied. The site of the responsible lesion is therefore atpresent uncertain and indeed it has been difficult toselect an anatomical locus which satisfactorilyaccounts for the neurological signs. Consideration ofthese cases requires discussion of the more generaltopic of appendicular ataxia resulting from supra-tentorial hemispheral lesions, a puzzling clinicalfinding. Furthermore it needs to be emphasizedthat in cases of stroke many kinds of incoordina-tion are often encountered which are difficult or

impossible to categorize accurately.

FIVE TYPICAL CASE HISTORIES

CASE 1 The patient, a man aged 44, while walking oneafternoon noted that the right leg 'acted queerly'. Tenminutes later while seated he found that on lighting acigarette the right arm overshot its target. As he walked200 yards to his physician's office, the right leg tended tofall out laterally as if the knee were 'wrong' and threatenedto buckle. As he waited, turning the pages of a magazinewas incoordinate. After a 30-minute examination, as thepatient was putting on his shoes, all symptoms clearedcompletely and he was able to walk with full stride. Itwas then approximately 50 minutes from the onset. Fiveminutes later the entire deficit recurred and the patientwas admitted to the Massachusetts General Hospital.'This study was aided by grant no. NB-O05 152 of the National Instituteof Neurological Diseases and Blindness, U.S. Public Health Service.

The general examination was not remarkable except formoderate obesity and mild hypertension (138/104 mm.Hg). On neurological examination the patient was alertand intelligent with no speech disorder. Visual acuity wasnormal and the visual fields were full. The optic fundiwere not remarkable. The pupils were equal and reactednormally. The extraocular movements were full. Hori-zontal nystagmus with the quick component to the leftwas present on left lateral gaze. Facial sensation andmovements were normal. Hearing was not impaired oncareful testing. There was neither dysarthria nor dys-phagia and the tongue protruded in the midline. Opto-kinetic nystagmus was diminished with targets movingto the left.Motor power was reduced to 6/10 on dorsiflexion of the

toes and ankle on the right side. Motion was strong at theright knee and hip as it was in the other limbs.The right arm and leg showed a severe intention tremor

of a cerebellar type. On the finger-nose test wild oscilla-tory movements caused the patient to strike himself inthe face. The patient could not use the right hand foreating. Abnormal rebound was easily demonstrated.On holding the arms out the right deviated laterally. Onthe heel-knee test there was a most severe dysmetria andthe right thigh tended to fall laterally although weaknesscould not be demonstrated.The tendon reflexes in the arms were average and equal.

The right knee jerk was 3/4, the left 1/4. The right anklejerk was 2/4, the left 0/4. The right plantar response wasbriskly extensor, the left flexor. Sucking and graspingreflexes were not obtained.

Sensation was normal to pin prick, temperature,vibration, position, and touch (graded hairs). Walkingwas unsteady and the right leg was dragged with toe-scraping and circumduction as in ordinary hemiplegia.On the Romberg test the patient toppled backwardsand to the right. The patient noted the right hand feltdifferent from the left-'swollen', 'stiff', 'larger', 'as ifthe tendons are tight'.A diagnosis of cerebral thrombosis was made and anti-

coagulant therapy was started. The ataxia of the rightupper extremity decreased gradually and at the end of oneweek the patient could use it for cutting his meat andeating. Nystagmus was still present on gaze to the left.The right thigh fell laterally on attempting the heel-kneetest; on hip flexion the force of contraction fluctuatedon different examinations. Dorsiflexion of the great toewas still weak and the patient said he could exert a better

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Hoinolateral ataxia and crural paresis: A vascular syndrome

force if he turned his head to one side. The tendon reflexeswere unchanged and there was still a right Babinski sign.On the Romberg test the patient swayed but remainedstanding. The gait was lurching and the right toe stillscraped the floor. After five months, recovery was judgedto be complete. There were no further incidents in thenext three years.

CASE 2 The patient, a man aged 57, got up during thenight to go to the bathroom and staggered badly. Onawakening in the morning he noted dragging of the leftleg, tingling of the left arm and leg, and a 'tremor' of theleft hand.

Examination later in the day disclosed an alert patientin full charge of his faculties. The cranial nerves were notremarkable except for slight inequality of the pupils(right 2 mm., left 2-5 mm.). Speech was clear. There was

no facial weakness. The left arm was slightly weak in allparts (9/10). The left leg was definitely weak with power

at the hip of 6/10, knee 4/10, dorsi- and plantar-flexion atthe ankle 3/10 and at the toes 0/10. There was a moststriking wild cerebellar ataxia of the left arm and leg on

all tests of coordination. The tendon reflexes were slightlyincreased on the left. The left plantar response was

absent, the right was flexor. Sensation was intact. Tonewas slightly increased in the left upper extremity. Therebound phenomenon was prominent in the left arm.

The blood pressure was 180/105 mm. Hg. The spinalfluid was normal. There had been no headache.

Great improvement took place in two weeks and a leftBabinski sign had become evident. Three months laterthere was very slight cerebellar ataxia in the left extremi-ties and a left Babinski sign. There was no further neuro-

logical trouble in the next two years.

CASE 3 A man, aged 78, on finishing supper at 6 p.m.

arose from the table and fell to the right to the floor.He was able to get to his feet and walked home a fewhundred yards but had to push himself to the left tocorrect an inclination to veer to the right. The next day hewalked a mile satisfactorily. Two days later he was worse

again and could scarcely stand, tending to wobble andfall to the right. He was admitted to the MassachusettsGeneral Hospital. The right leg distal to the mid-calffelt numb or tight for a few hours and then returned tonormal. There had been no dizziness, diplopia, numbnessof the face, tinnitus, or deafness.

Close questioning disclosed that the patient for aboutthree years had been 'unsteady' on his feet, described as a

sensation of staggering, lurching, or veering. Neurologicalexamination three months previously had shown no

ataxia and the patient could walk heel-to-toe.Five years previously the patient had suddenly suffered

severe upper thoracic back pain radiating downwardsinto the legs which became numb and collapsed underhim, causing him to fall to the ground. There was abdom-inal epigastric pain and the patient became ashen. He was

'a bit hazy mentally', and at night usually was delirious.Radiographs of the chest showed the recent developmentof a bulbous enlargement of the transverse portion of thearch of the aorta. Recovery from the illness was complete.On examination there was little abnormal except for

stance. The patient was alert and pleasant; memory wasmildly impaired. The fundi were not remarkable. Thevisual acuity was good and the visual fields were full. Thepupils could not be tested because of pilocarpine therapyfor glaucoma. The extraocular movements were fullexcept for upward gaze. Nystagmus was not present.There was no cranial bruit. The arms and legs were strong.Rapid alternating movements were performed wellbilaterally. Cerebellar ataxia of the mildest degree wasfound in the right leg but there was none in the left.Sensation in the arms was intact for pin prick, vibration,touch, position, and temperature. In the legs vibrationsense was almost absent below the knees and pin pricksensation was impaired over the dorsum of both feet.Position and light touch sensation were intact. The tendonreflexes were slightly brisker on the right, especially in thearm. The right knee jerk was 3/4, the left 2/4. Both anklejerks were absent; the plantar response was flexorbilaterally. On standing with the feet together the patientpromptly fell to the right; closing the eyes made nodifference. He was unable to walk heel-to-toe. Whensitting on the bed with the feet dangling he slowly toppledto the right. The blood pressure, which had been 180/120mm. Hg four months before, was 150/94 mm. Hg. Allpulses were absent at the ankles.

Cerebral thrombosis was diagnosed and anticoagulanttherapy begun. The patient improved slowly. On thethirteenth day he could walk better but on standing withthe feet together he still toppled to the right and on walk-ing leaned far to the right. On the eighteenth day ongetting out of bed in the morning he fell to the floorbecause of incoordination of the right leg. The next dayhe was worse and had little control over the right leg,veered to the right again, and could hardly walk. On thetwentieth day the right leg was weak. Control of the rightarm was impaired and he could not write his name orfeed himself. He could still wind his wristwatch. At timeshe noted a slight thickness of the tongue in talking and fora while the right arm was numb. The right foot distal tothe ankle felt as if bandaged. There was no diplopia orheadache. Examination now showed a marked cerebellar-like ataxia of the right arm. The finger-nose test discloseda gross wavering dysmetria chiefly at the shoulder andnot explained by weakness. Rapid alternating movementsof the distal parts, however, were carried out well. Powerin the right hand was approximately normal, possiblyreduced to 9/10 at the shoulder and elbow. The hand-dynamometer readings were right 80, left 70. The patientcould not handle a cup of coffee. There was a mild rightptosis. No dysphagia or dysarthria was noted although thepatient thought his articulation was not clear. There wasminimal flexion and extension of the toes and dorsiflexionof the ankle. At times good force was exerted momen-tarily only to fail immediately as in case 5. Plantarflexion of the ankles was 5/10. Motor power at the righthip was 5/10 to 7/10 but the patient had difficulty inmaking a coordinated effort. With the leg straight hecould hold the heel off the bed for at least 10 seconds. Onthe toe-finger test there was a gross swinging ataxia.The heel-knee test and heel-tapping could not be doneeven when the thigh was supported by the examiner.Muscle tone was normal.

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C. M. Fisher and Monroe Cole

The tendon reflexes in the right arm were exaggerated,the right knee jerk was much brisker than the left and theright plantar response was consistently extensor. Suckingand grasping reflexes were absent. The patient could notstand or walk and toppled to the right. On sitting thepatient fell to the right. The blood pressure was 160/100.Improvement took place in the next two weeks. The

ataxia of the right arm almost disappeared. There wasstill weakness of the right lower extremity at all jointsand all movements were grossly ataxic. It was difficult todecide if the incoordination was attributable to the weak-ness. When heel tappingwas carried out with the examinersupporting the right, gross dysmetric ataxia still persisted.The tendon reflexes remained brisker on the right. Thepatient could support his weight on the right leg but couldnot walk by himself. At the end of two months he walkedpushing a chair before him. Strength on extension andflexion of the knee was graded at 7/10 to 8/10. Yet hesaid, 'I have to get strength in that knee. It wobbles. It'sweak. It quivers'. The patient still toppled if he sat or ifhe stood with the feet 4 inches apart. The plantar responseon the right was flexor when the sole was stimulatedmedially, extensor on lateral stimulation; the left responsewas always flexor. On eliciting the left knee jerk, briskadduction of the opposite thigh occurred. This responsewas not evoked on tapping the right side. At the end ofseven months he could shuffle quickly along using thecane. The right upper extremity was normal. The rightleg was still ataxic and weak; on urging, however, andrepeated trials the power at each joint became almostnormal for a brief time. He could stand with his feettogether but on closing the eyes he lost his balance afterswaying for 30 seconds.

This case exemplifies well the difficulty in interpret-ing the clinical signs. Inability to stand or sit andtoppling to the right initially suggested a lesion of theright superior cerebellum. As the deficit increasedthe new signs seemed cerebellar in origin but whensevere weakness appeared in the right lower limbthe possibility arose that the ataxia was a manifesta-tion of weakness. This was unlikely, however, be-cause the instability persisted while sitting anddysmetria of the lower extremity was present evenwhen the thigh was supported. The pyramidal signson the right were never prominent but the tendonreflexes were clearly exaggerated and the plantarresponse was extensor for several weeks. Spasticityand clonus did not appear. Nystagmus was recordedonly once. Unlike the other cases the most persistentweakness in the leg involved the hip flexors, althoughdorsiflexion of the great toes and ankle was originallythe weakest movement. The sensory manifestationwas chiefly subjective and evanescent, tightness ofthe foot and numbness of the right hand. Ptosis wasnoted as in case 4. There was a hint of dysarthria attimes. The absence of any dizzy feeling in the headwas remarkable. The patient was right-handed yetthere was no dysphasia.

The patient lived three more years in which timehe had at least four more strokes, the final one beinga massive left sensorimotor hemiplegia caused by arecent occlusion of the right internal carotid artery.There were at least 11 old lesions in the brain, mostofthem taking the form of lacunes. The lesions on theleft side of the brain, which might account for thepatient's clinical picture described above, were asfollows: a 4 x 2 mm. lacune in the middle cerebellarpeduncle, a 2 mm. cavity in the mid-frontal whitematter, a 5 mm. cavity in the anterior frontal whitematter, a 1 cm. trabeculated slit in the mid-putamen,scattered small cortical infarcts in the border zonebetween the anterior and middle cerebral arteryterritories, and finally a large 9 by 5 mm. cavity inthe most posterior part of the internal capsule farsuperiorly adjacent to the corona radiata. The cere-bellum was not involved. The suggestion will beadvanced below that the large lesion in the internalcapsule was possibly responsible for the ataxia andcrural paralysis.

CASE 4 One afternoon this patient (right-handed), aman aged 84, got up from his chair and collapsed becauseof weakness of the right leg. He was admitted to thehospital where the right leg was found to be completelyparalyzed and the movements of the right arm weretotally lacking in control. There had been a pins andneedles feeling in the right leg at the onset. No warninghad been noted but for 'some time' the right leg or kneehad given out intermittently. There had been no dizziness,dysarthria, diplopia, dysphagia, incontinence, or head-ache. For several months he had noted numbness or asleeping feeling of the toes of the left foot and intermittentclaudication. Diabetes mellitus had been diagnosed 56years before. The patient was first examined by us twoweeks later when considerable improvement had alreadyoccurred.Examination on the fifteenth day of illness disclosed an

alert elderly man with good memory. The cranial nerveswere not remarkable except for a trace of right facialweakness on smiling. The visual fields were full. Thepupils were equal and reacted to light. The extraocularmovements were full without nystagmus. The tongue andpalate moved normally. There was no dysarthria ordysphasia. The carotid pulsations were strong and nobruit was heard in the neck or over the eyes.Motor power in the right arm was approximately

normal. Rapid alternating movements of the fingers andforearm were slowed. There was a moderately severecerebellar type of ataxia with clumsy oscillatory in-accurate movements on the finger-nose test. The patientcould not use his right hand for taking a drink or handlinghis eating utensils. On holding the upper limbs out-stretched the right drifted downwards. The right lowerextremity showed mild weakness of the flexors of the hipand knee and almost total paralysis of the ankle and toes.On the heel-knee and toe-finger test there was a moderatelysevere jerky cerebellar ataxia, possibly influenced by theweakness present at the hip. Tone was questionably in-

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Homolateral ataxia and crural paresis: A vascular syndrome

creased in the right limbs. Motor power and coordina-tion of the left limbs were normal.

Sensation in the arms was normal except for light touchwhich was decreased on the right hand and forearm. Inthe legs sensation for vibration, touch, and pin prick wasdiminished bilaterally; the appreciation of temperatureand position was intact.The tendon reflexes were brisker in the right limbs and

there was a right Babinski sign. The jaw jerk was minimal.Sucking and grasping reflexes were absent. The patientwas unable to stand by himself, failing to adjust his centreof gravity to the position of his leg.

All pulses were absent at the ankles. The blood pres-sure was 140/64 mm. Hg. Although the early course ofthe illness was not personally observed the picture aftertwo weeks was similar to that of the other patients.Nystagmus was missing and light touch may have beenimpaired in the right arm. The absence of dysarthriaand dysphasia was noteworthy.

CASE 5 On stepping out of a hot bath one morning thepatient, a man aged 66, staggered and almost fell. Thisunsteadiness lasted only a few minutes. On getting out ofthe car at his office one hour later he staggered again andcould scarcely stay upright. Within a few minutes heimproved and was able to walk to his office. One hourlater the ataxia returned and although lessening shortlythereafter it did not clear completely. At noon while beingexamined by his physician he lurched heavily and wouldhave fallen had he not been caught. On the Romberg testthe patient fell to the left. During the afternoon there wasdefinite improvement but at supper time he again couldscarcely stand. While sitting he felt perfectly comfortableand there was no dizzy feeling in the head. There was nonumbness, diplopia, dysarthria, or weakness. He de-scribed the difficulty in walking as though the left knee un-locked or bent with a jerk threatening to send him to thefloor.The patient had had a brief stroke involving the left

side four years before. On that occasion three briefattacks of numbness and tingling of the left arm and leghad occurred over a period of six hours at which time thesymptoms persisted. Examination showed minimal weak-ness of the left leg involving the flexor groups at the hip,knee, and ankle. The left heel-knee shin test was per-formed inaccurately. The left ankle jerk was brisker thanthe right and the left plantar response was extensor.Distal to the left knee, sensation was diminished minim-ally for pin prick and touch, severely for position andvibration. In one day all signs cleared except the leftBabinski sign which became equivocal a few days later.The patient received anticoagulant therapy at that timefor eight months.On examination the patient was clear mentally. The

cranial nerves were not remarkable except for an in-constant horizontal nystagmus on gaze to the left and aslight droop of the left upper eyelid which had beenpresent since the age of 12. Visual acuity was excellentand the fields were full on confrontation. The pupils wereequal and reacted to light and on convergence. The extra-ocular movements were full. There was no facial weaknessor dysarthria and the tongue protruded in the midline.

Hearing had been increasingly impaired bilaterally for20 years or more; this was familial. The carotids pulsatedwell and there was no intracranial bruit.Motor power was excellent everywhere except for

dorsiflexion of the left toes and ankle which was moder-ately weak. Occasionally brief contractions of almostnormal strength were possible. There was a cerebellar-like ataxia of moderate degree in the left upper and lowerextremities, most evident on the finger-nose, toe-finger,and shin-tapping test. Sensation to pin prick was slightlydecreased over the entire left side, including the upper partof the face, and cold stimuli were slightly cooler on theleft side. These changes were attributed to the previousstroke. Position sense, vibration, and light touch wereequal on the two sides. The tendon reflexes were briskeron the left at triceps, biceps, brachioradialis, knee, andankle. The left plantar response was extensor, the rightflexor. No sucking or grasping reflexes were obtained. Onstanding with the feet together the patient fell to the left.On walking he also tended to topple to the left. Theblood pressure was 180/100 mm. Hg.The cerebrospinal fluid was clear, under a pressure of

180, acellular, and contained 87 mg. protein per 100 ml.The blood cholesterol was 358 mg. per 100 ml. The sedi-mentation rate was 28 mm. in one hour.

After four days very gradual improvement took place.Anticoagulant therapy was maintained. On the fifth daythe patient still complained that the left knee tended tojerk into flexion producing instability. He attributed thisto weakness but careful motor testing showed normalpower. On the Romberg test swaying occurred but thepatient did not topple. Nystagmus on gaze to the left andcerebellar ataxia in the left arm and leg were still evident.The ataxia was greater in the leg than in the arm. Thetendon reflexes on the left were a little brisker and theleft plantar response remained extensor. At the end of fourweeks nystagmus, ataxia of the left upper extremity, andweakness of dorsiflexion of the left great toe had cleared.Ataxia of the left lower extremity gradually lessenedin the ensuing months and at the end of one year onlya minimal residuum of instability on walking remained.

In this case the previous occurrence of a left-sidedstroke involving the leg more than the arm com-plicated the picture. That attack, although evanes-cent, was associated the first day with incoordinationand weakness. The plantar response was extensor orequivocal for some months thereafter. In the presentillness a cerebellar-like ataxia of the left extremity(leg greater than arm) impaired stance and gait.Nystagmus, weakness of the dorsiflexors of the greattoe and ankle, and mild pyramidal signs bring thecase well within the scope of the syndrome underdiscussion. Since both strokes involved the sameparts it is likely that approximately the same cerebralregion was affected. The occurrence of a sensorydeficit in the first stroke might assist in determiningthe site of the lesion, although the combination ofsensory and cerebellar deficits on the same side ofthe body still poses a major problem in localization.

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DISCUSSION

THE CLINICAL PICTURE Thirteen of the 14 patientswere men. The ages ranged from 44 to 84. Twelve ofthe patients were hypertensive, the other two hadblood pressures of 140/64 and 150/92 mm. Hg.Preceding the stroke, half of the patients had one or

more brief transient ischaemic attacks occurring over

a relatively short period of a few hours. In the others,the stroke came on abruptly without warning. Insome the paralysis occurred in the night, in othersduring the morning or afternoon.

In the typical case at the onset there was difficultyin walking (imbalance, staggering, weak leg, 'legacted queerly') and incoordination of the arm on thesame side as the affected leg. At least eight patientsalso had transient paraesthesias (swollen stiff hand,tingling of the arm and leg, leg felt bandaged, atight feeling in the leg, tingling in the leg, tingling inthe opposite leg for a few hours followed by tinglingin the face, arm, and leg on the affected side for 24hours, tingling in the arm, numb feeling over thedorsum of one foot). Headache did not occur andthere was no record ofdiplopia, vertigo, incontinence,or mental change.On examination there was a gross oscillatory

ataxia of the limbs on the affected side. The leg wasweak, more distally than proximally, and in somecases only the toes and ankle were involved. Thearm showed little or no weakness. The face wasspared and usually there was no dysarthria. ABabinski sign was always present on the involvedside and the tendon reflexes were enhanced. Sensa-tion was intact in all but one case. Walking was im-possible, or almost so, without support. Two patientshad nystagmus. Residual deficits from former strokescomplicated the picture in two cases. The electro-encephalogram done in five cases was normal.

THE VASCULAR LESION The pathological process isalmost certainly cerebral thrombosis associated withatherosclerosis and hypertension. The stutteringonset, the occurrence of transient ischaemic spells,and in some cases the prompt recovery are all infavour of thrombosis with infarction. Haemorrhageand embolism can almost be excluded as possibilities.If the deductions to be made in the following para-graphs are correct, the site of the vascular occlusionlies in the lenticulo-striate artery or one of itsposterior branches, or in a penetrating branch of theanterior cerebral artery running to the coronaradiata.

TREATMENT AND PROGNOSIS We have used anti-coagulant therapy (Coumarin derivatives with orwithout heparin) and although the clinical course in

most cases was favourable we are unable to judgefrom our data whether such treatment was bene-ficial or not.

Recovery was almost complete in 12 of the 14cases and none progressed to a severe hemiplegia ordisabling aphasia. On this basis, it can be concludedthat the syndrome is relatively benign and thatarteriography and any therapy carrying a significantrisk are to be avoided. During convalescence severalof the patients complained of an instability of theknee.

INTERPRETATION OF SIGNS The weakness of thelower extremity can be attributed to a unilaterallesion of the cortico-spinal or pyramidal system; inall cases it was associated with a Babinski sign and inseveral cases there were increased tendon reflexes.Holmes (1922) found that lesions of a cerebellarhemisphere result in distinct weakness of the armbut not of the leg and a Babinski sign was neverassociated. We have tested motor power in a series ofpatients with severe cerebellar ataxia without pyra-midal signs and in none was there weakness of theflexors of the toes, ankles, or hips.The nature of the ataxia of the limbs is much less

certain. The semi-rhythmic oscillation of the limbsduring action, increasing on nearing the target, thedysmetria and dys-synergy on rapid alternatingmovements, and the presence of the rebound pheno-menon resulted in all observers labelling the inco-ordination as cerebellar in type. Nonetheless theunusual combination of pyramidal and cerebellarsigns on the same side of the body as the result of asingle stroke made it necessary to question the validityof designating the disorder as truly cerebellar. In theabsence of a crucial diagnostic laboratory criterionof cerebellar dysfunction, one must be satisfied withthe clinical impression that the disturbance of co-ordination was highly reminiscent of a cerebellardeficit.

In addition most of the patients showed a positiveRomberg test, toppling towards the affected side, animbalance which was not accounted for by weaknessof the leg.

THE SITE OF THE LESION If the lesion found in theupper part of the posterior limb of the internalcapsule in case 3 had been clearly established as thebasis of the syndrome, deliberation in the matter oflocalization could be brief. We would have to con-clude that a capsular lesion involving the cortico-spinal fibres to the opposite toes and ankle can alsoevoke cerebellar-like signs in the opposite arm andleg. The neurological picture in case 3, however, wasnot as straightforward as in several other cases andthe clinico-pathological correlation was far from

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satisfactory. Localization of the responsible lesion inthe internal capsule can therefore be only a tentativesuggestion.

If first we set aside for the moment the propositionthat the syndrome is the result of a supratentoriallesion, how might an infratentorial lesion explainthe symptoms? If a lesion lay bilaterally in the basispontis, the unusual combination of symptoms mightresult but it would be unlikely that such a lesion onone side would produce only pyramidal signs andon the other only cerebellar signs. Another possibilityis a unilateral lesion of the basis pontis affecting boththe pyramidal system and the pontine nuclei or theirprocesses destined to cross to the opposite middlecerebellar peduncle. This theoretically would pro-duce a combined pyramido-cerebellar disturbancein the same limbs but a clear example of this pheno-menon has never been reported. Bilateral cerebellarsigns would be more likely. A unilateral lesion lyingat any point in the brain-stem between the mid-medulla and the midbrain would ordinarily beexpected to produce crossed cerebellar ataxia andpyramidal signs. What is more, in our cases therewere few if any associated brain-stem signs. Theperipheral cranial nerves were spared and ocularmotor function was intact. Two of the patients had aweak horizontal nystagmus, a somewhat indefinitefinding but one which should not be dismissed tooreadily.

If theresponsible lesion were in the upper midbrainor subthalamic region it would be possible to involvethe superior cerebellar peduncle after it has crossedthe midline, and the cerebral peduncle lying immedi-ately anterior to it, producing a combined cerebello-pyramidal disturbance on the opposite side. In ourcases there were no signs of midbrain involvementsuch as a third nerve palsy, adventitious movements,or impairment of memory. In case 3 there was nomidbrain lesion at necropsy. The description ofsensory symptoms and the objective sensory findingsdid not suggest any anatomical pattern of involve-ment and were of no help in localization. Of the eightpatients with paraesthesias, the face was involved inonly one. This brief discussion points to the difficul-ties of finding a suitable locus in the brain-stem.To return to a consideration of a supratentorial

site for the lesion such a localization would havethe advantage of readily accounting for the homo-laterality of the pyramidal and cerebellar signs.Although 'cerebellar' ataxia as the result of hemi-spheral (or capsular) lesions seems to be uncommonthe literature contains several references to its occur-ence, not only with bifrontal tumours but withother lesions as well.A paracentral ataxia was described by Claude and

Lhermitte (1916) in three patients with bilateral

war wounds of the pate who showed a spasticparaparesis combined with prominent cerebellarsigns in the legs. The gait was wide-based, slow,festinating, uncertain, drunken, and the patient hadto look at the floor. Rombergism was obvious. Thelegs showed dysmetria and asynergy. Sensation wasintact. Two of the three had in addition a less severedysmetria of the left arm. Two further patients withsimilar wounds had much the same clinical signsexcept that sensation in the legs was severely dis-turbed. Cerebellar signs were found only with bi-lateral lesions in the paracentral region and not withhead wounds at other sites. The authors concludedthat cortical lesions can produce cerebellar as wellas pyramidal signs, probably by interrupting thecorticopontine paths running via the basis pontisto the opposite cerebellar hemisphere. Benisti (1918)reported another patient with identical symptoms.These cases, in so far as they demonstrate that apurely motor deficit in the legs can be associatedwith cerebellar ataxia, are directly relevant to thesyndrome under discussion, in which the lower limbis primarily affected. Furthermore it has been ourexperience that in ordinary hemiplegia, presumablyof supratentorial origin, it is not uncommon to findon the heel-knee, toe-finger, and heel-tapping tests amoderate incoordination of the leg which is difficultto distinguish from cerebellar ataxia and this mightbe taken as a hint that cerebellar symptoms may beclosely allied to pyramidal disorders affecting the leg.Even in some cases of paraparesis resulting fromspinal cord disease the legs may show a gross dys-synergy and dysmetria not easily attributable tospasticity or weakness.

Foix and Thevenard (1922) described a patientwith a verified tuberculoma of the right posteriorparacentral region who showed cerebellar signs inthe left extremities. In the lower extremity the heel-knee test elicited dysmetria and trembling. In theupper extremity movements were clumsy, and thefinger-nose test showed trembling similar to thatseen in multiple sclerosis. Adiadochokinesis waspresent. On the test of prehension, the fingers werespread excessively. The tendon reflexes were in-creased in the left lower extremity and the leftplantar response was not as clearly flexor as the right.There was no ideomotor apraxia. The affected limbswere hypotonic and the tendon reflexes were pendu-lar. Sensation was intact except for the sense ofposition in the left great toe. On walking the leftleg was flung forward more strongly than the right.Equilibrium was normal. On walking on all fours,dysmetria and asynergy of the left arm and leg wereclearly present.

Alajouanine and Lemaire (1925) described apatient with a paracentral tumour (unverified) who,

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on examination, showed clumsiness of fine fingermovements and oscillation and decomposition ofmovement in the heel-knee and finger-nose tests.Hypotonia was noted but the knee jerks were notpendular and terminal tremor was not present. Onstanding with the eyes closed or walking tandemthe patient fell to the right. On gaze to the left inter-mittent nystagmus appeared. Hearing was normal.Any weakness was questionable and involved thehip flexors.

In cerebrovascular disease, involvement of theparacentral region would indicate occlusion of theanterior cerebral artery. Yet in some 15 such casesexamined personally and proved by arteriographyor at necropsy, the syndrome under discussion hasfailed to appear. In case 3 the brain showed scat-tered cortical lesions in the border-zone regionbetween the anterior and middle cerebral territoriesbut the paracentral lobule was intact and the anteriorcerebral arteries were patent. Nevertheless, a lesionin the territory of the anterior cerebral artery mustfor the present remain one of the possible explana-tions of the syndrome.A case closely resembling our group of cases was

mentioned by Nicolescu, Cretu, and Demetresco(1920) in their discussion of the interior cerebralartery syndrome. A 78-year-old man, as the result ofa small stroke, developed an appreciable motordeficit of the right lower limb with lively reflexeson that side but no Babinski sign. Movementswere brusque and clumsy, the knee jerks pendular,and the gait a mixture of clumsiness and motordeficit. There was no apractic or sensory disturb-ance. No pathological study was made. The authorsattributed the syndrome to a lacune involving thecortico-pontine cerebellar fibres in the frontal-paracentral region above the anterior limb of theinternal capsule.The 'frontal ataxia' of Bruns and other pseudo-

cerebellar signs with tumours and injuries of thefrontal lobe have long been held by many authors tobe the result of involvement of the fronto-pontineradiation. In the original cases of Bruns (1892) thedisturbance was predominantly one of stance andgait with complete loss of balance and inability towalk without support (atasia-abasia). Incoordina-tion of the individual limbs was not mentioned.Gerstmann (1916) described a similar picture inpenetrating head injuries of the frontal region andsuggested as the cause a lesion of the cortical originof the fronto-pontine cerebellar system. His patientsshowed complete helplessness or great uncertainty instanding and walking, falling (the direction de-termined by the position of the head), nystagmus,vestibular hypersensitivity and past-pointing, butvery little dys-synergy or disturbance of rapid alter-

nating movements of the individual limbs. Gerst-mann and Schilder (1926) later attributed at leastsome of the gait disturbances of this type to motorapraxia or apraxia of gait.Hare (1931) in 50 cases of frontal lobe tumour

found severe cerebellar signs (staggering gait,Rombergism, drifting of one or both arms, dys-metria in the upper extremities, hypotonia, nystag-mus, ataxia in one or both lower extremities, past-pointing, dysdiadochokinesis, and asynergy of thepelvic muscles) in five cases and minor cerebellarsigns in 20 further cases. Brun (1932) dividedcases of frontal ataxia into two groups, a bilateralform resulting from advanced bilateral frontaltumours in association with raised intracranialpressure, and a mhore specific type of ataxia alwaysunilateral and involving the opposite leg. Examina-tion in the second group could be normal in the earlystages except for walking a straight line heel-to-toe,when the affected foot moved more or less uncer-tainly and was not placed squarely in front of theother; and at times the legs might be crossed. Theauthor was inclined to attribute the unilateraldisturbance to interruption of the fronto-pontinefibres to the opposite cerebellar hemisphere. Othershad also noted faulty placing of the affected foot inwalking: for example, one foot often trod on theother (Bruns, 1892), one foot crossed in front of theother (Gerstmann, 1916), and the patient put theaffected foot in the position the other foot was toassume in the next step (Gerstmann and Schilder,1926). Frazier (1936) in a large series of frontallobe tumours found frontal ataxia in 49% of cases.Gordon (1934) concluded that frontal ataxia differedfrom the cerebellar variety in that the reboundphenomenon, hypotonia, and pendular knee jerkswere missing in the former.These descriptions of frontal ataxia are almost

exclusively from cases of tumour or trauma, notreliable conditions on which to base inferencesconcerning localization of function. We were unableto find any reference to the occurrence of 'frontalataxia' in cases of pathologically verified infarction ofeither the cerebral cortex or the tracts descendingtherefrom in the corona radiata or internal capsule.It should be pointed out that in case 3 the fronto-pontine tracts, at least as depicted in anatomicaltexts, were apparently spared. Also these tracts arenot immediately adjacent to the cortico-spinal footandleg fibres in thecerebral hemisphereand the weak-ness of the foot would fail to be accounted for. Astrictly frontal location is unlikely because of theabsence of sucking and grasping reflexes, the intactmentality, normal limb postures, and control ofsphincters.

It should be pointed out that although the fronto-

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pontine system occupies the anterior limb of theinternal capsule, parieto-pontine and occipito-pontine fibres are said to course in the posteriorlimb of the internal capsule (Ranson and Clark,1947) where the lesion lay in case 3. There appearto be several sites at which hemispheral lesionstheoretically might evoke a cerebellar-like deficit.

Parietal ataxia, in which dys-synergy accompaniesa post-central lesion, has also been reported. Foix,Chavany, and Levy (1927) described dysmetria,decomposition of movement, and end-point tremorin a patient with a mild hemiparesis and severesensory loss as the result of an infarct of the parietalregion. Recently on the Stroke Service of the Massa-chusetts General Hospital a case of parietal ataxiahas been studied clinically and neuropathologically(Fisher, Appenzeller, and Descarries, 1964). Thelesion, which was a well-localized infarct, involved thecortex of the post-central arm area. The clinicalpicture in these cases of parietal ataxia clearly differsfrom that of the cases being reported here.Dwelling at further length on theoretical matters

is unwarranted. In summary we are inclined tobelieve that a supratentorial lesion of the cruralpyramidal fibres is responsible for the clinical picture.Possibly it lies in the posterior-superior part of theinternal capsule as in case 3 or in the adjacent coronaradiata. The ataxia would then not be attributableto a direct injury to the cerebellum or its main path-ways. Only further clinicopathological studies willclarify the matter.

SUMMARY

A cerebrovascular syndrome has been describedwhich comprises cerebellar ataxia, weakness, andpyramidal signs involving the limbs of the same side,the leg more than the arm. The pathological examina-tion in one case showed a conspicuous infarct in theposterior limb of the internal capsule but the clinico-pathological correlation was not conclusive becausethe brain showed many other infarcts. The respons-

ible lesion is nevertheless tentatively located in thecapsular-corona radiata region supratentorially.The ataxia is probably not due to direct involvementof the cerebellum or its main pathways but is relatedto damage to cortico-pontine connexions or tocertain fibres of the pyramidal system. The under-lying vascular process is probably thrombotic.The subject of ataxia arising from supratentorial

lesions has been briefly reviewed.

REFERENCES

Alajouanine, T., and Lemaire, A. (1925). Tumeur de la region para-centrale posterieure avec symptoms 'pseudo-cerebelleux'. Rev.neurol., 1, 71-75.

Benisti (nee Athanasiu) (1918). Les lesions de la zone rolandique(zone motrice et zone sensitive) par blessures de guerre. Thesede Paris, 127, Paris, Vigot.

Brun, R. (1932). Zur frage der stirnhirnataxie. Ein neues stirnhirn-symptom. Z. ges. Neurol. Psychiat., 138, 122-130.

Bruns, L. (1892). Ueber storungen des gleichgewichtes bei stirnhirn-tumoren. Dtsch. med. Wschr., 18, 138-140.

Claude, H., and Lhermitte, J. (1916). Les paraplegies cerebello-spasmodique et ataxo-cerebello-spasmodique consecutives auxIbsions bilaterales des lobules paracentraux par projectiles deguerre. Bull. Soc. Med. H6p. Paris, 40, 796-804.

Fisher, C. M., Appenzeller, O., and Descarries, L. P. (1964). A case ofparietal ataxia. In preparation.

Foix, C., Chavany, J. A., and Levy, M. (1927). Syndrome pseudo-thalamique d'origine parietale. Rev. neurol., 1, 68-76.

-, and Thevenard, A. (1922). Symptomes pseudo-cerebelleuxd'origine cerebrale, tubercule de la region paracentrale posteri-eure. Ibid., 1, 1502-1504.

Frazier, C. H. (1936). Tumour involving the frontal lobe alone;symptomatic survey of 105 verified cases. Arch. Neurol.Psychiat., 35, 525-571.

Gerstmann, J. (1916). Zur kenntnis der storungen des korpergleich-gewichtes nach schubverletzungen des stirnhirns. Mschr.Psychiat. Neurol., 40, 354-377.and Schilder, P. (1926). Ueber eine besondere Gangstorung beiStirnhirnerkrankung. Wien. med. Wschr., 76, 97-102.

Gordon, A. (1934). Frontal lobe lesions with cerebellar manifesta-tions; contribution to frontal lobe as center of equilibrium.J. nerv. ment. Dis., 79, 411-422.

Hare, C. C. (1931). The frequency and significance of cerebellarsymptoms in tumours of the frontal lobes. Bull. neurol. Inst.New York, 1, 532-562.

Holmes, G. (1922). Clinical symptoms of cerebellar disease and theirinterpretation. Lancet, 1, 1177-1182, 1231-1237, and 2, 59-65,111-115.

Nicolescu, J., Cretu, V., and Demetresco, L. (1920). Syndrome del'artere cerebrale anterieure. Monoplegie crurale droite avecsymptomatologie cerebelleuse preponderante. Bull. Soc. med.hop. Bucarest, vol. 10. Synopsis in Rev. neurol. (1932), 1, 563.

Ranson, S. W., and Clark, S. L. (1947). The Anatomy of the NervousSystem, 8th ed., Saunders, Philadelphia and London.

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