NEUROLOGIC STATUS IN THE LONG TERM FOLLOWING
COMPLETE CEREBRAL COMMISSUROTOMY
by
Joseph E. BOGEN,M.D. and Philip J. VOGEL, M.D.
White Memorial Medical Center, Los Angeles
[{This paper originally appeared as a chapter in the book Les Syndromes de Disconnexion Calleuse chez lHomme, F.Michel and B. Schott(eds.) Hôpital Lyon,1974. A few comments have been added ,enclosed in double brackets,straight and curly, in1999 and 2000}]
I Introduction
11 Case Summaries With Recent Neurologic Examinations
111 Discussion of the Neurologic Findings
IV Concluding Comment
I Introduction
The usefulness of cerebral commissurotomy as a treatment for epilepsy (1,2,3) depends not only on its effectiveness in reducing the frequency and severity of seizures; it is also necessary to consider the psychological and neurological deficits which are consequent to the operation. These are of two main types: those which can be expected in every case, and those which occasionally occur as complications, and for which the risk must therefore be considered in terms of each individual patient.
The psychological effects have been extensively studied under the direction of Professor Sperry and have been published voluminously-the
single reference to which we most often refer is the article in the Dutch Handbook of Ciinical Neurology (4).
In this paper I will present the results of neurological examinations carried out as part of a recent long term review of our results. Most of the examinations reported here were done in 1973; the shortest postoperative followup is four years, the longest ten years. For each patient there is first presented a summary of the clinical history; this is followed by an account of recent neurological examination. The paper concludes with a discussion based on the findings which typically persist in the long term after a complete surgical section.
Before presenting the individual cases, it will be helpful to describe briefly the general nature of the findings.
The most notable feature of these patients is the essentially normal .
appearance which they present in the ordinary social situation. By contrast,when special testing is done, a wide variety of disconnection deficits can be elicited. Most of these special tests have been carried out in the laboratory setting. However, some of the deficits can be readily demonstrated in the course of a neurological examination, if the examiner employs the appropriate maneuvers.
There are certain findings present in all patients with complete cerebral
commissurotomy; these have the widest theoretical import. Notable among these is an anomia in the left hand-with the exception that the monomanual anomia occurred in the right hand of our single strongly lefthanded patient.
The anomia was found in all of these patients and has been quite persistent over many years. Another finding which uniformly appears and indefinitely persists is a double hemianopia. By this I mean that the left hemisphere has a left halffield defect and the right hemisphere has a right halffield defect.
Other deficits are less uniform In their occurrence or in their persistence; when they do persist, they reflect individual differences among the patients.
~Although these less regular findings have a narrower significance with respect to our understanding of cerebral physiology, they are nevertheless of importance from the clinical point of view. One example of this is the dyspraxia of the left hand for verbal command which continues to be a significant residual in several patients, these being in general the patients who had considerable righthemisphere damage before operation.
Another finding of less uniform appearance is the extent to which crosscueing permits the patient to compensate for the double hemianopia which is such a universal feature ofthe surgical disconnection syndrome.
Examples of the above findings will be given in the case descriptions to follow. Some of the terms and tests used in the examinations are described in appendix 1.
11 Case Summaries with recent neurologic examinations.
The Patient W. J.
W.J. (born 12 Dec 1913) was a thirty year old man of normal development and above average intelligence at the time he suffered muitiple head injuries in World War II. A parachute jump in 1944 resulted in fractures of the left leg, hematuria and unconsciousness of about two days. While in prison camp, he was rendered unconscious by a riflebutt blow to the left parietal reglon.
Following his return home he was plagued by " lost time" ; he once drove from home one morn ng and arrived 5 miles away that afernoon with no recollection of the intervening events.
n l9Sl, he had his first convulsion. Thereafter, the convulsions increased in severity and frequency in spite.of a variety of medications.
He was first seen at the White Memoriai Medicai Center (WMMC) in 1956.
Electroencephalograms (EEG )showed a left temporoparietal focus as well as bitemporal abnormaities. A left carotid angiogram was normal as was a pneumoencephalogram. In 1961 the patient was studied for six weeks in an East Coast research hospital [{the NIH Hospital, care of Dr. Maitland Baldwin}] specializing in epilepsy.
He was told that removai of the left convexity focus was inadvisabie because of the risk of aphasia, and because of evidence for other independent foci. [{ He was told that no further treatment was advisable and was quite depressed on his return to Calif. See chapter 24 in Greenblatts A History of Neurosurgery, 1997 }]
W.J. had a complete cerebral commissurotomy on 6 February 1962 (at age 48). Distinct atrophy of the right frontal lobe was observed at operation. His case has been described in greater detail elsewhere ( 1). After recovery from operation, he continued free of convulsions, led an active soc~al life, but remained on a full disability pension.
Whereas before operation status epilepticus occurred every 2 to 3 months, requiring hospitalization or intravenous medication in the emergency room, no such episode occurred after operation. Whereas major seizures (with unconsciousness, apnea and severe clonism) occurred almost every week before operation, there were only 2 episodes of complete ioss of consciousness in the first postoperative year and none subsequently. Brief dreamy states, largely unnoticed before operation, continued. Muscular phenomena were limited after surgery to mild shaking or stiffness of the left side lasting a minute or two.
In addition to epilepsy the patient had cardiomegaly with coronary atherosclerosis, and scleroderma. The latter disease affected not only his hands but also his liver and lungs; as a consequence he had one or two bouts of pneumonia every winter. His last seige of pneumonia was in January, 1973 and was the cause of his death on 20 January 1973. The anatomical examination of the brain, by Professor B. Campbell, has not yet been published. [{The brain was subsequently ruined by a novel processingmethod-a disappointing outcome indeed{[
Our last neurological examination of this patient was in September, 1969 just before he moved to Utah. Significant findings included a persistent dyspraxia of the left hand and a double hemianopia on examination of visual fields. The following is extracted from the examination:
<< He has the usual apraxia in that he is unable to hold up the appropriate finger on the left hand unless he has previously done it with both hands at the same time; in fact, he can not do it even after he has just done It with the right hand although on previous occasions this sequence has often succeeded. When asked to put both hands on the top of his head he does it quite readily. After he put his hands back in his lap, he was then asked to put the left hand on his chest; immediately after the instruction his left hand went up to the top of his head. In other
words, the apraxia [{appearing here as a sort of perseveration}]affects his arm as well as his fingers. In contrast, he usually succeeds when
asked to make a fist with the left hand... The patient seems definitely better [{than when previously examined]}so far as locaiization of touch is concerned in that he localizes a point fairly well on the left side of the body so long as a reasonable amount of pressure is used rather than touch; but he completely ignores any stimulus on the left side of the body (other than pain) when this stimulus is applied simuitaneously with stimulation on the right side... He seems to have a dense left hemianopia when using his right hand to indicate the presence of a stimulus (both eyes being open and his left hand restricted by having him sit on it). But when his left hand is free, he almost invariably reaches with it toward stimuli in the left visual field.
The Patient N. G.
N.G. (born 29 June 1933) was premature (in sixth gestation month), was in an incubator for some weeks, and on ieaving the hospital weighed five pounds (2300 grams). In spite of this, she was considered normal in development until she began having convulsions at the age of eighteen.
Her first convulsion was in June 1951, when she was four months pregnant, Her blood pressure was then 170/llOmm Hg. She had a miscarriage. Her blood pressure returned to normal, but she continued to have seizures.
She was first admitted to the White Memorial Hospital on 6 April 1952, having had a total of eight known convulsions. Each of these was observed by her husband, who was awakened at night by a strange sound to find his wife " stiffened out " for two or three minutes. Examination
was unrevealing. Chest xrays showed calcifications near the right hilum. The EEG showed [{focal]} left temporal slowing. The skull xray showed a muiberry calcification, 1 cm in diameter, beneath the right central cortex. A right carotid angiogram was normal.
Her condition worsened as described previously (2) and she had a complete cerebral commissurotomy on 5 September 1963 (at age 30).She needed help from her husband the first year, subsequently assuming full management of her household. Several generalized seizures occurred in the hospital shortly after operation when anticonvulsant medication was withdrawn but none subsequently for eight years. During the ninth year, while medication was being reduced the patient had a tooth infection coincident with the appearance of status epilepticus. In the hospital, the EEG which had been essentially normal for seven years showed a left temporal focus.
Anticonvulsant medication was reinstituted to the original levels. The EEG again returned to normal (in less than one year), and the patient has had no seizures of any kind for two years. [{There have been some fleeting episodes of right sided numbness but no definite seizures since, still on medications]}
The Examination of 12 September 1972:
~ She walks, talks and stands in an essentially normal manner. She can walk well either on her toes or on her heels and needs no external support to keep her balance while doing this. Finger-to-nose testing is done excellently with the eyes closed and the feet together; it is of interest that although instructed to use her index finger, she used the middle finger on her right hand and the index finger on the left, an asymmetry which is almost unknown to me in thousands of consultations. There is a good grip with a negative Wartenberg sign bilaterally.
Deep tendon reflexes are essentially normal at the biceps, triceps, knee and ankle, except that the left knee and ankle jerks are just detectably more brisk than the left. Plantar reflexes are definitely flexor. Vibratory sense is excellent at both ankles.
The face is generally symmetrical in movement with respect to both voluntary and involuntary motion with one exception; when a voluntary motion is requested, such as a smile or a grimace, which involves the mouth, the right angle of the mouth rises just before the left angle
of the mouth, and this very slight delay in the motion of the !eft side was repeatedly present. The routine for facial apraxia was carried out, the only deficit being an inability to wink with the right eye.
No deficit is elicited in left hand responses to verbal command. There is the usual deficit of transfer of passively imposed hand postures.Errors on the test for crosslocalization of fingers
are about 50% in each direction with no errors for samesided localization.
.In the left hand, with the eyes held closed, a safety pin couid not be identified, a dime was called "cotton ", a paper clip was called "a rubber band", and a pencilclip was called "a pencil ". These same objects were presented to the right hand with the eyes closed a short time
later (not immediately after) and the first three items were correctly named immediately whereas the last item was described as "a clip ? some kind of a tack ?". When she was ailowed to
examine this with her eyes open she said, "Oh, that must be some kind of clip which you put on a pencil ".
Visual acuity in the right eye uncorrected is 10/13 and in the left eye uncorrected 10/25. The extraocular movements are full. The pupils are normal in reactivity, the left pupil in dim light is just perceptibly larger than the right, being 6 mm as opposed to 5 mm. She can stereo to 100''(uncorrected). The fundi are normal.
Testing of visual fields by confrontation demontrates a reliable, dense hemianopia on the left, ending abruptly at the midline. This was retested subsequently and in all of the testing which was done, there was only one occasion on which finger movement in the left field was recognized. On this one occasion, the recognition was proceded by a quick dart of the eyes to the left. On no occasion when simultaneous stimulation was given in both fields was there any apparent awareness of the stimulation in the left field.
It is noteworthy that on conclusion of the examination, the patient redressed and then turned left instead of right when leaving the examining room, and was somewhat bewildered in the hallway. Her husband noted that she still tends to get lost, particularly in unfamiliar
surroundings. She does surprisingly well on the faces tests, poorly on Street and overiapping figures, and got 15 correct (of 16) on the token test.
The Patient A. M.
A.M. (born 26 June 1933) was considered normal untii the age of fifteen when, coincident with a head injury, he fell into a swimming pool and was apparently anoxic for a time. He did not return to schooi but helped his father at glass polishing for a while. Within a year he began having convulsions, beginning with twitching of the right side of the face. As the convulsive disorder worsened, he also gradually developed severe incoordination of the legs so that by the age of 25 he was confined to a wheelchair.
When seen in June, 1964, he appeared mentally retarded with a poor memory, but alert and cooperative; with either hand he could write legibly and could copy various geometric figures. There were multiple scalp scars, and an ataxia of the legs associated with hypoactive reflexes and impaired position sense. EEGs showed a severe generalized abnormality. A pneumoencephalogram showed marked cerebral atrophy, most evident biparietally, more so on the right side. A bilateral carotid angiogram was normal.
A.M. was operated on 7 July 1964 (at age 3 l ). He had a rather stormy postoperative course but gradually improved. However, he has had a persistent apraxia of the left hand and some degree of mutism ever since.
Since operation, his seizures are much less severe. Spells of unilateral stiffening of the right side, occasionally with unresponsiveness and rarely with incontinence, have continued in great profusion. Generalized convulsions have occurred on a few occasions when medication was decreased or omitted.
He was essentially speechless for several years after surgery, although he could write short sentences (5). More recently, speech therapy has seemed somewhat helpful. He rareiy speaks spontaneously and when he does speak, it is in a hoarse voice with poor articulation. He remains affable and interested in watching TV which is his principal occupation. He remains confined to a wheelchair as before operation.
The Examination of 26 February 1973:
He was pushed into the office in his wheelchair and seemed somewhat dour: but he readily extended his right arm to shake my hand when it was offered. He shook his head when he was asked If he could say " hello ", but when he was asked " how are you ? ", he smiled and
nodded his head . I then asked, "can you say O.K. ? " and he then replied "0h Kay" in a loud, uncontrolled and very hoarse voice. This mutism is the most important neurologic deficit; its interpretation is dependent to some extent upon the subsequent examination which shows that he understands quite readily and also that he has good control of his tongue and lips, the principal deficit apparently being in voluntary control of his larynx. This conclusion is supported by
the following results of the facial apraxia routine:
Can you close your eyes?"- the patient opened his mouth in a rather grotesque manner ! When he was asked if he could imitate the examiner in closing the eyes quickly, he did so without hesitation or error.
"Can you wink with your left eye ?"- this was done fairly well (2 plus out of four).
"Can you wink with your right eye ? "- this was done even better!
" Can you ciose your eyes ? "- this was done promptly and perfectly.
<< Let's see you close your eyes again ? >> on this request (the third for this movement) the response was the same as on the first, a bizarre opening of the mouth and a rolling about of the tongue inside a rather cavernous aperture.
" Can you open your mouth ? "- his response was to open it very wide as before, again with the tongue rolling about inside, in a somewhat grotesque fashion. a"Can you close your mouth ? ~"- the patient opened and closed his mouth quickly.
" Can you grit your teeth ? "- the patient's response to this question was the previously described wide opening of the mouth with moving of the jaw to some extent from side to side and bizarre rolling about of the tongue.
" Can you click your teeth three times ? "-the answer was the same grotesque mouth opening.
a"Can you show me your teeth ? "- the patient began with the usual cavernous opening of the mouth and then suddenly grimaced, baring his false teeth briefly.
" Can you make a smile ? "- the patients response was the same mouth opening described above; however when I then smiled at him he smiled very broadly and quite naturally with no apparent deficit
" Can you stick out your lips?- the same wide openfng of the mouth occurred , interrupted briefly by some poutng which was transient but occurred on two separate occassions, mixed in with a variety of other facial grimaces.
"Can you whistle ?"- in response to this the patient vocalized rather loudly making a sound which sounded like a retch. He then tried to stick his fingers in his mouth in order to whistle, but was unabie to do so and laughed in a slightly hoarse fashion, shaking his head.
"Can you cough ?"- the patient made the previous retching noise. I then demonstrated for him with my hand in front of my mouth. He put his hand in front of his mouth and made several attempts producing only a slight clearing of the throat, but at no time a distinct cough.
" Can you puff out your cheeks ?"- this question resulted in a whole series of bizarre facial gestures and movements including the previous rolling about of the tongue and widely open mouth, pouting of the lips, swinging of the tongue from side to side, grimacing and in the course of this he came to a position in which there was some puffing of both lips, containing air, but not the cheeks, and at this point he stopped, nodding his head briskly and his facial movements ceased entirely.
" Can you stick out your tongue ?"- the patient made a sort of retching sound and suddenly stuck his tongue straight out completely and perfectly.
" Can you swing your tongue from one side to the other ?"- this request was obeyed quickly and completely.
"Can you lick your lips all around ?"- this was not done as smoothly as normal, but was quite good, with some perseveration, and was rated 3 plus out of 4.
"Can you say yes?"- In reply to this the patient uttered a hoarse " yeah".
Aside from the mutism, the patient's principal deficit is an imbalance which makes it impossible for him to stand for any length of time unaided. Furthermore he has some titubation while sitting on the end of the examining table. There is a gross dysmetria with the right arm on finger-to-nose testing, but there is never any significant tremor. The left arm could not be tested satisfactorily because of the apraxia. In spite of the apraxia of the left arm, he was able to obey some commands with both legs and in moving the heel to his knee, and then down the shin, there was a distinct ataxia on both sides, the right leg being slightly worse than the left.
The most interesting finding.in the entire examination is the frequent occurrence of well coordinated movements of the left arm which are at crosspurposes with whatever else is going on. These sometimes seem to occur spontaneously, but on other occasions are clearly in conflict with the behavior of the right arm. For example, when attempting a Jendrassic reinforcement, the patient reached with his right hand to hold his left, but the left hand actually pushed his right hand away. While testing finger-to-nose (with the patient sitting), his left hand suddenly
started slapping his chest like Tarzan.
The patient follows verbal instructions readily with the right hand, but poorly if at all with the left hand. However, he can raise and lower the left arm with considerable reliability, the defect being more obvious the more distal are the joints involved in the requested act. After a variety of maneuvers (the left hand often following a visual demonstration fairly well) it was possible to test the grip of the left hand while keeping the thumb extended so it was apparent that there is a
negative Wartenberg sign on the left as well as on.the right.
Deep tendon reflexes at biceps, triceps, knee and ankle were just barely obtainable bilaterally.
No ankle jerks could be elicited. The plantar reflexes were distinctly flexor bilaterally. Vibratory sense was intact at both ankles, some time being given to this part of the examination in order to be
certain of the findings. (The usual method was used in which the tuning fork was applied to the examiner's thumbnail, the thumb gripping the ankle bone tightly so that there was no cue from the presence or absence of a tactile or pressure stimulus, the only change in stimulation being the vibration.
The patient wears glasses;using these, he has fairly good eyesight; he was able to read small print as evidenced by following written commands (with right arm and hand only); such commands as" Put your hand on your head " or "Take off your glasses ". No stereo ability was present The pupils were normal in size and reactivity. The optic discs were sharp. The extra ocular movements had full range of motion, but there was a prominent nystagmus: this was a vertical nystagmus with the quick component downwards. The nystagmus was continuously present at a fairly slow frequency, beating about two per second; the frequency increasing detectably on right lateral gaze. Auditory acuity (tested by finger rub) appeared to be good bilaterally.
He made seven errors on the token test. It was also at this time that the patient was given the Street, and when verbal replies were required he was able to name only the second item. However, when he was given a multipie choice (Peabody pictures), he obtained seven out of twelve correctly, which is a remarkable score considering his retardation, not only with respect to speech but with respect to other intellectual attainments. He was similarly surprising in his performance on the Mooney faces, where he made only eight errors in the 24 faces, quickly and unerringly pointing out the various facial parts of all of the other items.
The Patient A. A.
A.A. (born 25 November 1950) was his mother's first child, The labor was induced because of toxemia, and he was delivered by forceps fourteen hours later. At the age of four months he had two probable convuisions associated with fever. But he was thought to be developing
normally until age five and a half when generalized convulsions appeared, sometimes beginning with stiffness or "spasms" in the right hand and arm. He began to fail in school in the fourth grade. Medical treatment did not control the seizures; a fractured clavicle and a number of head
injuries were sustained during the year before operation. EEGs showed bilateral abnormalities, usually more marked over the left hemisphere. Pneumoencephalogram and bilateral carotid .angiogram were normal .,
He was operated on 14 October 1964 (near age 14). The operation was difficult and included interruption of two large bridging veins, causing right cerebral swelling. There has persisted since operation a spastic left leg with a positive Babinski sign; but his left arm recovered well. [{ this was eventually the basis for a malpractice suit in the course of which we were not allowed by our lawyers to contact the patient. Contact resumed ,as if nothing unusual had transpired, about three years later, after the suit was settled}].
He subsequently attended schools for the handicapped where he made a good adjustment. On quantitative testing by Dr. B. Milner, three years postoperatively, A.A. was found to have a twopoint threshold of 18 mm on the right palm, as compared with a normal threshold of12 mm on the left. This, together with a slight impairment of position sense in the fingers of the right hand, provides further evidence (together with the seizure pattern) for a lesion involving the left perirolandic cortex. The patient has not been available for formal neurologic examination since 1972. [{He was next seen in 1975 and has been followed since}].
The Neurologic Examination of 2 September 1971:
<< The patient was seen today in the office and is his usual very pleasant and agreeable self. He is spending most of his time in social activity, going out ", as he says. He had a great many seizures on the day following his mistaken arrest by some policeman, but since then has done fairly well.
At present he walks well though favoring his left leg where there is some residual foot drop. He is actually doing quite well, without the shortleg brace which he used to wear. He is alert, cooperative, well oriented and has no detectable speech impairment.
The deep tendon reflexes are normal at the biceps and triceps, whereas the left knee jerk and left ankie jerk are distinctly more active than on the right. There is a distinctly positive toe sign on the left, whereas the plantar reflex is normal on the right. There is excellent vibratory sense at both ankles. There is a bare trace of Wartenberg sign bilaterally.
No abnormality is present of the pupils, extraocular movements or fundi. In testing the visual fields, it was noted at first that he would not indicate a stimulus in the left field when using his right hand unless this was immediately preceded by a sudden shift of the eyes to the left. But then subsequently he wiggled his fingers with a stimulus in either field while using only his left hand, so it appeared that there may be some ipsilateral control. On furtner testing the only deficit appeared to be an extinction in the left field when the patient was asked to indicate whether the stimulus was on the right or the left and it was actually both. However, on further testing, there seemed to be an occasional extinction in the right field. At the present time, the patient appears
to have developed several compensatory methods for obviating the double hemianopia but these break down with repeated testing and are most evident with unexpected simultaneous stimulation.
He has the usual intermanual transfer deficits for passively imposed
postures. Repeated attempts were made to demonstrate some dyspraxia in the left hand, but none was found. He has a distinct anomia in the left hand except for three items: a pencil (which he detected in his usual manner by pressing the point with his finger) and a bar of soap (which had a faint but typical odor) and a large eraser (how he detected this is not certain, although he bent it back and forth between his
fingers several times before naming it).
The Patient C. C.
C.C. (born 10 December 1951) ~ turned blue from mucus ~ on several occasions postpartum, but after that seemed well until age eight when he started having fights and began falling behind in school. At age ten his family first noticed spells of speechlessness associated with turning of
233
tne head toward the right. These spells occasionally included a fall and transient loss of consciousness. C.C. says that all his seizures begin with a feeling of pain over the right eye. His head and eyes then deviate to the right and he is unable to talk, although he can often understand what is said to him. Occasionally his body also turns to the right and then he may lose his balance and fall to the ground.
In spite of medication, both the frequency of the seizures and the severity of antisocial behavior progressed. On the verge of institutional commitment he was admitted to White Memorial Medical Center for study. There were no positive neurologicai findings. EEGs showed
spiking over the left hemisphere, more marked in the temporoparietal region. Pneumoencephalogram and bilateral carotid angiogram were normal. He had a cerebral commissurotomy on 27 March 1965 (at age 13). The commissurotomy, which was preceded by electrocorticography of
the left convexity [{C.C. Was the only pt. of this series to have this procedure}] was exceedingly difficult because of the unusual head position; there was consequently excessive retraction and manipulation of the left hemisphere. The patient was mute for over three months. After returning home he did fairly weli in a private school. More recently he has been employed as a stock clerk, but has had frequent problems
because of temper outbursts. He has often threatened coworkers but never been involved in a physical assault. Seizures involving the right side, often with right adversion, have continued.
The Examination of 15 June 1973:
"The patient walks, talks and stands in a normal manner It is noted that he has an obvious mental retardation although he is quite affable and is able to do surprisingly well on certain subtests such as the overlapping figures test. He got all but four correct on the token test whereas he is barely able to answer any questions on the Similarities or the Street.
The patient walks well on his toes and also on his heels. He does finger-to-nose test excellently with his feet together and his eyes closed. There is a strong grip with negative Wartenberg sign bilaterally. There is no facial apraxia: his one error (" can you stick out your lips ? ") vvas probably ascribable to a failure to understand. He pouted readily when shown by demonstration what was expected.
Apraxia in the left hand is tested as follows: { Make a fist "- done correctly.
" Stick out your thumb "- done correctly. "Make a fist "- this was done correctly.
"Stick out your little finger "- the patient stuck out the thumb and index finger of his left hand (which was in a fist) and the patient, immediately as this happened said, "No ,the little finger " and the thumb and index were immediately pulled into the fist and the little finger was extended.
"Put your littie finger and thumb together to make a circle "- the patient said, "I can't do that". He was told, "Yes, you can.". He then did it correctly on the first attempt.
"Open your hand "- done correctly.
"Stick out the ring finger on your left hand ".- the patient held up his right hand and said, "Here is my ring finger ", meanwhile wiggling the correct finger of his right hand which has a ring.
"Yes, hold up the same finger on the other hand "- this was done correctly.
It is apparent from the above that the patient may be said to have a minimal dyspraxia although there appears to be no specific movement which he cannot follow readily given the appropriate instructions; most of the difficutty is apparently related to his inability to retain a complicated instruction for any length of time.
The deep tendon reflexes at biceps, triceps, knee and ankle are all normally active. The superficial abdominal reflexes are normally active bilaterally. With plantar stimulation, there is a minimal flexion on the left: in contrast, the right hallux briefly dorsiflexed on one occasion but thereafter gives no significant response. The Oppenheim maneuver produces no response except for a faint fanning on the right.
Thorough testing with the pinwheel elicits no deficit. There is. excellent vibratory sense at both ankles.
Visual acuity is 10/25 with the left eye and 10/15 with the right eye, both uncorrected. The pupils are normal in size and reactivity. The extraocular movements are full. The optic discs are sharp and bilaterally present rather pronounced cupping. He can stereo only to 400''.
The visual fields are notable for the fact that the patient indicated stimuli in the left visual half-field but not in the manner in which he was verbally instructed. He was told, "When you see the fingers move, point to them ." He was then told to look at the examiner's nose and before any stimulus could be applied he said, " Oh, the hands ", and he held both hands in readiness near his waist level. A stimulus (wiggling of the fingers) was then applied in the right visual half-field at which time the
patient pointed to these fingers immediately with his right index finger. When a stimulus was applied in the left visual half-field, the fingers of the left hand (which was held palm outward) were wiggled. Another stimulus was given in the right field and the patient pointed with the index finger of his right hand. When stimuli were present simultaneously in both fields the patient pointed in the proper direction with his right index finger and at the same time he wiggled the
fingers of his left hand, continuing this as iong as the[{examiners}] stimulus fingers were wiggling in the left field and then
stopped wiggling the fingers of his left hand immediately as the stimulus ceased.
It should be mentioned that the patient is distinctly obese, he was measured today and is six feet two inches tall and weighs 258 pounds.
The Neurologic Examination of 13 October 1973:
" C.C. was at Cal Tech yesterday and the testing concerned mainly memory function. His father says that his memory is very much better: he remembers all kinds of things from yesterday and the day before and the like, markedly better than his situation one year ago.
The patient has had no spells recently (apparently none of consequence since he was last in this office). In addition he has not been having headaches and moreover his tendency to impulsive shouting or threatening has also been in abeyance . It seemed to me that these all go together and before I could say this his father spontaneousiy said the same thing (that is, that seizures, headaches, and aggressive behavior all seemed to be linked in this case).
The patient was given a series of tests for facial recognition and, in marked contrast to his performance on verbal or other usual intelligence tests, he really did quite well.
The question of apraxia was reviewed and he has no difficulty saluting, placing his hand on his head, pretending to brush his teeth and the like. There is also no apraxia of individual movements of the individual fingers at the present time. Ipsilateral finger identification is invariably correct whereas cross localization is grossly defective.
With respect to anomia, the patient's performance is somewhat variable; it is remarkable how he can sometimes identify objects in the left hand on the basis of quite minimal cues. For example, when a pencil was placed in his left hand he was rather slow and deliberate about
answerng, turning the pencil over in his hand and pressing first on the eraser end and then on the: pointed end, and then saying, " It's a pencil ".
When a dime was placed in his left hand he said, "it's a penny "., and then said, "Well, isn't it?" ~ .
When told that he was not correct he said, "Maybe it's a dime"
When a rubber band was put into his left hand he first shook his head. However, after rolling it around and then maneuvering it into a position in which it looped over his thumb and opposing fingers so that there was some springy resistance to partial opening of the hand, he said, ,'"a rubber band ".
When a pair of glasses was put in his left hand his fingers rubbed the lenses, closed one bow with a snap, and he then said, `` Are these glasses?". When he was told that he should try again, he said, "Well, I don't know ". When this same object was then placed into his right hand he immediately said, "yes, it's a pair of glasses ".
When a pipe (still warm) was placed in his left hand he said, "is it a cigar ? ". When he was told that this was incorrect, he shook his head and said. "I don't know ". When it was placed in his right hand he immediately said, "it is a pipe ".
When a small paper clip was put into his left hand, he turned it over several times and finally said, "I don't know ". When it was placed into his right hand he immediately said, "It's a clip ". . He was then asked, "What kind of a clip ? ", and he replied, "To keep papers together".
.A small pine cone was placed into the left hand and he was at a total loss to say what it was. When this same object was placed into the right hand he said, "it's one of those - I don't
know what you call it- it comes from a tree ". When he was allowed to see it, he said, "Yes it is from a tree what do you call it ? " When he was told, "pine cone ", he said, "Oh yes, that's what it is".
From the above it is apparent that C C. readily recognized and named objects in the right hand except with the last object (the pine cone) which he could no more readily neme in full view than he could when palpating it in his nght hand. In contrast, obJects in the left hand were much less readily identified either by verbal description or by naming: any success in this regard apparently depended upon partial information which sometimes led to the correct answer and sometimes to an answer which was related but not correct. The use of the pain, from a pencii
point, is a cue which has been observed in a number of complete commissurotomy patients.
Identification of the rubber band apparently involved some proprioceptive information. The glasses provided a definitive auditory cue, and possibly a slight temperature cue. The identification of a pipe as a cigar is particularly interesting ; it is possibly ascribable to an
olfactory cue. Alternatively, his speaking left hemisphere may have received some sort of sparse categorical information through internal pathways. In any event, it is quite impressive how an
individual with an l.Q. of 70 can use minimal sensory cues to identify objects which are familiar to him.
The Patient L. B.
L.B. (born 15 May 1952) was his mother's third Caesarean delivery, he weighed five pounds, was cyanotic and remained in an Isolette for eight days. Childhood development was considered normal, except for persistent enuresis. His first convulsion occurred at age three and a half and these spells became intermittently worse. No neurologic abnormality was found on preoperative examination. EEGs showed mild, diffuse abnormalities. somewhat more evident bitemporally. Pneumoencephalogram (in 1956) was normal. Bilateral carotid antiogram was normal.
He had a complete cerebral commissurotomy (except that no search was made for the massa intermedia) on 1 April 1965 (near age 13). His postoperative course was particularly smooth (he spoke well the next day) and his progress continued since that time. He attended public school, doing passable work except in the "new math ". More recently, he has done well at onthejob training as a shop assistant.
The preoperative attacks were without lateralizing sign. Since the operation he has had only 7 major seizures and none for four years (compared with an average of one a week in the year preceding the operation) In the third year there were nine leftsided Jacksonian attacks which probably indicate some righthemisphere abnormality.
The Neurologic Examination of 13 August 1973:
"The patient walks, talks and stands in a normai manner. Because of his slight build he seems younger, and his behavior is somewhat adolescent for the age of twentyone. He does finger-to-nose testing well with his feet together and his eyes closed. There is a good grip with
negative Wartenberg thumb sign bilaterally. There is definitely no sign of dyspraxia in the left hand for verbal command. No errors occur in testing for facial apraxia.
No deficit is elicited with the pinwheel. Vibratory sense is excellent at both ankles. He definitely has an anomia in the left hand: he called a rubber band a "bobbypin ". When given a paperclip in the left hand he was quite unable to tell what it was. When a pencil clip was put in his left hand he thought for some time and said, "Oh, is this a pencil or something, it seems as if it is a pencil-is it cylindrical ? ". With all of these objects, he rapidly and correctly named the object when it was placed into his right hand.
Ipsilateral finger recognition is without error. Cross localization gave the usual 50% errors; his correct results are usually with the little finger of either hand.
Deep tendon reflexes at biceps and triceps are slightly hypoactive but within the normal range: the reflexes are normally active at the knees and ankle bilaterally. Superficial abdominal reflexes are quite brisk bilaterally as is the right cremasteric reflex but the left cremasteric reflex is absent. Plantar reflexes are flexor bilaterally.
Visual acuity is rather poor uncorrected, because of considerable astigmatism; but when he uses his glasses the visual acuity in the left eye is 10/20 and in the right eye is 10/10. There is a
mild left exotropia which gives him a slightly walleyed appearance. His stereo resolution without correction is ratherpoor(400''at 16inches = Shepard 15%) .When this was repeatedlaterwith his glasses, he was found to have normal stereoscopy (50''). The extraocular movements are full in
in all directions and there is no nystagmus. The pupils are normal in size and reactivity. The optic discs are sharp. Auditory acuity is good bilaterally. The tongue, jaw and uvula move in the midline.
Visual fields appear almost normal by confrontation. Stimuli in the right half-field are recognrzed immediately. Stimuli in the left half-field are pointed to almost as quickly as those in .the right field. When there was simultaneous stimulation he pointed to the right and then to the
left on the first two trials only; on all subsequent trials there was a pause, and then, instead of pointing to the right or left he said, ~"both". His performance on confrontation testing of visual fields is much better than the other complete commissurotomy patients in two respects: first, he said, "both" as would a normal person, at least on the later trials,instead of indicating first one half-field and then the other. The second and much more notable feature of his performance is
that his eyes do not dart towards the left when there is a stimulus in the left half-field; rather they maintain, throughout the testing, a straighahead line of gaze. Although a sudden saccade to
the left seems to be an important cue for other patients, making possible a verbal or righthanded identification of a left half-field stimulus, L.B. no ionger uses this technic. Apparently, he has acquired some other source for his information.
On the Street silhouette completion test he attained an average score, and he improved his score 1 l /2 point with the Peabody pictures. On the faces tests he did only fair on the Tzavaras short form (4 errors of 18) and about average (12 errors) on BentonVan Allen, but he did remarkably well on the Mooney (only 2 missed of 24). The overlapping figures performance is within normal limits (he verbalizes constantly) and he made no errors on the token test.
The Patient R Y. .
R.Y. (born 16 August 1923) was the last of eight children: there was no difficulty at birth, and he developed normally through age twelve.
At age thirteen he was struck by a car and was stuporous for about half an hour. At age sixteen he began having "spells"; according to his family, generalized convulsions began one year later. He was never employed on a regular basis, although he occasionally worked as a painter or mason lender.
The patient has never given a very clear or consistent account of his seizures, except that he often had a visual aura (some change in the appearance of things) suggesting a right posterior cortical origin. Preoperative EEG findings were nonlocalizing and the neurological examination was normal, as were air study and angiography.
He had a complete cerebral commissurotomy on 7 March 1966 (near age 43). He was talking the following morning, although speech tended to be monosyllabic and lacked spontaneity for some weeks. Since operation, he has returned to living with relations, cares for his own needs, and helps about the house.
Although he rare!y takes the prescribed anticonvulsant medication (none being detectable when blood tests have been done), his seizures are much improved since operation. He has l or 2 generalized convulsions per year and reported no visual aura for over four years.
The examination of 8 May 1973
<< The patient states that he is using his left side fairly well now, and attributes this to "Iam doing what you said and I concentrate on using it-the feeling seems to be coming back-for example, when I want to pick something up I always use my left hand to pick it up".
He walks and taiks in a normai fashion. He does finger-to-nose testing excellently with his feet together and his eyes closed. There is a good grip with negative Wartenberg sign bilaterally.
It takes special attention to elicit any dyspraxia in the left hand as the patient readily extends his thumb and keeps it extended when squeezing: but it is possible to elicit dyspraxia as in the following:
" Using your left hand, make a fist. "- Done correctly.
"Stick out your thumb. "- Done correctly.
"Make a fist. "- Done correctly.
"Stick out your little finger. "- Done correctly after an abortive false start with the thumb.
Make a fist. "- Done correctly.
"Stick out your index finger."- The little finger was extended, but hesitantly.
"Make a fist. "- Done correctly.
"Put your thumb and little finger together to make a circle"- the patient tried a variety of different maneuvers of his left hand but was unable to achieve the effect desired.
" Imitate what I am doing."- (The patient was shown a circlet with the thumb and little finger, and he did this quite readily with his left hand, but only after the examiner's hand was placed with the palm toward the patient. The examiner's hand was originally placed with the palm up, and the patient tried to attain a similar posture by putting his thumb against the index finger with the other fingers extended, but he assumed the correct posture as soon as the hand was presented with the palm toward the'patient.)
There is no facial dyspraxia.
No deficit could be elicited using a pinwheel. The patient has the usual inability to give the name of an object in the left hand when the vision is occluded. Excellent vibratory sense is present at both ankles. Finger identification is immediate and correct ipsilaterally, but impossible from one hand to the other.
Deep tendon reflexes at biceps and triceps are slightly hypoactive whereas at the knees they are normally active. No ankle jerk could be elicited with the patient kneeling on a chair. The superficial abdominal reflexes seem slightly decreased on the right and normal on the left: the cremasteric reflexes are normal bilaterally. Plantar reflexes are distinctly flexor bilaterally.
Visual acuity is at least 10/13 with each eye uncorrected. His stereoscopy is fair (100" = Shepard 50 % ). The pupils are normal in size and reactivity. Extra ocular movements are full. The optic discs are sharp. The tongue, jaw and uvula move normally. Auditory acuity is adequate bilaterally (using finger rub).
Visual field testing gives a result similar to what is usually expected; that is, the patient was told to point with his right hand and when a slight movement occurred in the right visual field, he immediately pointed to it. He was then told to fixate on the examiner's nose, and a slight movement was made in the left visual field, and although he had been instructed to use his right hand, his left hand immediately rose and pointed toward the moving fingers at the same time that the head and eyes were turned toward the left. This procedure was repeated several times. The first time that bilateral simuitaneous stimuiation was given, the patient failed to exhibit any awareness of the stimulus in the left visual half-field but pointed only to the right half-field and then pointed with his right hand to the moving fingers as they came across the midline from the left. On the second and third time that simultaneous stimuiation was tried (being separated by some other tests) he pointed to both visual fields using the right hand to point to the right field and the left hand to point to the left.
The examination of 3 November 1973:
<< He does poorly on all the faces tests but made only one error on the token test. He had the overlapping figures test and does excellently on the verbal or nameable sections but has remarkable trouble in selecting out the other items. This is consistent with his good performance on Similarities as contrasted with the Street where he does very poorly. This contrast between verbal ability and visuospatial disability is reflected in various ways. The patient is quite affable and quite alert in conversation and catches on to a great many things very quickly: but he still has some spatial difficulties, not only in formal testing, but also in that he becomes easily lost in strange surroundings, as finding his way from the laboratory back to the elevator this morning. >>
The Patient N. W.
N. W. (born 9 May 1930) was her mother's third child, was born at home and apparently had a normal delivery. There was no significant illness of infancy.
The patient's first seizure was at the age of 3. She continued to have seizures at intervals thereafter, commonly with tongue biting and incontinence. No localizing pattern was evident; on some occasions there was a warning consisting of a headache for 20 or 30 seconds before the onset of loss of consciousness. On most occasions there was no warning; for example, in 1960 she had a convulsion at home without any warning and suffered a broken arm.
The patient was repeatedly admitted to the Los Angeles County Hospital. In October, 1962 she had bilateral tubal ligation on the basis that "the patient is an epileptic, worsening during pregnancy and resulting in four stillborn infants ". Four subsequent admissions were for severe convulsions; on several occasions after a bout of status epilepticus she was noted to have left hemihypalgesia.
The patient was admitted to the White Memorial Hospital on 6 April 1966. There was no neurologic finding on admission but she had a mild left hypesthesia after she had a seizure in the hospital which resuited in a fall from bed. '
The patient was seen repeatedly throughout the summer and fall of 1966 for recurrent seizures making impossible her previous activity as a political organizer. She was readmitted to the White Memorial Hospital on 18 October 1966 after she had three generalized convulsions in a single day during which she struck her head on the concrete. Repeated EEGs had shown only diffuse disorganization without localization. Bilateral carotid angiography and air study were unremarkable. She had a complete cerebral commissurotomy on 12 December 1966. A postoperative meningitis responded readily to treatment; but subsequently she had evidence of ventricuiar dilatation relieved by a rightsided ventriculojugular shunt which required revision on several occasions.
The patient's seizures have continued, although less frequently. She leads an intermittently active social existence but remains unemployed.
The examination of 8 October 1973: The patient is alert and quite talkative and has a lively sense of humor. She finds a great deal to talk about including the fact that her 22year old boy was recently promoted to be a "foreman of special projects ". The patient's station and gait are not abnormal (although her gait is slightly waddling because of her excessive obesity). When asked to do the finger-to-nose test she was unable to stand unaided with her feet together, swaying and requiring some assistance to remain erect. When her eyes were closed there was no increase in the difficulty. When she was permitted to spread her feet slightly further apart to obtain a wider base, she was able to stand without assistance and closing the eyes in this condition did not produce any imbalance, so again the Romberg is negative.
Finger-to-nose is performed excellently with the right hand. In contrast, the patient performes very poorly with the left hand, putting her finger on the middle of her forehead on one try and on the second try iaying the palm of her left hand up against the side of her face. The interpretation of this deficit is not easy since it probably does involve some apraxia with the left arm but is complicated by the fact that she has a generally flamboyant approach to any performance with the left iimbs.
She foliows verbal commands readily with the right hand and has a good grip with negative Wartenberg sign. With the left hand there is considerable difficulty and, unlike the other patients who do have some dyspraxia, this manifests itself in a flamboyant disability. For example, when she is asked to make a fist with the left hand she opens it wider. It is possible, however, to obtain a fist in the left hand by waiting for her to slowly follow the instruction which she does with an air of great effort, gritting her teeth and staring at the hand and going through a variety of other histrionics until finally the hand is closed at which time there is a strong grip. Similar tedious waiting and persuasion and retrial is required in order to obtain extension of the left thumb, which
is done quite well when it finally occurs, and by continual effort it is possible to demonstrate that there is probably a negative Wartenberg sign on the left.
Testing for dyspraxia in the left hand is difficult; but there probably is some dyspraxia as manifested not only by extension of the incorrect finger when given verbal commands but also by the inactivity or lack or response on other occasions and by inappropriate responses of a
variety of different kinds. In any event, there is no incoordination or lack of strength in the left arm and hand. One of the striking differences between this patient and all of the others is that when it is difficult for her to follow a verbal command with the left hand, doing it with both hands at the same time does not provide any significant assistance.
There is no deficit on sensory testing with respect to pain or temperature. In contrast, the patient manifests the usual anomia in the left hand differing from the other patients only in that she talks a great deal about why it is that she cannot name objects in the left hand. There is also, as there is in no other patient, a distinct lack of report to vibratory stimulus. Vibratory sense at the right ankle is excellent as it is on the right wrist but she rarely if ever reports correctly the onset or offset of vibration at the left ankle or left wrist. Moreover, although she is quite accurate with vibbratory onandoff on the nght side of the mandible, she does not give correct responses on the left side of the mandible except on one occassion when she said "on" after sometime, and then stated, "it took me a while to feel that".
Deep tendon reflexes at biceps,triceps, knee and ankle are all slightly reduced but within normal range. The ankle jer ks return somewhat more siowly than normai. (Although the patient is obese there is no particular dryness of the skin or hair and certainly no psychomotor retardation).
No superficial abdominal reflexes were obtained (probably because of the obesity). Piantar reflexes are distinctly flexor bilaterally.
Visual acuity is at least 10/15 with each eye corrected. The extraocular movements are full. The pupiis are normal in size and reactivity. The tongue, jaw and uvula move in the midline. Using finger rub, auditory acuity appears to be minimally diminished on the right and mildly diminished on the left.
In testing for visual fields, the patient appears to have a complete and dense left hemianopia. On a few occasions with stimulus in the left field alone she seemed to recognize the presence of a stimulus just before it reached the midline, but this is possibly attributable to some
shift in fixation although the patient was cooperating quite well with her gaze fixed on the examiner's nose most of the time. She regularly recognizes a stimulus in the right half-field in any position and this is true whether the stimulus occurs in the right field alone or together with a simultaneous stimulus in the other field. It is of interest that she does
Not use crosscueing to point to stimuli in the left field, nor does the left hand point to stimuli in the left field as is the case with almost all of the other patients. The interpretation of this is not ciear but it is probably
related to the hysteria which confounds the examination of vibratory sense and dyspraxia. That there is no blindness on the left side is easily seen because the patient not only blinks but, in fact, flinches from threatening motions from that direction.
The usual facial apraxia routine was carried out and the only deficit was an inability to wink with the right eye and the patient says ,"I never could do that at anytime ". Finger localization is excellent in the right hand but cannot be adequately tested in the other three parts of the procedure. In contrast to her disability in some other respects, she is good on the faces tests including Tzavaras, BentonVan Ailen and is also reasonably good on the Mooney. A variety of other tests including Similarities and Street were done at this time with fairly good results. On the Bobertag she gave excellent descriptions to both pictures. Overlapping figures is poor whereas she made only one error on the token test, and that one probably because she was so busy talking and I would not repeat the instructions.
The Patient M. K.
M. K. (born 7 April 1938) had a normal delivery and infancy. About the age of three she had "rheumatic fever ". She recalls that she would frequently trip as a child because her left foot dragged. She attributed her seizure disorder to being hit by a playmate with a rock (but was not
unconscious at the time). Her spells first appeared about the age of nine, often beginning with jerking in the left toes, foot, leg and then becoming generalized. By age thirteen she was having spells almost daily,
She has had numerous injuries from her spells including cicatrizing burns on the left leg and both arms, and multiple head lacerations. When her head was shaved for the craniotomy there were 15 distinct scars, one of which was received in an automobile accident at the age of twentyseven but the remainder from falls during her spells.
Later in her teens the spells generalized very rapidly so that in adulthood she rarely had Jacksonian episodes. The
generaiized convulsions had been occurring at least twice a week for the entire six years before operation; they were commonly accompanied by urinary incontinence. There were also occasional minor spells consisting of a sudden cessation of conversation for a few seconds
The patient was studied at the WMMC where bilateral angiography and brain scan were normal. Serial EEG's were dominated by intermittent slow and some sharp wave activity, often paroxysmal, more evident bitemporally and more on the right side. The air study showed an increase of subarachnoid air and a moderately dilated ventricle on the right. A complete cerebral commissurotomy was done on 18 October 1967 following which the patient had a relatively smooth recovery, possibly because the single bone plate was quite large and had been left out. The bone plate was replaced on 29 November 1967.
There have been no generalized convulsions since operation. Almost every month, about two days before the beginning of menses, she has a spell or two, consisting of a brief episode of unresponsiveness. When these have occurred while she was sitting or standing there has been no loss of body posture.
The patient helps her nusband about the house but has a striking lack of initiative unless repeatedly coaxed;this must be done carefully so as not to elicit the temper tantrums which occasionally erupt trom time to time. Her social activity most recently has consisted of following her husband around the golf course. [{The patient s husband reported when she seen two months later that she was much more active. mainly in independent cooking and shopping}]
~ _
The Examination of 6 August 1973:
The patient stands and talks in a normal fashion. When she walks there is a pronounced left foot drop with minimal but detectable circumduction of the left leg, and there is a slight diminution in the swing of the left arm. Her temper was evident when she was asked to enter the examination room. She acIually screamed at loud volume that she would not cooperate and "no by God! I sure as hell will not ! ". She.attributed her temper to the mess which the EEG technician had made of her hair. The patient was gradually calmed and from then on she cooperated beautifully, although she fatigued somewhat after an hour of testing.
Finger-to-nose testing with the eyes closed and the feet together is done well except for a minimal dysmetria with the left hand.
Deep Iendon reflexes at biceps, triceps, knee and ankie are all within normal limits, but the reflexes on the left are all slightly more brisk than those on the right.
There is a definitely positive toe sign on the left with plantar stimulation if Szapiro reinforcement is used; otherwise, the toe sign on the left is equivocal. The Oppenheim maneuver produces no significant result on either side.
The patient cannot name any object paced in the left hand.
Vibratory sense seems normal at both wrists and at the iliac crests but cannot be determined with confidence at either the knee or the ankle.
The patient has a definite apraxia of the left hand to verbal command for individual finger motions. She makes a fist whenever asked, but she commonly confuses individual fingers. There is no question of understanding since she always holds up the correct finger on the right hand. Sometimes when asked to do a certain maneuver with the left hand (such as, " put your thumb up against your ring finger ") the left hand makes peculiar posturing motions; these are of various types, most commonly there is a sudden pronation, flexion at the wrist and extension of the thumb between the second and third finger.
When the patient is asked to do the same motion with both hands at the same time she almost always succeeds. In this way it is possibte to test the grip (which is good) bilaterally. The extension of the thumb on the left was maintained on several occasions but imperfectly, leaving the impression that there is a mild Wartenberg thumb sign on the left.
Visual acuity is 10/15 with each eye uncorrected. The pupils are normai in size and reactivity. The extraocular movements are full. The optic discs are sharp. The tongue, jaw and uvula move in the midline. Air conduction is good bilaterally (as tested by finger rub).
Visual field testing elicits no deficit of any kind in the right half-field. In the left half-field, correct responses occur only when preceded by a darting movement of the eyes to the left. This occurs on most of the occasions when the left field is tested alone and also on approximately half of the occasions when there is simultaneous stimulation. When there is simultaneous stimulation in both fields, the patient says, "there" pointing with the right hand to the right field stimulus, and at the same time there is often a movement of the eyes toward the left and the patient immediately says, "over there " pointing toward the left half-field. There is never any response (as is almost invariable in a normal person) such as, " both" or, "on both sides".
Facial apraxia is tested with the usual routine; the only deficit is that the patient is unable to cough voluntarily either on command or when a cough is demonstrated (neither can she click her teeth but this is because she left at home her lower denture).
Finger localization follows the usual pattern (good monomanually for each hand but no transfer.
Results on the Faces tests are surprising in that she does rather poorly on Tzavaras and also on the Benton - at these times she uses individual clues such as "fat face" or " big ears ", "full lips " etc. But she does fairly well on the Mooney faces, pointing out the various facial features on most of the items.
On the Street test she names only four of the items but when allowed to make a multiple choice from the Peabody booklet she gives an additional five correct answers to obtain a total of nine. She made no errors on the token test. The overlapping figures test is quite difficult for her,
especially the abstract designs.
The Patient J. M.
J.M. (born 2 Sept 1931) was thought to have a normal delivery, infancy and childhood. At age 15 she was rendered briefly unconscious in an auto accident. One year later she began having seizures which eventually were of several types: generalized convulsions. Automatisms with amnesia, and brief episodes of unresponsiveness lasting a few seconds. The generalized
convulsions were only partially controled by a variety of medications; the other spells continued in profusion.
She was seen at WMMC by Dr. C.B. Courville in 1954. EEG showed right temporal spikng and air study was normai. On 21 February 1955, the patient had an operation (by Prof. P.J. Vogel) which began with an electrocorticogram: the most prominent abnormal activity was found in the right superior temporal gyrus near the temporal pole. The right temporal pole was removed to a distance from the tip of 4.5 cm along the superior gyrus and further posteriorly on the lower gyri. The right temporal horn was entered and left widely open. It was thought that the amygdala was removed at least in part. Pathologicai examination by Dr. Courville disclosed a cortical scar 0.8 by 0.6 cm extending the full depth of the cortex. it was considered by Dr. Courvilie to be "obviously an old contusion ".
The patient's postoperative course was uneventful although she had two psychomotor spells in the hospital following operation. After discharge she continued to have spells with major seizures occurring approximately two per month, in spite of medication to toxic levels.
In 1956 the patient was hospitalized at a large State Hospital for the mentally ill, for three months, because of a "nervous breakdown ".
In 1966 the patient became worse in association with an in;ury to her back A lumbar laminectomy in April, 1967 gave her partial relief of her back difficuity but had no effect on the seizures. During 1967 while on Dilantin 600 mg /day, Mysoline 1000 mg /day, and Mesantoin
300 mg /day she had approximately fifteen major seizures and continued to have her small spells at the rate of approximat Iy three every day.
On readmission to WMMC in January 1968, no neurological abnormality was found ;in particular, there was no deficit in crosslocaiization or in the identification of small objects with
the left hand, or transfer of hand postures: and there was no dyspraxia.
EEGs including sphenoidal electrodes showed bitemporal sharp wave activity, somewhat more prominent on the
right, as well as evidence for a right frontal focus. Bilateral carotid angiogram was unremarkable. An air study was considered normal (in spite of the previous operation).
The patient had a complete cerebral commissurotomy on 2 February 1968. Her acute postoperative course has been described in detail elsewhere (5).
Toward the end of the first postoperative year the patient visited her mother who discontinued the medication after which she had two generalized convulsions. The same thing occurred in the third postoperative year with three generalized convulsions; none have occurred
since. Automatisms with screaming and seemingly purposeful activity have recurred about three times per year.
The Examination of 22 January 1973:
The patient walks, talks and stands in a normal fashion. She does finger-to-nose testing well with her feet together and her eyes closed and with both arms. There is a good grip with both hands and a definitely negative Wartenberg on the right. It is difficult to get her left thumb
extended not only because of the cast but also because she has a very marked apraxia to verbal command with the left hand: she was unable to extend any of a variety of fingers on verbal command, whereas she readily did this with her right hand.
The deep tendon reflexes at biceps, triceps, knee and ankle are normally active throughout. Plantar reflexes are not reliably testable since the patient was extraordinarily ticklish. There is excellent vibratory sense at both ankles.
The patient was given the facial apraxia routine, and performed on all items excellently.
She is quite unable to identify any of various objects which were placed in her left hand.
Visual acuity (corrected) is at least 10/15 with each eye. In the semidarkness, the right pupil may be minimally larger than the left and there is a bare trace of ptosis. Both pupils react
normally. The extraocular movements are full. The optic discs are sharp. There appears to be a dense homonymous hemianopia on the left, but after the patient was coaxed on a number of occasions to point with her left hand instead of her right, there were several occasions when she
pointed to the wiggling fingers in the left field with the left hand. Auditory acuity is adequate bilaterally. The tongue, jaw and uvula move in the midline.
She describes well the Bobertag pictures and does perfectly on the token test. On the Street test she named oniy two items (compared with four when tested a year and half ago), and when given the Peabody bookiet for help she obtained only a total score of three. On the faces tests,
both pointing and naming the numbers, she makes a great many errors, twentythree by pointing and twenty by naming the numbers. (Some crosscueing seems to be present during the verbal
numbering version, since she points with her finger after first fixating her gaze. When she was retested but not aliowed to point, she made additional errors.
111 Discussion of the Neurologic Findings
We consider first the station of the patient; this is without notable peculiarity, and such minimal changes as may be present are well within the range of normal variation.
With respect to gait, there is nothing notable except that in two patients
there is a left foot drop of the cerebral type, associated with other evidence ofuppermotorneuron damage includin mild hyperreflexia
in the leg. In one of these patients (M K.), the deficit was present before operation and is attributabie to a febrile illness in childhood producing mild cerebral hemiatrophy.
In the other case (A.A.) the footdrop was a surgical complication. In order to section the corpus callosum, it is necessary to retract one hemisphere away from the midline. This sometimes requires the deliberate sacrifice of bridging veins. In this case there was, temporarily at least, insufficient drainage with consequent swelling in the right parietal region. We suppose the volume of the associated infarction to be rather small, since the patient's left hand has completely recovered. In his leg there remains a foot drop and positive Babinski sign. He had for some years a fairly prominent, unilateral steppage gait. Since a tendon transplant about two years ago, his gait no longer presents any gross deficit.
After considering station and gait, we next consider speech.
With respect to speech, there is in most of these patients little to note with respect to execution, except shortly after operation. There was in
almost every case a postoperative period of mutism, of varying duration,
during which speech was absent or extremely sparse, although comprehen
sion and writing were retained How this might be interpreted I have discussed in greater detaii elsewhere (5). For the present, we note that as the mutism subsided, there was a stage of partial recovery which usually incuded hoarseness or whispering, but without paraphasia, anomia or novel semantic or syntactic errors. Subsidence of the mutism was complete within a few months in all cases but one. This patient (A.M.) continues, even years later, to speak very little and when he does speak, it is in a hoarse, poorly phonated manner. He had severe brain damage before operation; and we believe that he was, to an unusual degree, dependent upon his corpus callosum for articulatory control.
It is appropriate here to comment briefly on the indications for operation. With respect to the nature and severity of epilepsy, our current criteria for commissurotomy are much the same as they were at the outset. But we have acquired some additional criteria. I should like to mention, in particular, seven of these criteria. The first three concern seizure status; the remaining four are more general.
1. The patient should be having at least one daytime generalized convulsion per month in spite of intensive medical treatment. What is meant by "intensive " may depend to some extent upon what is available and what is tolerated by the patient. In the past few years we have added Tegretol (carbamazepine) to the list of medications which should be tried before considering surgery. The criterion of at least one daytime convulsion per month might be relaxed under certain circumstances. But more often, in our hands, it has been intensified; in most of our cases there has been recurrent status epilepticus
2. There should be either clinical, electrical or radiographic evidence for at least one nonablatable, cortical focus. Here again, some flexibility may occasionally be appropriate; but the rule is a good one, and it is well supported both by our therapeutic expenence and by our understanding of the extensive experimental literature.
3. Focailty attributable to progressive disease including expanding lesions is, in general, an absolute contrindication.
4. There must be confidence that the patient's intellectual status is more than barely marginal, so that loss of the corpus callosum, with its probable compensatory potential, will not be grossly disabling. There may still be a consideration for easing the difficulties of seizure management in certain mentally retarded individuals, but our own experience in this regard has been dissuasive.
5. In addition to overall intelligence, there must be assurance that the
commissural connections are not essential to certain specific functions, notably speech and short term memory. In this regard, we have adopted
carotid amytal testing (by percutaneous femoral technique) as a preoperative routine. I will return to this in a moment.
6. With respect to intellectual attainment, there may well be individuals who are dependent upon the commissures for activity such as poetry or
musical creativity which may be so dear to the patient that impairment of
such abilities would not be outweighed by control of the seizures.
7. If all of the above criteria are satisfied, there is an additional social one. Since recovery from cerebral commissurotomy often requires the better part of a year, adequate family support is essential. Without it, the potential of the operation will not be realized, and to operate under these circumstances would be a mistake.
Let me return now to the question of a possible role of the corpus callosum in speech production. The role of the cerebral commissures in speech is not yet well understood. However, we can be reassured by a favorable response to carotid amytal testing which, as I mentioned above, is now routinely done before a decision is made to recommend commissurotomy. This test can give ambiguous results, but it can, we believe, be used to exclude a critical dependence upon the commissures. That is, if the patient continues to speak intelligibly at the time when the hemisphere minor for speech is narcotized. then disconnection of this minor hemisphere will probably not deprive the patient of an essential resource with respect to speech production.
To return now to the postoperative neurologic status: a further comment should be made with respect to vocalization.
In general, these patients present a normal appearance in ordinary social
situations, including their conversation, and their demeanor during the taking of history and physical examination. The persistent mutism in a single case, I have already mentioned. The speech of all the other patients is fluent and confident However several patients have expressed some disappointment in their singing ability which, although it is not obviously deficient, is not In their opinion as good as it was befor surgery.
It is to be noted that the low average intelligence of most of these patients occasionally gives the impression of mental slowness in following complicated instructions. On an abbreviated version of the DeRenzi-Vignolo TokenTest, performance is within the normal range showing none of those deficits commonly seen in aphasic patients.
One further aspect of language deserves at least brief mention. Although the speech content seems in social situations to be as normal as the
execution, a more subtle consideration may obtain. To this end, the eminent psychiatrist Dr. Klaus HOPPE has examined these patients at length in a systematic fashion. And he has some tentative impressions, as to the poverty of fantasy or of dream recall, which he is preparing for presentation elsewhere.
We pass on now to the motor examination. With respect to individual
limbs one finds no uniformly appearing abnormality. For example, if the
patient is asked to stand erect with his feet together and his eyes closed, and with his arms extended before him with the palms upward, and is then asked to touch, with each index finger in turn, the tip of his nose, there is no deficit.
With rapid supination -pronation there is likewise no abnormality in most of the patients. Although a search has been made for certain kinds of
pathological associated movements, none have been consistently identified. [{ Dr. M. Bolez subsequently demonstrated in several of these patients an asymmetry of tonic grasping of the foot : these results were published separately at a later date: see Botez,M.I. And Bogen, J.E. In Acta Neurologica Scandinavica 54:453-463,1976}]
In particular it should be noted that the thumb of each hand can be maintained in an extended position while the patient forcibly closes the rest of his hand into a fist (in other words, the patient has a normal response to the Wartenberg maneuver).
Each limb presents, therefore, no consistent peculiarities with respect to either strength or coordination as ordinarily tested. But there is a distinct disability in coordinating the two hands without the aid of vision. Since none of these patients is required to work in the dark or with both hands out of sight, this disability has presented no problem for most of them. One notable exception occurred in the case of the patient R.Y. This disability was responsible for his loss of employment.
Although R.Y. never had regular employment before his operation (ostensibly because of his seizure disorder), it was our hope that with his seizure status improved, he might be able to hold a job. A position was therefore arranged for him in the kitchen of a convalescent hospital. (I may note parenthetically that the Workmen s Compensation Laws in the state of California make it quite difficult for even an interested employer to hire anyone who has a known disability. It seems to us that the obvious solution for such a problem would be for the law to include a waver on the known disability-but this solution has not yet percolated through the bureaucratic minds insurance administrators. In any event a hospital administrator was found who was willing to take a chance.) And so R.Y., who was very happy at the opportunity, took a job loading dirty dishes into a large automatic dishwasher.
For the first few days he did excellently. But the next week there occurred a catastrophe from which he fled without warning and without explanation, thus alienating permanently his erstwhile employer. What happened, as he explained it later, was that so long as he kept his attention on what he was doing, he did very well. But then, he said, "I got bored because it was so easy and started looking around at all those girls". His hands, no longer under visual control, dropped several dishes which broke noisily, and so he panicked and fled, never to return.
In retrospect the problem appears to have been unnecessarily and tragically compounded by the blow to his selfesteem. Ever since, he has refused any job offer, although he is reasonably helpful about the house were he lives with his sister and brotherinlaw.
Some difficulty in coordinating the two hands, when vision is excluded, is a persistent phenomenon in all of these patients with complete cerebral
commmissurotomy . This is in contrast to another motor problem which
uniformly appears, but in most patients soon subsides.
Shortly after operation it is routine for these patients to have an apraxia of the left hand for verbal command. By the time of the long term examinations reported here, this dyspraxia had largely subsided except in four patients. These include the patient (M.K.) with a mild right hemicerebral atrophy,thepatient (J.M.) who had previously had a right temporal lobectomy, the patient (A.M.) with marked, diffuse cerebral atrophy and the patient (W.J.) with marked right frontal lobe atrophy observed at operation.
We consider now the reflexes.
No deep tendon reflex abnormality was found, including the patients with residual dyspraxia, except in the two patients previously mentioned as having a cortical foot drop in those two cases there was hyperactivity of the Achilles reflex. In one of these patients (A.A.) the hyperreflexia at the ankle was accompanied by a distinctly positive Babinski sign. Abnormal plantar reflexes were absent from all the other patients, although all of these patients had abnormal plantar reflexes, often bilateraliy, in the first few weeks after surgery. Superficial abdominal and cremasteric reflexes are typically absent after the operation, but eventually return.
We consider next the salient sensory findings.
Sensory abnormalities were confined to the left side of the body with rare exception. The most notable finding is an anomia, or inability to name objects placed in the left hand, although the test objects are manipulated correctly, and can be reliably retrieved by the left hand from a large collection of other objects. Over sixty papers have been published on these patients, reporting a wide variety of behaviors in various testing situations. This variety results not only from the considerable individual differences among these patients, but it is also a result of the special interests of the many investigators who have been involved. It is therefore worth emphasizing that there are certain features which are common to every patient and which persist indefinitely. Of these ubiquitous and persistent deficits, the anomia of the left hand is particularly notable. It is notable not only for its importance in
demonstrating a capacity for discrimination, deliberation and correct decision making in the absence of any speech, but it is also of interest because it can be reliably demonstrated in the clinic without special equipment.
With respect to simple sensation, there is no significant change in threshold for pain or temperature.Vibratory sensation is tested in the
following manner: the ball of the thumb is pressed against a bony prominence and the patient is instructed to say "on " or "off " when the base of the vibrating fork is applied and then subsequently removed from the examiner's thumb nail. In several older patients there has been some
diminution at both ankles for vibratory sense; but this was probably related to mild peripheral neuropathy and not significantly related to the operation. In contrast, when 2 point discrimination and baresthesia are investigated in a more detaiied fashion, there are occasionally some deficits. But again these are not sufficiently uniform to be considered a necessary consequence of the operation Some preliminary report on this has already been made by Prof. Milner who will publish a more detailed report in the future.
It is worth repeating that the most striking deficit, in the course of a routine sensory examination, is the inability of the patient to name objects which are placed in the left hand. In the course of routine neurological examination we always include this step. That is, we place a safety pin, a paper clip, a small coin, and similar objects in the left hand. The intact patient rarely has any difficulty naming any of these objects. The patient who has had a complete cerebral commissurotomy invariably fails to name the objects although he quickly names them in normal fashion when the object is transferred to his right hand.
The patient who has been frequently tested for anomia can often confuse an inexperienced examiner by the use of clever crosscueing strategies. Some examples were given in the foregoing accounts of individual examinations. What I wish to emphasize, however, is that the experienced examiner can circumvent these strategies if he is aware of them.
We pass on now to the examination of the cranial nerves.
There is no significant alteration of olfaction, unless one tests each nostril separately. In that case, odors presented to the right nostril (of the righthanded patient) cannot be named. [{As shown by H.Gordon}].
There is no consistent finding with respect to visual or auditory acuity. Extraocular movements are full and there is no abnormality on fundoscopy. Movements of the face, tongue, jaw, and palate present, with the exception of certain speciai cases, no peculiarity.
The confrontation testing of visual fields gives particularly interesting
results. All of the patients have an apparent left homonymous hemianopia for some weeks after surgery. As the right hemisphere becomes more active and cooperates in the testing, a left hemianopia is apparent only when the patient replies verbally, or by pointing with the right hand. Conversely, when the left hand is used for pointing to the test stimulus, there appears an homonymous hemianopia to the right. In a few patients, there is stabilization at this level of performance, so that it continues to be possible to demonstrate the right hemianopia of the right hemisphere (that is, the left hand) and the left
hemianopia of the left hemisphere (the right hand).
In most patients, however, there eventually appears a condition in which no field defect can be demonstrated by the usual confrontation technique.
This apparently depends mainiy upon the ability of each hemisphere to direct the head and eyes. For example, if the patient is instructed to point with the right hand to those fingers of the examiner which move, and if the examiner then wiggles the fingers which are in the patient's left visual half-field, the patient's head and eyes quickly turn to the ieft and then the right hand points to the correct target.
If turning of the head is prevented, a leftward glance will suffice the
patient's need for a cue. Also, the right hand may point to the left visual halffield as soon as it is apparent that there is no suitable stimulus in the right visual half-field. This"cheating " by the left hemisphere can often be exposed by having no stimulus at all. Furthermore, if stimuli are presented simultaneously in both visual halffields, only the stimulus in the right halffield is described by the patient, that is, by the left hemisphere. There is usually no verbal response
to the stimulus in the left visual half-field until the left hemisphere realizes that the left hand is also in action, pointing to the left halffield stimulus.
When a patient has been tested repeatedly, so that the occurrence of bilateral stimuli can be expected, both may be identified by a single hand. In this circumstance, the patient will point first to the right and then to the left halffield.With those patients who have been frequently tested, the appearance of a stimulus in the left visual halffield is occasionally recognized in spite of our attempts to circumvent the crosscueing strategies. However, even in the patient who does the best (L.B.) performance on confrontation testing of visual fields is still distinguishable from normal.
IV Concluding Comment
sThe philosopher, L.J. Rather once said, with respect to the ontological argument between dualists and monists, "the problem is not likely to be resolved within the set of presuppositions that helped to generate it ".
The controversy between holists and topists in neurology is hardly as
ancient as that argument; but it is sufficiently longstanding that we might easily wonder if the nonresolution of which we are all aware is perhaps ascribable to some similar source [{I..e. Improper framing of the issue}].
Our feeling about holism and topism is that we are unlikely to
comprehend with either of these two ~ singlebrain ~> theories the organization of a system which is, in fact, double.
There has not yet been an intensive and systematic attempt to reconcile the available data in terms of a view which holds that each hemisphere can function holistically, while at the same time recognizing that each hemisphere functions differently. Essential in this approach is the assertion that the hemispheres are not material specific (for example, verbal versus spatial), but that they are process specific.
Of course, if each hemisphere has a dominance for a particular type of information processing, then we might expect that certain kinds of material will be more readily processed by one hemisphere than the other. And this can give the appearance of a material specificity. A lesion of either hemisphere will therefore result in some defect for almost any kind of material and the overall behavioral resuit will be an extremely complicated integration of two different contributions, one of which is partially defective.
I cannot offer now a comprehensive answer to this longstanding holism-topism issue, except to note that data from humans who have had
hemispherectomy or commissurotomy have already been helpful. And
we can reasonably expect more help from the application of lateralized and doublestimulation testing.
In conclusion, some recasting of our conceptions and terminology is indicated. In particular, we would urge the value of thinking, as often as possible, in terms of two brains rather than one.
APPENDIX I
In preliminary reviews of this.paper it became evident that descriptions of the neurologic examinations include some terms which may not be familiar to all readers. These include the following:
The plantar reflex was elicited in the manner and with the patient positioned as described by Wartenberg (6), both with and without Szapiro (7) reinforcement, A flexion of the big toe in a volar (plantar) direction is here described as " a flexor response ", or as a downgoing toe and is normal in the adult. The uppoing toe or "extensor response " or "positive Babinski " is abnormal. Other aspects (such as fanning, Puusepp, thigh muscle contraction) of this complicated lower extremity flexion reflex were not used in determining if the response was normal (negative) or abnormal in these examinations.
The "deep tendon reflexes " were elicited by a blow with a rubber reflex hammer to the relevant tendon. As Wartenberg (8) has insisted, these are in fact "deep muscle reflexes " or myotatic reflexes, but we have retained here the more common (Erb) terminology.
The "Wartenberg sign " was called by him (6) the test for "associated movement of the thumb ". A positive (abnormal) response means here the inability (in a patient over the age of about six years) to keep the thumb extended while forcibly squeezing the examiner's index and
middle fingers (introduced from the uinar side of the patient's hand).
The ability of the patient to << stereo >>, i.e. to interpret retinal disparity as a third dimension, was determined with the polaroid lenses and quantitative sections of the Fly Test published by
the Titmus Opticai Co. of Petersburg, Va., U.S.A. 23803.
The BinetBobertag pictures used here were those in the article by Kinsbourne and Warrington (9), The Street pictures were those we have used previously (10, 11). The overlapping figures test was kindly provided by Professor Di Renzi of Milan.
The faces tests included the one by Benton and Van Allen obtainable from the Neurosensory Center, Univ. of lowa Hospitals at lowa City, a series of some of the more difficult items from those furnished us by Dr. A. Tzavaras of Paris, and a selection of the Mooney items provided by Dr H. Lansdell of Bethesda. The short form ( 16 item) token test was obtained from Dr O. Spreen of Vancouv
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