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Home , Athetosis, Hemiballismus, Hemiparesis, Hyperkinesia, Hyperreflexia, Monoplegia

J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.13.2.115 on 1 May 1950. Downloaded from

J. Neurol. Neurosurg. Psychiat., 1950, 13, 115.

HEMIBALLISMUS: /ETIOLOGY AND SURGICAL TREATMENT BY RUSSELL MEYERS, DONALD B. SWEENEY, and JESS T. SCHWIDDE From the Division of Neurosurgery, State University of Iowa, College ofMedicine, Iowa City, Iowa is a relatively uncommon hyper- 1949; Whittier). A few instances are on record in kinesia characterized by vigorous, extensive, and which the disorder has run an extended chronic rapidly executed, non-patterned, seemingly pur- course (Touche, 1901 ; Marcus and Sjogren, 1938), poseless movements involving one side of the body. while in one case reported by Lea-Plaza and Uiberall The movements are almost unceasing during the (1945) the abnormal movements are said to have waking state and, as with other con- ceased spontaneously after seven weeks. Hemi- sidered to be of extrapyramidal origin, they cease ballismus has also been known to cease following during sleep. the supervention of a haemorrhagic ictus. Clinical Aspects Terminology.-There appears to be among writers on this subject no agreement regarding the precise

Cases are on record (Whittier, 1947) in which the Protected by copyright. abnormal movements have been confined to a single features of the clinical phenomena to which the limb (" monoballismus ") or to both limbs of both term hemiballismus may properly be applied. sides (" biballismus ") (Martin and Alcock, 1934; Various authors have credited Kussmaul and Fischer von Santha, 1932). In a majority of recorded (1911) with introducing the term hemiballismus to instances, however, the face, neck, and trunk as well signify the flinging or flipping character of the limb as the limbs appear to have been involved. movements, but in general each observer's concept The patient usually retains a measure of voluntary of the features of hemiballismus represents but an control over the affected member so that acts such abstraction of his own limited experiences. The as walking, putting on slippers, and touching the consequence is that at most clinical demonstrations hand to the face can be performed even if with of a case alleged to be one of hemiballismus one or more members of the audience are moved to assert difficulty. The ballistic movements cannot however " be voluntarily checked for more than a few moments that the case in question is not " really an example at a time. The muscle tonus of the involved limb(s) of hemiballismus, but one of hemichorea, , may be normal but in many instances is slightly , or perhaps hysteria. If, on the other diminished (Martin and Alcock), and in consequence hand, the case is presented initially as an instance of the reduced initial tension of the muscles the deep of hemiathetosis or , similar protests may be http://jnnp.bmj.com/ reflexes of the affected limbs are often moderately raised. diminished. The terms athetosis, chorea, and ballismus refer The vast majority of patients are at or beyond to involuntary, non-patterned movements of bodily middle life at the time of onset of hemiballismus. members, which are unpredictable in respect of time as and form. They may be distinguished from the However, reports of patients young as seven myorhythmias, , the coarse, alternating (Bonhoeffer, 1930) and eighteen (Bianchi, 1909) and the years have been recorded. There appears to be no of , fine tremors of significant difference in sex incidence. thyrotoxicosis. They are present in the waking state on September 30, 2021 by guest. Spontaneous recovery is not to be expected and and absent during sleep. They appear both " at rest'" by far the majority of patients succumb within a and during the execution and maintenance of few days to several months ofprogressive exhaustion, "voluntary" movements. In our view, the three cardiac or and varieties may be distinguished from one another on failure, pneumonia (Grinker Bucy, the following grounds. In athetosis the movements *Case I of this communication was reported with motion picture are relatively slow, vermiform and writhing, and in illustrations at the Sixteenth Annual Meeting of the Harvey Cushing Society on August 19, 1948, at San Francisco, Cal. The paper in the limbs are chiefly due to the action of distal its present form was given with motion picture illustrations at the Seventy-fourth Annual Meeting of the American Neurological muscles. The proximal limb muscles may also Association on June 13, 1949, at Atlantic City, N.J. participate. Athetotic movements are character- 115 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.13.2.115 on 1 May 1950. Downloaded from

116 RUSSELL MEYERS, DONALD B. SWEENEY, AND JESS T. SCHWIDDE istically intermittent so that periods of several quent post-mortem studies have provided over minutes may elapse without the exhibition of fifty instances in which the corpus Luysi has been involuntary activity. In chorea the movements are damaged, though often along with other neural much more sudden and, insofar as the limbs are structures. Indeed, the number of studies in which, concerned, are subtended chiefly by action of the like those of Matzdorff (1927) and Thurel and more distal rather than the proximal muscles. As Grenier (1947), the only demonstrable in athetosis, choreic movements are frequently is limited strictly to the corpus subthalamicum is intermittent. Ballistic movements resemble choreic very small. movements in that they also are rapidly executed. In a review of the literature up to 1934, Martin The muscles of the face, neck and/or trunk may or and Alcock (1934) asserted that no case of hemi- may not be involved. The proximal muscles of the ballismus had been fully described in which the limbs are regularly involved, with the consequence corpus subthalamicum was not found damaged. that the limbs exhibit large excursions. Close This extreme position no longer appears tenable, inspection however usually reveals involvement but Whittier's (1947) observation that hemi- also of the more distal limb muscles. In contrast ballism is the " apparently inevitable symptom in to the intermittent activity characteristic of chorea man of destruction of the " is and athetosis, that of hemiballismus is almost probably quite correct. at the wakeful state. ceaselessly play during Involving Other Structures.-Several cases Additional Clinical Manifestations.-The intellec- of hemiballismus have been reported in which the tual, emotional, and sensory functions are seldom corpus subthalamicum is asserted to have been found deranged in hemiballismus. However, the coexis- intact at necropsy. In these the lesion considered tence of psychological aberrations, vegetative dis- responsible for the was disclosed in turbances, , dysphasia and/or oculo- other parts of the brain. Protected by copyright. motor disorders with hemiballismus has not in- Lesions of the Afferent or Efferent Fibres of the frequently been reported. In most such instances Corpus Subthalamicum.-In two of the earliest cases necropsy has demonstrated multiple or extended on record, those of Bianchi (1909) and Bonhoeffer lesions involving neural structures other than those (1897), the abnormal movements were imputed considered responsible for the ballistic movements. (Martin, 1928) to lesions at a level ")lower than" Aetiology and Pathology the corpus Luysi, presumably situated so as to destroy its efferent connexions to the midbrain. The pathological process most frequently en- That damage to either the afferent or efferent fibres countered in hemiballismus is a circumscribed of the nucleus might produce hemiballismus was encephalomalacia which is usually the result of postulated in 1939 by Moersch and Kernohan. cerebral thrombosis. mellitus not infre- Subsequently, Papez, Bennett, and Cash (1942) quently underlies the thrombotic process. Somewhat described a case which they considered illustrative less frequently small circumscribed hemorrhages of the point. The presence of multiple lesions in and emboli appear to be the responsible agents their case unfortunately equivocates their interpre- (Martin and Alcock, 1934). Other lesions, in- Kelman however, recorded the tation. (1945), http://jnnp.bmj.com/ cluding tuberculoma, gumma, metastatic , necropsy findings of a hemiballistic subject in which metastatic , and the co-called a small metastatic carcinoma from the lung involved primary degenerative processes of the brain, have the afferent subthalamic tracts. been identified. In two instances trauma has been considered the causative agent (Bucy, 1944; Lesions ofthe Corpus .-In 1926 Fragnito Schob, 1920). and Scarpini described the necropsy findings in an The anatomical sites at which lesions have been 80-year-old male who had suffered from hemi- encountered may be conveniently described under ballismus for three years before his death. An two main headings. encephalomalacic process had markedly damaged on September 30, 2021 by guest. the medial portion of the and, to a lesser Lesions Involving the Corpus Subthalamicum.- degree, the head of the . The The structure most frequently involved in hemi- corpus Luysi, medial lemniscus, and fields of Forel ballismus is the corpus subthalamicum contralateral were reported to be undamaged.* Several other to that side of the body exhibiting abnormal move- investigators (Vogt and Vogt, 1920; Austregesiol ments. Greiff (1883) is credited with having been *This is the only case acknowledged by Martin and Alcock to the first to observe this correlation. In a carefully constitute a valid exception to the doctrine concerning the necessary involvement of the corpus subthalamicum in the pathogenesis of executed study forty years later this observation hemiballismus. Its importance theoretically and practically is was firmly substantiated by Jakob (1923). Subse- obvious. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.13.2.115 on 1 May 1950. Downloaded from

SURGICAL TREATMENT OF HEMIBALLISMUS 117 and Gallotti, 1924) have reported cases in which cases compel reservation concerning the doctrine lesions of the corpus striatum were considered that hemiballismus is invariably subtended by a responsible for hemiballismus or for hyperkinesias lesion of the corpus Luysi. simulating the latter. Certain of these cases have Thus far animal experimentation has shed but been objected to by Martin and Alcock (1934) on little light on the anatomico-clinical problems the ground that conclusive evidence clearing the related to hemiballismus. Morgan (1927) reported corpus subthalamicum of damage has not been failure to produce hyperkinesia in a series of dogs in proffered. However, Austregesilo and Borges- which lesions were placed in the corpus subthala- Fortes (1937) and Davison and Goodhart (1938) micum and varying adjacent structures. These have each reported well-studied cases in which the findings contrast sharply with those in the human neostriatum alone has been the seat of damage. in whom damage of the corpus subthalamicum by Although their cases were published under the titles disease is regularly followed by ballistic movements. of " hemichorea " and " monochorea ", the des- Wilson (1914) and Delmas-Marsalet (1925) criptions of the abnormal movements conform so reported inability to produce choreic or ballistic closely to those of hemiballismus that differentiation movements in laboratory animals by placing appears difficult if not impossible. experimental lesions in the caudate nucleus. Similar In Case 2 of the present communication, the negative evidence from human material was adduced lesion apparently responsible for the patient's by one of us (Meyers, 1941; 1942a ; 1942b). hyperkinesia was disclosed in and adjacent to the Extirpation of all available portions of the head of right lenticular nucleus, i.e., involving both the the caudate nucleus in nine patients with Parkinson- neo- and paleostriatum. Histological examination ism failed to produce hyperkinesia of any variety. revealed no implication of the corpus Luysi. In certain instances the rostral third of the putamen Lesions in the and and in one instance the oral pole of the globus .-Lewandowsky pallidus were removed in addition to the caudate Protected by copyright. Stadelman (1912) reported a case of hemiballismus head without producing abnormal movements. in which a large lesion was disclosed in the lateral Browder (1948) in an independent study on the thalamic nuclei extending inferiorly into Forel's surgery of Parkinsonism, has reported ablation of Haubenfelde. Unfortunately, however, the authors the superior half of the head of the caudate nucleus made no specific report regarding the corpus sub- in a series of humans without the development of thalamicum. In a case described by Nikitin (quoted hyperkinesia. by von Santha, 1932) a relatively large lesion was Mettler and Carpenter (1949) have recently encountered in the ventrolateral thalamus. Its in ventral border is stated to have extended to within succeeded producing choreo-athetoid movements 2 mm. of the dorsal border of the corpus Luysi, the in rhesus monkeys by electrocoagulation of the uninvolved. At subthalamus. These investigators are at present latter structure being evidently endeavouring to assay the effects of " analytic" least two other instances have been recorded in surgery in these animals. Their work constitutes which the apparently responsible lesions were the first promising animal experimentation in the demonstrated in the thalamus at a sufficient distance area. from the corpus Luysi to vitiate the frequently hyperkinetic raised objection that the blood supply of the latter Therapy http://jnnp.bmj.com/ must have been compromised (Malan and Civalleri, 1921; Zontoni, 1908). In the endeavour to treat the distressing symptoms of hemiballismus numerous physical and pharma- Lesions in the Postcentral Gyrus.-In 1929 Wilson cological agents have been employed. As with reported a case in which degenerative, atrophic most other extrapyramidal disorders, the results of changes of the contralateral postcentral gyrus were such therapy have been largely disappointing the only lesions demonstrable at necropsy. Although (Meyers, 1942a). The treatment of hemiballismus this report is unique, it must be retained for con- may be conveniently discussed under three general on September 30, 2021 by guest. sideration in view of Wilson's reputation for headings: physical, pharmacological, and surgical. thoroughness and objectivity. In briefrecapitulation, then, apparently valid cases Physical Measures.-Bertrand and Garcin (1933) of hemiballismus have resulted from lesions in the described a case in which it became necessary to afferent and efferent fibres of the corpus Luysi, the lash the hemiballistic arm to the patient's side in neo- and paleostriatum, the ventral and lateral order to prevent self-. Wilson described a thalamus and the postcentral gyrus. Although few similar case in which the hyperactive upper limb in number compared with those in which the was secured to an iron frame in order to prevent the responsible lesion involves the corpus Luysi, such patient from raining blows upon herself. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.13.2.115 on 1 May 1950. Downloaded from

118 RUSSELL MEYERS, DONALD B. SWEENEY, AND JESS T. SCHWIDDE Pharmacological Measures.-It has been a matter Peripheral Operations.-Amputation has been of general experience that sedatives, hypnotics, and resorted to as a measure of desperation in at least narcotics prove effective in hemiballismus onlv when one case of hemiballismus (Schaller, 1937), and in administered in doses sufficiently large to induce another instance of the brachial plexus sleep. The atropine derivatives have yielded uni- by multiple injections of alcohol was employed formly indifferent results. Stramonium has been (Kulenkampff, 1938). In 1931 Jermutowicz re- said to exert a " somewhat quieting " effect and ported a salutary result following mechanical tobacco is stated to be " useful ", but in no instance stretching of the brachial plexus. A severe has it been claimed that these agents have abolished of the limb was produced, but the ballistic move- the hyperkinesia. ments were sufficiently damped to permit the patient to resume work "albeit with difficulty ". Central Operations.- Jermutowicz quite properly anticipated a return of ballistic movements in his patient. It is of con- siderable historic interest that he pro- posed among other possibilities extirpa- tion of the contra- Protected by copyright. lateral " cortical center for the shoulder and arm ". Just such anoperation was performed in 1939 by Bucy (1944) for the relief of left upper monoballismus in a 21-year-old man who four years before had sustained a severe head injury. Pneumo- e ce phalography revealed a dilated

right lateral ventricle. http://jnnp.bmj.com/ FIG. 1.-Diagram illustrating the cortical area resected in Bucy's case of left AAt operation theh rightih hemiballismus. (Reprinted through the courttesy of Dr. Paul Bucy and prto the University of Illinois Press from " The IPrecentral " was 1944, p. 362, Urbana, Ill.) identified by electrical stimulation and that Ballistic movements were temporarily arrested in portion from which movements of the upper onecase (Papez andothers, 1942) by the intramuscular limb were elicited was extirpated together with use of curare. Unfortunately, the dose required portions of the frontal gyri lying rostral thereto to achieve this effect (80 units) produced also a (Fig. 1). The anterior wall of the central fissure on September 30, 2021 by guest. generalized paresis. The unpleasant side-effects of was included in the resection. curare, erythroidin hydrochloride, and related agents, The patient's abnormal movements were abolished their short-lived action, and the necessity of often- following this procedure. By the end of the first repeated parenteral administration impose serious year they had returned to a slight degree but at the limitations on their continued use. end of the third year they were described as " mini- mal and usually present only when he is conscious Surgical Measures.7-Amputation, peripheral, and of being observed ". Following operation, con- central operations have been reported, with vulsive supervened for a time. These were varying results. ultimately brought under control by . J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.13.2.115 on 1 May 1950. Downloaded from

SURGICAL TREATMENT OF HEMIBALLISMUS 119 The enduring neurological residua included a marked blood sugar was 100 mg. % and the urine showed a spastic paresis of upper motor type, hyper- 3 plus sugar reaction. The was clear reflexia and dyspraxia of the previously ballistic and under an initial pressure of 125 mm. H2O. Its total limb. Gross movements, however, could be per- protein measured 37 mg. %. The cell count was normal formed and the patient was gainfully employed. and the Wassermann test negative. Special Studies.-Pre-operative electrographic studies Linear Cortico-subcortical Section just Anterior to of the right corpus striatum and neighbouring structures Area 4: A New Procedure (deep leads) were carried out simultaneously with electro- With the objects of ameliorating hemiballistic encephalographic recordings from standard placements movements and of scalp leads. The technique employed has been preserving all possible function, previously described (Meyers and others, 1949). The fre- the writers recently employed a surgical procedure quency, amplitude, wave-form, and polarity character- based on a tentative theory of the pathogenesis of istics of the patient's recordings were compared with hemiballismus.* In essence, the operation consists data similarly derived from 11 control subjects. They of subpial section of the cortex and subcortical disclosed no distinguishing features (Figs. 2 and 3). white matter immediately rostral to Area 4 in such Operation, March 2, 1948.-A right fronto-parietal fashion as to interrupt the U-fibres coursing between bone flap was reflected under local procaine anxsthesia Areas 4, 3, 1, and 2 posteriorly, and 4-s and 6 and the excitable motor cortex identified by means of anteriorly. Abstracts of two cases treated in this bipolar stimulation with a 60 cps. sine-wave. The manner follow. threshold was disclosed at 3V and *25 ma. (approx.). Case Reports The extent of the motor cortex was mapped out with a 3 5V. stimulus and Area 4 was thus envisioned. Toward Case I.-F.B., a 50-year-old woman, was admitted to the end of this the University on procedure a generalized convulsion was Hospital Feb. 14, 1948, complaining of inadvertently produced and during the ensuing period ceaseless agitation of the limbs of the left side. The of stupor the ballistic movements were absent. When, Protected by copyright. disorder had begun in mid-December, 1947, as irregular after some twenty minutes, the patient again became twitchings of the left half of the face. The movements alert, the hyperkinesia reappeared. A subpial incision increased steadily in frequency and amplitude so that at the was then made through the cortex and subcortical end of the second week the left upper and lower U-fibres a line anterior to the limbs exhibited violent and for the most part uncon- along just envisioned trollable Area 4. It was carried to a depth of approximately movements. Toward the end of the first month 2-5 cm. and ran through those portions of the precentral some slowing of speech and a slight weakness of the left gyrus described von Bonin lower limb were noted. by as Areas 4a and 4y (Fig. 4). The patient considered her The suppressor area, 4-s, and the premotor cortex, condition intolerable. Area 6, were presumed to lie anterior to the line of The previous medical history was non-contributory, incision. except that for the past seven years she had been known During execution of this incision the to have diabetes mellitus. This condition had been well ballistic move- controlled ments ceased abruptly and a flaccid, areflexic by insulin and diet. of the left upper limb was demonstrable. A slight degree Examination disclosed an intelligent, affable, white of voluntary control over the proximal muscles of the woman in good general condition. The blood pressure extremity was retained. The left half of the face and the was 180/100 mm. Hg. The optic fundi disclosed a left lower limb proved to be only moderately paretic. moderate degree of diabetic retinopathy. The out- standing finding consisted of vigorous, irregular, and Early Postoperative Course.-No hyperkinesia was http://jnnp.bmj.com/ rapidly executed movements of flinging type implicating observed at examination eight hours after operation. the proximal and distal muscles of the left-sided The left upper limb remained paralyzed but the patient appendages. The head jerked irregularly towards the executed fairly vigorous movements of the left lower left, the jaws champed and the left side of the face limb, both spontaneously and to command. The supra- exhibited short-lived grimaces. These movements were nuclear left facial paresis remained unaltered. Plantar almost constantly in play during repose and during stimulation evoked a flexor response bilaterally. The voluntary activity they often became violent. They left upper limb was both analgesic and anxsthetic. were absent only in sleep. Quick-succession movements The patient's subsequent course was generally satis- and the finger-to-nose tests were awkwardly performed factory. On the fourth postoperative day she suddenly on September 30, 2021 by guest. on the left side. The right biceps jerk was sluggish, the became unresponsive for a period of one minute. The left absent. The knee jerks were sluggish but equal, and eyes deviated to the left but no tonic or clonic phenomena the achilles reflexes were absent. The remainder of appeared. Upon recovery from this episode the patient the examination was non-contributory. was immediately able to engage in conversation. She was neither confused nor distressed. A similar pheno- Laboratory Studies.-The blood counts, hemoglobin menon occurred on the fifth postoperative day. After level, blood Wassermann test and radiographs ofthe skull the institution of phenobarbital and sodium diphenyl and chest revealed no remarkable findings. The fasting hydantoinate therapy there was no recurrence. The *This pathogenetic theory will be made the subject of a separate drugs were continued until the end of the communication. third month. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.13.2.115 on 1 May 1950. Downloaded from .S'J''#r4\Ai.A SOA$ W * i i -. ., t P: . V

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FIG. 2.-EEG of Case I. The scalp electrodes in the standard positions were connected to reference electrodes on the ears (e). The prevailing alpha wave frequency is 8/s., the amplitude ranging between 25 and 50,uv. The frequency, amplitude, wave-form and polarity characteristics of the recordings were similar to those obtained in 11 normal controls. LF and RF=left and right frontal LP and RP=left and right motor; LO and RO=left and right occipital; RT=right temporal zones. Protected by copyright.

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FIG. 3.-EEGs of Case I derived from ring-type pick-up electrodes placed in the right corpus striatum and neigh- bouring structures. Electrode No. 1 was the deepest of a series ofeight, and was located just ventral to the head of the right caudate nucleus. l-e, 3-e, 5-e, and 7-e indicate that monopolar recording technique was used, the numbered ring being one of a pair of pick-up leads the second member of which was attached to the right ear (-e). The mean frequency of waves derived from such sources was 8k/s., with a range of 61-11. The amplitude ranged between 10 and 6O0,v. 1-3, 3-5, and 5-7 indicate that bipolar recording technique was used, the rings being connected to each other as numbered. The mean frequency of waves thus recorded was 11/s., with a range of 8-16. The amplitude ranged between 5-25,uv. RP-e=scalp electrode placed over right motor area and connected to a reference electrode on the ear (cf. RP-e, Fig. 2). These electrostriatograms are indis- tinguishable from those obtained in 11 normal controls. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.13.2.115 on 1 May 1950. Downloaded from

SURGICAL TREATMENT OF HEMIBALLISMUS 121 The paresis of the left lower extremity increased and within a month possessed also the left lower limb. somewhat during the third, fourth, and fifth postoperative During the three weeks before being admitted to hospital days, but repeated examinations failed to disclose they became increasingly more vigorous and were almost evidence of , , , or Babinski's ceaselessly at play during the waking state. The patient toe phenomenon. Early in the second week the paresis could not voluntarily suppress the movements. The of this limb improved perceptibly. The paralysis of the involved muscles felt sore and fatigued but were not left upper limb, however, continued unaltered. The painful. Sedatives were required for sleep. patient was discharged to her home on March 19, 1948. The patient became very irritable and weary, and Later Postoperative Course.-At the end of the third during the third and fourth months of her illness lost month the patient was still free of hyperkinesia. The over 25 pounds. For the ten days preceding her admis- left facial paresis had completely receded and the sion she had been unable to walk, even with assistance. functions of the left upper extremity had returned Anorexia and nausea were prominent symptoms during sufficiently to permit of the cutting offood, tying of shoe- the week before admission. laces, and similar practical skills. The motor power of Past History.-Dyspncea and palpitation had been the left upper limb was considered approximately half present " for many years ", and the patient was known normal and quick-succession movements and the finger- to have,had arterial for about a decade. to-nose test could be executed, although awkwardly. Digitalis therapy had been administered for one and The left biceps and triceps jerks were more brisk than one-half years before the present illness and there had those on the right but Hoffman's sign was absent. There been no symptoms of cardiac failure. She had borne was a slight stereognostic defect. Otherwise, the five healthy children. Except for " nervousness " and sensory functions on the left side were unimpaired. In intermittent attacks of gall bladder colic over the course walking, the patient exhibited a slight to moderate degree of 25 years, the past history was unremarkable. of circumduction. She could, however, walk on her Physical Examination.-The patient appeared ill and heels and there was no Babinski sign. A residue of the harassed. The blood pressure measured 220/80 mm. previous hyperkinesia was discernible as short-lived, Hg. The retinal arteries were somewhat narrow, and irregular, incoordinate movements of small amplitude, owing to opacities of the ocular media, the optic disc Protected by copyright. present chiefly when the patient was walking or executing margins appeared hazy. The cranial nerves were " voluntary " movements with the left upper limb. In clinically intact. A small nodule was palpable in the repose, she exhibited no hyperkinesia. left lobe of the thyroid gland. The heart was slightly The patient was re-examined at the end of the fifth enlarged to the left and exhibited an irregular irregularity month and at intervals of three months thereafter. To of beat. The chest was emphysematous and the radial date, improvement has been progressive and she is in arteries felt sclerotic. good health, gainfully employed as a housekeeper. Her Almost continuous ballistic movements possessed the is entirely normal and the left lower limb is entirely left-sided limbs, the head, tongue and left half of the face. free ofhyperkinesia. The upper limb exhibits occasional Owing to their presence the tendon reflexes of the left twitches of small amplitude, particularly when the side could not be reliably evaluated; however, they patient is excited. She is able to use both hands for seemed in general less brisk than those of the opposite washing, cooking, ironing, sewing, fastening buttons, side. In the same manner, the muscle tone of the tying shoelaces, etc. Dyspraxia is apparent only in such hyperkinetic limbs seemed diminished. The ballistic complex skills as crocheting. Stereognosis has returned movements (as analyzed by slow-motion cinematography) to normal. The tendon jerks continue somewhat over- were manifestly produced by activities of the muscles active, but there is no spasticity and the Hoffman sign of the shoulder and hip girdles as well as of the proximal cannot be elicited. and distal muscles of the extremities themselves. The http://jnnp.bmj.com/ Comment.-Without such information as would be strength of the left grip was approximately half that afforded by necropsy study it is manifestly impossible to exhibited on the right. Tests for gait and coordination assert with confidence where the pathological lesion could not be carried out. responsible for this patient's hyperkinesia was located. Operation, Jan. 25, 1949.-Under rectal avertin and Irrespective of its locus, however, it seems reasonable light intravenous pentobarbital anmsthesia the motor to infer that the salutary effect of operation was achieved cortex was outlined and the threshold of that portion by interrupting some neural circuit upon the integrity corresponding to the upper extremity determined at of which the mediation of ballistic movements depended. 2 25 V. and 0 25 ma., a 60 cps. sine-wave.

using on September 30, 2021 by guest. Case II.-J.S., a 71-year-old housewife was admitted Stimulation along the anterior margin of the precentral to the University Hospital on January 19, 1949, com- gyrus and the posterior margins of the frontal gyri just plaining of severe jerking movements of the limbs of anterior thereto with 4 0-50 V. evoked no motor dis- the left side of three months' duration. Early in charges but following the application of such stimuli by September, 1948, she had experienced unsteadiness and 8-15 seconds the previously ascertained threshold value " dizziness " for a week. In mid-October uncontrollable of stimulus failed on several trials to evoke its usual jerking movements appeared, first on the left side of the response when applied to the motor cortex. The sub- face and neck and shortly thereafter in the left shoulder sequent return of excitability of the latter at 2-25 V and arm. After a few days the movements abated appeared to indicate that no spontaneous heightening of somewhat, but they soon returned with renewed vigour, its threshold had taken place. Other portions of the D J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.13.2.115 on 1 May 1950. Downloaded from

122 RUSSELL MEYERS, DONALD B. SWEENEY, AND JESS T. SCHWIDDE exposed cortex failed to show this phenomenon. seen again. The patient was moderately stuporous for Although not without reservation, the operators con- the better part of the first week; after this she became sidered that the forward region probably corresponded more responsive and by the tenth day was fairly rational. to the suppressor strip, 4-s. A subpial cortico-subcortical Muscle tonus was first detectable by clinical tests in the incision was then carried out as described in Case I(Fig. 4). left lower limb. The tendon reflexes were at this time absent on the left side and normal on the right. The heart 4.MOTOR dORTEX rate was 88 per minute and the 4a (FA) blood pressure 130/60 mm. Hg. During the third postoperative 4\ 4- s, SUPPR ESSIOR AREAX week the patient at times seemed PREMO' rOD, .ORTEX bewildered although most of the - .., 6,, time her speech was rational. On the seventeenth day she executed weak movements with the left lower extremity and inquired when she might go home. Although examination was considered not completely reliable, her sensory functions appeared essentially undisturbed. The left hemiplegia persisted. Terminal Course.-On the twenty-sixth postoperative day the patient's general condition appeared satisfactory. While sitting up eating supper she Protected by copyright. aspirated a quantity of milk soup. Dyspnoea and cyanosis super- vened at once. The skin became C. .PRE-C. cool, mottled, and profusely wet <-/> with perspiration. The heart rate increased to 140 per minute and the blood pressure rose to 4 220/80 mm. Hg. Auscultation (y l,I:{WG/ .#I' revealed no unusual heart findings, but coarse rales were heard anteriorly over both sides FIG. 4.-Diagram of lateral aspect of right hemisphere shiowing the presumed of the chest. Speech was slow topographic relations of the line of subpial section cEarried out in Cases I and measured. The patient and II. The motor cortex was identified by electrical stimulation, and the remained conscious throughout disposition of Brodmann's Area 4 then estimated by visual inspection. and followed verbal directions The subpial incision followed a line just anterior to Are,a 4, probably cutting well. A moderate quantity of through 4y and 4a of von Bonin. Area 4-s, the s;uppressor strip, and thin mucoid q y http://jnnp.bmj.com/ Area 6, the premotor cortex, are represented rostral t(o the line of incision thm bchsomaterial was aspir- The pyramidal and parapyramidal corticofugal fibres arising from Area 4 ated by bronchoscopy from the are represented in their course through the depth of the hemisphere. In trachea and left upper and the inset, the depth of the incision through the sulbcortical U-fibres of lower bronchi. Oxygen therapy adjoining and near-neighbouring gyri is illustrated. IInt.C. =right internal was instituted. The acute episode capsule; Crus=right crus cerebri; G.Pre-C. =precent,ral gyrus; G.Post-C. subsided within 45 minutes. =postcentral gyrus. Electrocardiography failed to provide evidence of a coronary movements were occlusion or pulmonary infarct. A few hours later Early Postoperative Course.-Ballistic on September 30, 2021 by guest. absent throughout the remainder of the patient's course. fine and coarse rales were audible over the left lower A left hemiplegia of flaccid, areflexic type was demon- lobe. Despite the prophylactic administration of strable following recovery from anesthesia. The vital penicillin, pneumonia developed and the patient died signs reached normal levels on the third postoperative three days later, on the twenty-ninth postoperative day. day. At this time a well-patterned Jacksonian ofslight to moderate severity and 30-40 seconds' duration Necropsy.-An acute bronchopneumonic consolidation occurred. It began in the left thumb and spread to was revealed throughout the right lung. There was implicate the entire left upper limb and the left side of evidence of a generalized arteriosclerosis. All other the face. Several such episodes occurred on the third significant post-mortem findings were confined to the and fourth days. They then regressed and were not brain. Some residual subpial hvmorrhages and localized J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.13.2.115 on 1 May 1950. Downloaded from

SURGICAL TREATMENT OF HEMIBALLISMUS 123 swelling were noted in the gyri adjacent to the line of microglia were present but on the whole the glial elements, cortico-subcortical incision. The i-nterface of the like the neurocytic, appeared devitalized (Fig. 7, a and b). incision was well agglutinated. In depth, it closely All portions of the right corpus subthalamicum approximated 2-5 cm. throughout its course. The exhibited well-preserved ganglion cells, normal glial operative site was not otherwise remarkable. elements, and granular ground substance (Fig. 8). The ansa and fasciculus lenticulares F ....;...,.:;,.,..i .:: ..... disclosed moderate degrees: of -' j'¾ demyelination traceable into the ventromedial nucleus of the thalamus, the reticular formation .. .,%{ ,,|. of the anterior midbrain and the 4%_\ substantia nigra. Comment.-The absence of

.. pathological changes in the ,.s %. _. corpus subthalamicum and the \ ... circumscribed character of the \ } -4: pathological findings in the s i: :. b. .: stv_ e F lenticular nucleus opposite the s F: # side of the hyperkinesia renders l. .. o.lit :; this a critical case. It may of F w: course be denied that this was a genuine example of hemiballis- 'Fr .A_,S,,f. _$rt '. mus; if so, criteria other than those set forth above are in need of definition. If, however, the FIG. 5.-Gross coronal section of brain of Case II at level ofmammillary bodies case be accepted as one of hemi- Protected by copyright. (seen from behind), showing encephalomalacic shrinking and obscuration ballismus, it follows that lesions of structures in and neighbouring upon the right lenticular nucleus, external lying outside the corpus Luysi capsule, and claustrum. The corpora Luysi appear normal. may subtend the disorder, a circumstance which in its turn The brain was suspended in formalin for a week. suggests that the inteZgrity of a neural circuit, rather than Coronal sections were then cut at intervals of approxi- a particular structuire (e.g., the corpus Luysi and its mately 0-5 cm. The was moderately centripetal and centi:rifugal fibres) is normally required dilated. The gross sections revealed only one lesion: to suppress hemiballiismus. a brownish-purple, granular-appearing discoloration within an obviously shrunken lenticular nucleus (Fig.5). Discussion This finding was most intense in those portions of the Bucy's case and tthe two cases cited above demon- putamen and both crura of the strate that by the use of relatively simple surgical appearing in the coronal section through the mid- portion of the mammillary bodies. The internal and external medullary lamine were almost wiped out. The external capsule and claustrum were likewise obscured and the appeared http://jnnp.bmj.com/ to be but slightly involved. More rostrally and caudally, the lenticular nucleus exhibited less intense pathological changes. The corpus subthalamicum exhibited no gross changes in colour, size, shape, or texture (Fig. 6). Microscopic study of serial sections alternating through Nissl, Weil, and Masson stains confirmed the gross pathological findings. The right putamen and globus pallidus were the seats of well-established on September 30, 2021 by guest. encephalomalacic changes. Their neurocytes were in various stages of disintegration. The less severely affected cells exhibited chromatolysis, swelling, and nuclei in eccentric positions. The more severely affected cells appeared as mere smudges. Many cells had evidently completely disappeared. No in- FIG. 6.-Gross coronal section ofbrain of Case II seen from flammatory cells or vascular proliferative processes in front at level juist anterior to emergence of oculo- were discernible. The heterogeneous appearance of motor nerves show]ring apparently intact corpora Luysi the ground substance betrayed its general dis- and substantia nigrra. Arrow points to the side of the organization. A few gemistocytic astrocytes and damaged------right lentilicular nucleus. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.13.2.115 on 1 May 1950. Downloaded from

124 RUSSELL MEYERS, DONALD B. SWEENEY, AND JESS T. SCHWIDDE A

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.. ;i~~. 9 ,~~~~~~~~~~~~~'. I? FIG. 7.-Photomicrographs f ~ ~~~~~~~~~~~~,-l 4 *# b:~~~~~~~~~~~~~~~~. of putamen (A) and X Ss:. : q crus II ofglobuspallidus * > X \ S I*o '11 a 9. ?. : (B). The neurocytic * J and glial elements ex- .] q X u hibit various stages of A 1. 0 .1. devitalization in con- w -W. fs b # sequence of encephalo- ag glg | 11? ml 4 4 .E4'. ak !v. et a.f jja sz 4, malacia presumably s..... t S.@ *~~~~~~~ 449. i4 9 secondary to athero- ¢ :.: .'a sclerosis. A con- t: siderable outfall of x J: * # - I%1 4* . 4 neurocytes is apparent -: :. o.% r. b4 W4, 9.s *4 9 and the ground sub- I *9 stance of the globus .: . pallidus reveals patches # :s * 44.~~ ~ ~ ..4< of early coagulation necrosis. (Nissl stain.) .499 '.4 v .e. .. ;, -* S JS :: T. V .:bi 1 944 t.4: -

-P .4.94 -low ., ..A.. -s 4 or' 0: J, ..' 4. .. '9 ik 5t, I Protected by copyright. R .Isom iml mr.. Kum' 'As J 0

-w procedures ballistic move- :s P~~~~~~~~W ... k ments can be _ Y4:: promptly .,Gk . 'k 1t :A

'O. 4:.: arrested, or so signifi- * .- 4J cantly suppressed as to v . .l afford gratifying relief and. I thus avert exhaustion. In r view of the refractoriness of hemiballismus to con- servative therapy, the e. negligible prospect of spontaneous recovery, the severity of the , and the frequency with which the illness ends http://jnnp.bmj.com/ fatally, it seems desirable to recommend early surgical intervention. It must be acknowledged that by no means all or even a large majority of cases of hemiballismus can be saved by surgical on September 30, 2021 by guest. intervention. Pathological inquiry clearly reveals that in some instances the to urge operation for all cases on the ground that responsible lesions are progressive in nature, coming this provides the only effective therapy at present ultimately to involve those mesencephalic and available. diencephalic structures upon which life itself depends. In yet other cases multiple lesions Summary develop. Though surgery appears to offer most 1. The excessive, almost ceaseless movements of promise in those cases where there is a well-circum- hemiballismus commonly lead to exhaustion, scribed, non-progressive lesion, it seems reasonable cardiac failure, and/or pneumonia. The disorder J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.13.2.115 on 1 May 1950. Downloaded from

SURGICAL TREATMENT OF HEMIBALLISMUS 125

I * ' * *9*t ,~ i * 0 * 9 *** 4,~~ ~ ~ ~ ~ ~ * E~~~ ~ ,,s 'o9q L . *;X . 9& '* *"X skt''9*0-^# ' -' * 's '; FIG. 8.-Photomicro- *a oo ' i i #** * t = ' * _ graph ofright corpus * m9' ''S 8 999 t* , .. - ' ' , .S' Luysi showing the integrity ofits neuro- cytic and glial com- ponents. All parts AS~~ ~ ~ ~ of the nucleus were lb* ~ 9 4 examined and none showed evidence of 4~~P~ 4' 9,, '*~70 Is9 pathological change. d.~~~~~~~~~~~~~- .~S9. (Nissl stain.) 'p~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~4 ;NL~ ~ ' , 4 . 9' *

s I, ~ i .. --7 v.~~~~ 44 1: *r ,b A * %. & I9 * St < & 1% _'. .i 5o Protected by copyright. thus complicated almost always terminates fatally Bucy, P. C. (1944). "The Precentral Motor Cortex" within a few weeks to several months of onset. (case 2). Pp. 361 and 404. Univ. Illinois Press. Civalleri, A. (1921). Policlinico, sez. med., 28, 242. 2. The lesions responsible for hemiballismus may Davison, C., and Goodhart, S. P. (1938). Arch. Neur. be located within the contralateral neostriatum, Psychiat., Chicago, 39, 939. paleostriatum, thalamus or postcentral gyrus as Delmas-Marsalet, P. (1925). " L'etude des fonctions well as in the corpus subthalamicum and/or its du noyau caude." These de Bordeaux, p. 159. and Pachon, V. (1924). C. R. Soc. Biol., Paris, afferent and efferent connexions. 91, 558. 3. Spontaneous cessation of hemiballismus is Fischer, 0. (1911). Z. ges. Neurol. Psychiat, 7, 463. rare and treatment by physical measures, drugs or Fragnito, O., and Scarpini, V. (1926). Riv. pat. nerv. peripheral surgical procedures has proved to be ment., 31, 524. Greiff(1883). Quoted by Lea-Plaza, H., and Uiberall, E. disappointing. On the other hand, "central" (1945). neurosurgical procedures have afforded relief in a Grinker, R. R., and Bucy, P. C. (1949). " " few instances. Two cases subjected to linear (Ed. 4), p. 315. C. C. Thomas, Springfield, Ill. cortico-subcortical section just anterior to Area 4 Hayne, R., Meyers, R., and Knott, J. R. (1949). J. Neurophysiol., 12, 185. are reported. This procedure appears to produce Jakob, A. (1923). " Die extrapyramidalen Erkran- http://jnnp.bmj.com/ less neurological deficit than does cortical ablation. kungen." Berlin, J. Springer, p. 183. 4. The " central " neurosurgical procedures (cor- Jermutowicz, W. (1931). Rev. neurol., 55, 374. tical extirpation, linear cortico-subcortical section, Kelman, H. (1945). J. nerv. ment. Dis., 101, 363. Kulenkampff, D. (1938). Zbl. Chir., 65, 2466. and midbrain pyramidotomy) constitute the only Lea-Plaza, H., and Uiberall, E. (1945). Rev. meid. Chile, definitive treatment upon which reliance may be 73, 938. placed for arresting ballistic movements. In view Lewandowsky, M., and Stadelmann, E. (1912). Z. ges. of the grave prognosis of the disorder, surgery Neurol. Psychiat., 12, 530. Marcus, H., and Sjogren, H. (1938). Rev. neurol., 70, 1. should be regarded as a semi-emergency measure. Martin, J. P. (1927). Brain, 50, 637. on September 30, 2021 by guest. and Alcock, N. S. (1934). Ibid., 57, 504. Matzdorff, P. (1927). Z. ges. Neurol. Psychiat., 109, 538. REFERENCES Mettler, F. A., and Carpenter, M. B. (1949). " The Austregesilo, A., and Borges-Fortes, A. (1937). Rev. modification of subthalamic hyperkinesia in primates" neurol., 67, 477. (to be published in Trans. Amer. neur. Assn.). and Gallotti, 0. (1924). Ibid., 1, 41. Meyers, R. (1941). Res. Publ. Ass. Nerv. Ment. Dis., Bertrand. I., and Garcin, R. (1933). ibid., 2, 820. 21, 602. Bianchi, L. (1909). Ann. Nevrol., 27, 1. (1942a). N. Y. St. J. Med., 42, 535. Bonhoeffer, K. (1897). Mschr. Psychiat. Neurol., 1, 6. (1942b). Ibid., 42, 317. (1930). Ibid., 77, 127. , Hayne, R., and Knott, J. (1949). Journal of Browder, J. (1948). Amer. J. Surg., 75, 264. Neurology, Neurosurgery and Psychiatry, 12, 111. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.13.2.115 on 1 May 1950. Downloaded from

126 RUSSELL MEYERS, DONALD B. SWEENEY, AND JESS T. SCHWIDDE Moersch, F. P., and Kernohan, J. W. (1939). Arch. Vogt, C., and Vogt, 0. (1920). J. Psychol. Neurol., NeuroL. Psychiat., Chicago, 41, 365. Lpz., 25, 627. Morgan, L. 0. (1927). J. Comp. Neurol., 44, 379. von SAntha, K. (1932). Z. ges. Neurol. Psychiat., 141, Papez., J. W., Bennett, A. E., and Cash, P. T. (1942). 321. Arch. Neurol. Psychiat., Chicago, 47, 667. Whittier, J. R. (1947). Arch. Neurol. Psychiat., Chicago, Schaller, W. F. (1937). Ibid., 37, 983. 58, 672. Schob, F. (1920). Dtsch. Z., Nervenheilk., 65, 210. Wilson, S. A. K. (1914). Brain, 36, 427. Thurel, R., and Grenier, J. (1947). Rev. neurol., 79, 502. (1929). Dtsch. Z. Nervenheilk, 108, 4. Touche, M. (1901). Ibid., 9, 1080. Zonnoni, U. (1908). Gazz. Osped., 29(2), 1156. Protected by copyright. http://jnnp.bmj.com/ on September 30, 2021 by guest.