DECEREBRATE RIGIDITY IN MAN
In September, 1923, the only instruction I received from Harvey Cushing when I arrived to work for a year in his clinic was to make myself "at home." I had watched one of his long operations which began at eleven o'clock in the morning and was completed at four thirty. There was an early breakfast and no lunch. As he sat in the surgeon's dressing room, still rubber-gloved, with his cigarette and a cup of tea, sketching the location of the tumor he had found and removed, I stood quietly, waiting for him to speak. Finally, he looked up silently. I reminded him that he had agreed to Allen Kanavel's request that I spend a year with him. He nodded in agreement, dismissed me with this short instruction, and returned to the task of completing the operative note.
My morning greetings were not answered; I was not recognized for two months during which I examined his patients, typed my own clinical notes, and sat in the uncomfortable narrow pipe seats of the movable audience stand. I carried a fresh package of Herbert Tareyton cigarettes, hoping that he might reach for one in his pocket and come up empty-handed, and that I might dramatically step into the breach and gain some approval. The opportunity came in November when I was told by the head nurse on Ward C that I had better answer my call ring. I had not known that I had been assigned a call ring. She advised me to hurry down to the Chief's office because he had been paging for me for about an hour.
Again, I stood in silence while Cushing turned the pages in one of the many books he had on his desk flanking a patient's chart. Finally, I was asked why I had examined one of his private patients on Ward A. As calmly as I could, I replied that I had been told to make myself at home, that I had been educated and trained in examining patients, and knew how to avoid giving my advice by saying that I would transmit their questions to their doctor, from whom they should get all their answers, and thus avoid confusion and misunderstanding. I said that I had had three years of training in surgery, that I had learned a great deal from my examinations and from observing him operate. In answer to his question, why hadn't I operated with him, I replied that I believed this directive should come from him. There was silence as he searched his pockets and the drawers of his desk for a cigarette. I offered him the fresh package of Herbert Tareytons; he opened it, took out a cigarette, lighted it, and put the package into his pocket. There followed an hour's lecture on the differential diagnosis of multiple sclerosis and a cervical spinal cord tumor. It was a magnificent discourse but when he asked if I now realized I had made the incorrect diagnosis of multiple sclerosis, I could only reply that when he removed the tumor I would know that I was wrong. His angry dismissal was a command to operate with him on all patients on Ward C in the future.
Before I went to the Brigham, Lewis Pollock and I had devised an experimental preparation for producing decerebrate rigidity by ligating the basilar artery and tying off the common carotid arteries. We had been able to demonstrate the presence of reflexes that had never been present in previous decerebrate preparations because of the extreme shock produced by the crude method of cutting through the cerebral and brainstem tissue. We studied Magnus-de Kleijn reflexes produced by passively changing the position of the head in relation to the body. By changing the level of the basilar tie, we could determine the level of other reflexes.
It was with great interest, and some trepidation, that I had discovered a young lad with a brainstem tumor who was decerebrate and upon whom I could demonstrate Magnus-de Kleijn reflexes. I tried to show the patient to Dr. Cushing on his rounds, but without success. Without his permission, therefore, I arranged with Alice Thing, his photographer and histological technician, to photograph the patient for me. With considerable misgiving she agreed, and I brought the patient to the clinical laboratory, placed him on a mattress on the floor, and was busily helping Miss Thing arrange her camera and lights when the Chief walked in. He gazed silently at the patient, and quietly asked me what we were doing. I explained and, without waiting for his reply, demonstrated the reflexes. I repeated the maneuvers over and over again at his request. When he became satisfied with the demonstration, he curtly ordered Miss Thing to proceed with the photography and to develop her films before we took the patient back to the ward to be sure she had good pictures. He then turned to me and said, "Be sure you obtain an autopsy on this child. If you need help, call me. You'll have to verify the level of decerebration grossly and microscopically."
So it was that this manuscript developed, but only after a session of brain cutting at which Dr. Cushing presided. He gave explicit instructions for the microscopic sections and staining technique. I wrote the manuscript several times; each time the copy was returned with his penciled comments in the margin, until I thought a final draft could never be obtained. I have never forgotten his efforts to help me develop a style of medical writing, and after I returned to Chicago to practice neurological surgery, my publications received such postcard recognitions as "More power to your elbow" and "Certainly, not your best effort," and they were signed "H.C."
LOYAL DAVIS
DECEREBRATE RIGITY IN MAN
Loyal E. Davis, M.D., PhD.
Archives of Neurology and Psychiatry, 13: 569-579, 1925.
Our knowledge of muscle tone has been enriched by the study of experimental decerebrate rigidity. Recently many clinical cases have been described in which the condition of the patient was said to have fulfilled completely or only in part the requisite characteristics of that condition.Archives of Neurology and Psychiatry, 13: 569-579, 1925.
The anatomic and physiologic considerations involved in decerebrate rigidity have been emphasized recently by Walshe and by Pollock and Davis. It is clear that if we are to apply the condition of decerebrate rigidity to clinical cases, certain anatomic and physiologic requirements must be met. Anatomically, of course, the complete experimental lesion will never be attained in the clinic. On the other hand, the essentials of that lesion may be obtained; namely, interference with voluntary motor power and abolition of postural reflexes in the midbrain. Physiologically, there should be a true hypertonus which selects the extensor muscles of the extremities, and this should be accompanied by reciprocal flaccidity of the flexors. This rigidity should have the qualities of experimental decerebrate rigidity; that is to say, the rigidity should be plastic. This characteristic may be brought out by the demonstration of sudden yielding of the rigidity on passive stretching of the muscle or by the prolonged tendon reflexes which may be elicited. Further, it should be possible to offset the rigidity by phasic spinal reflexes. The exhibition of the tonic reflexes described by Magnus and deKleijn are not necessary to the presence of decerebrate rigidity. Their presence in the absence of postural reflexes, however, adds strong evidence to the existence of such a condition.
Magnus and deKleijn have briefly reported several cases of clinical interest. They were, of course, concerned primarily with the presence of postural and tonic reflexes and not with decerebrate rigidity. The greater number of their cases are so incompletely reported that it is extremely doubtful that they can be considered as clinical examples of decerebrate rigidity. In 1920, Wilson reported a series of cases which he divided rather generally into those of decerebrate rigidity with and without tonic fits. He also expressed the opinion that many of the involuntary postures of a limb in various nervous diseases, as well as the transient positions assumed in chorea and athetosis, are parts of the decerebrate attitude. If one carefully considers each case reported by Wilson in the light of the experimental decerebrate animal, one must of necessity rule out many of his case reports as examples of true decerebrate rigidity. Tonic fits do not occur in the decerebrate animal except as a result of hemorrhage or undue irritative trauma to the medulla. The decerebrate animal has a gradual loss o thermic control without any irregularities of pulse or respiration. Further, the assumption of an attitude similar in some respects to the decerebrate rigidity of experimental animals is not alone sufficient to classify that case as an example of partial or complete decerebration. Strychnin poisoning and meningitis produce an attitude similar in appearance to that of the decerebrate animal, yet these conditions are not analogous. Wilson has thought of the red nucleus as the center which produces decerebrate rigidity. Bazett and Penfield and Pollock and Davis have produced decerebrate animals in which the red nucleus was destroyed or was completely isolated above the section.
Meyers has reported two cases in which the important lesion common to both was degeneration in the lenticular zone. His conception of decerebrate rigidity is different from that described by Sherrington, and consequently the clinical cases are difficult to analyze for evidence of symptoms of decerebrate rigidity. Simons has reported several clinical cases, among them many cases of hemiplegia, which showed tonic reflexes. He believes that patients with an extrapyramidal syndrome and with no evidence of pyramidal tract disease never show these reflexes. The cases reported were not critically studied from the standpoint of clinical decerebrate rigidity. Brouwer likewise studied the tonic reflexes, but made no analysis of the quality of the rigidity which was present.
The case of decerebrate rigidity in man reported by Walshe, to my mind meets the requirements of experimental decerebrate rigidity more closely than any of the cases so far considered in the literature. Walshe carefully studied a young woman, 23 years of age, who at necropsy was found to have a typical suprasellar cyst which contained calcareous deposits. One year previously, she complained of headaches, the onset of which were followed by sudden loss of vision. On examination, she presented marked bilateral papilledema with secondary atrophy. There were bilateral Babinski signs, with increased deep tendon reflexes and sluggish superficial abdominal reflexes. A right-sided subtemporal decompression was performed, and subsequently the patient developed a left hemiplegia. She later became semicomatose, and could neither answer nor carry out simple orders. After four weeks, she developed bilateral hemiplegia and was unable to swallow. The pulse and respirations were at all times regular and unembarrassed, while the temperature remained subnormal. She exhibited many interesting symptoms viewed from the standpoint of decerebration. The arms were held lying across the body semiflexed at the elbows, with the forearms slightly pronated and the wrists and fingers flexed. The legs were held extended and adducted, with the feet in plantar flexion. The rigidity was plastic and was typically "clasp knife" in character. The tendon reflexes were brisk and showed a tonic prolongation as well as muscle shortening after a series of rhythmically produced tendon reflexes. Plantar stimulation was followed by a flexion reflex. The tonic reflexes of Magnus and deKleijn were present, and in the article are illustrated on a model. More recently, Marinesco and Radovici have reported another case that may be classed as clinical decerebrate rigidity. Their patient developed a quadriplegia and exhibited Magnus and deKleijn reflexes in addition to an extensor rigidity which apparently was quite typical of that found in the decerebrate animal. The lesion in this case is predicated as a vascular hemorrhage or thrombosis in both internal capsules, although no necropsy report is available.
I wish to report a case similar to that described by Walshe. The condition in this case was diagnosed as suprasellar cyst and was shown by operation and necropsy to be so. While many suprasellar cysts and other lesions of the central nervous system have been observed in Dr. Cushing's clinic, this case was the only one which to my mind presented symptoms typically characteristic of decerebration. I do not believe the tonic spasms so frequently encountered in cerebellar tumors and accompanied by respiratory and circulatory irregularities are representative of decerebrate rigidity in man. It is necessary to establish the clinical picture of decerebrate rigidity conclusively and thoroughly before attempting to describe pictures of partial decerebration.
REPORT OF A CASE
Complete blindness, quadriplegia, headaches, semiconsciousness-preoperative diagnosis of suprasellar cyst. Partial evacuation of cyst; death. Necropsy verified a large suprasellar cyst with calcareous deposits.Clinical History.-T. H. C., a boy, aged 4 years, referred by Dr. L. C. Heidger, Bridgeport, Conn., was admitted to the hospital, Feb. 13, 1924, with the history that at the age of 18 months visual difficulties were first noted by the parents. It was then apparent that the child could see objects in the upper visual fields much better than he could in the lower or lateral fields. At that time, he developed an internal squint in the right eye. The patient's vision gradually grew worse, until he became completely blind at the age of 2½ years. The child, however, was active and played about his home until June 1923, when he began to complain of severe frontal headaches which recurred every second or third day and were accompanied by vomiting. He remained in that condition until December, 1923, when he became drowsy and stuporous, and his headaches became almost constant. He was placed in an asylum for the blind, where he was found and referred to Dr. Cushing's clinic. There was nothing of note in his past history except a trauma to the head in infancy, which was not looked on as particularly serious at the time. He had never had convulsions or any other evidences of an irritative lesion.
Neurologic Examination.-On entrance, the child was lying in bed in a semistuporous condition. He was wholly unable to perform any voluntary movements with his upper or lower extremities, all of which were definitely spastic. The legs were held in strong adduction and in full extension with the feet in plantar flexion. The arms were held in semiflexion at the elbows and were kept adducted to the body. The left arm was more spastic and was held more closely adducted than was the right. The extensor rigidity was also more pronounced in the left lower extremity. The deep tendon reflexes were much exaggerated, but equally so, while all of the superficial reflexes were absent, and there were bilateral Babinski phenomena.
The neck was held quite rigid and stiff, but there was no retraction (Fig. 1 A). There was an absence of papillary response to light. There was conjugate deviation of the eyeballs to the right. The optic disks were almost paper white, with sharply defined edges, while the retinal vessels were normal in size and distribution. Both optic cups were visible. The picture of the fundi was characteristic of a primary optic atrophy. Stimulation of the skin over the body evoked ample motor responses. At this time, the pulse averaged about 86; the respirations were 26, and the temperature per rectum was normal.
Roentgen-ray examination of the skull showed the typical picture of calcification in the region of the sella turcica. The roentgen-ray evidence of suprasellar cysts has been referred to recently by McKenzie and Sosman.
Operation.-On Feb. 19, 1924, Dr. Cushing made a burr hold through the skull at a point usually selected for the upper angle of a transfrontal osteo-plastic flap. A brain needle introduced toward the pituitary fossa first entered the lateral ventricle, and a large internal hydrocephalus was verified. The needle when introduced to a deeper level in the same direction struck a large cyst from which only about 15 c.c. of characteristic yellow fluid containing cholesterin crystals could be obtained.
Clinical Course.-The child was somewhat better for twenty-four hours, and then resumed his stuporous condition and gradually became unconscious. He was unable to swallow and had to be fed by an intranasal catheter.
The extensor rigidity became more pronounced in all the extremities. The rigidity is shown in Figure 3 A. After persistent efforts to overcome it, the rigidity would melt away in the "clasp knife" fashion. The upper extremities were held in semiflexion as described previously, but could be molded into various positions where they would remain for from five to ten minutes, after which they would gradually assume their previous posture (Fig. 3 B). The deep tendon reflexes were brisk and showed definite tonic prolongation with muscle shortening after a series of induced rhythmical reflexes. Plantar stimulation produced plantar flexion of the toes with a massive flexor withdrawal response at the knee and hip. This withdrawal reflex was often accompanied by movements of the upper extremities, as is shown in Figure 3 C. Sneezing and coughing reflexes were easily elicited. The jaws were held tightly closed and were opened with difficulty.
Magnus and deKleijn tonic neck reflexes were easily demonstrated, as is shown in Figure 1 B and C. Turning the chin toward the right shoulder produced increased extensor rigidity in the right leg with movement of the right upper extremity from the position shown in Figure 1 A to that of extension and pronation of the hand shown in Figure 1 B. At the same time, the left leg became less rigid and the left arm assumed a position of increased flexion and supination at the wrist. Similarly, rotation of the chin to the left shoulder produced increased extensor rigidity in the left leg with increase of plantar flexion; extension at the elbow and pronation at the wrist in the left upper extremity. At the same time, rigidity in the right lower extremity became less marked, and the right upper extremity became more flexed and the wrist supinated.
The muscle tonus was at all times more marked with the child lying on its back, and he kept this position without change unless turned by the nurses. He was picked up and supported beneath the axillae. In this position, the chin fell forward on the chest and the rigidity in the extremities became much less marked, although still present. The child had no power of assuming a normal posture voluntarily, but on the other hand maintained any posture imposed on him.
The temperature taken per rectum was at all times subnormal, while the pulse and respiratory rates were regular. There was at no time anything resembling tonic fits, which invariably are accompanied by respiratory and circulatory irregularities both clinically and experimentally.
Operation.-Dr. Cushing again punctured the cyst and secured 30 c.c. of the same type of cystic fluid, in the hope that the child might improve sufficiently to justify more extensive operative procedures. The patient succumbed three days later. Forty-eight hours before death, the tonic reflexes described were not demonstrable, and remained absent.
Fig. 4.-Saggital section of the brain. Size and relation to the midbrain and pons of the suprasellar cyst are shown.
Necropsy Finding.-The brain, hardened in situ, showed a large suprasellar cyst. Such cysts are congenital in origin and arise from the craniopharyngeal pouch. A saggital section (Fig. 4) showed the size and relations of the tumor. There were large masses of calcareous material within the cyst cavity, which produced the characteristic shadow on the roentgenogram. There can be no doubt that such a tumor was capable of producing definite and marked compression on the structures in the anterior part of the brain stem and could produce a physiologic decerebration quite analogous to that seen in an experimental lesion through this same region.
Pathology.-Sections of the cerebral cortex from the precentral gyrus showed definite retrograde degenerative changes in the giant pyramidal cells. The cells were shrunken and ill-shaped and the processes were swollen. The nuclei stained poorly and were placed excentrically. The Nissl granules were gathered in large clumps near the cell periphery and stained poorly. In most instances, they were represented by an indefinite mass which was difficult to distinguish from the cytoplasm. The most cells of the midbrain showed definite chromatolytic changes. The cells of the basal ganglia did not show any typical changes. The appearance of the motor cells in the cortex and midbrain contrasted strongly to that of the motor cells of the lower pons, medulla and spinal cord. In these areas, typical large Nissl bodies were seen.
Figure 8 shows the presence of fat droplets in the cytoplasm of the motor cells of the cerebral cortex and midbrain. No pathologic changes could be found in the neuroglia, nerve fibers or connective tissue by the Heidenhain, Walter, ethyl violet-orange G, Perdrau of Loyez methods.
SUMMARY
This case represents decerebrate rigidity in man. The suprasellar cyst produced sufficient compression upward on the brain stem to produce clinical symptoms exactly similar to the picture of experimental decerebrate rigidity. While the anatomic lesion of experimental decerebration cannot be encountered in the clinic, a physiologic lesion may occur which meets the basic requisites of such an experimentally produced decerebrate rigidity. In the nature of things, such cases are infrequently encountered.Decerebrate rigidity is an entity by the study of which our knowledge of muscle tonus has been enriched. The application of such a physiologic entity to clinical cases should be made only after all the principles underlying that condition can be satisfied. Attention again has been called to the symptoms presented by the decerebrate animal. Although the presence of certain of the Magnus-deKleijn phenomena is not necessary, their presence corroborates the diagnosis of clinical decerebrate rigidity. The pathologic finding in the case reported would tend to snow that such extensor rigidity is accompanied by definite changes in the pyramidal system cephalad to the site of the lesion. The rigidity in extension present in this case was of a character exactly analogous to that found in experimental decerebration. This rigidity was quite unlike that encountered in paralysis agitans or athetosis. Magnus-deKleijn tonic neck reflexes were also present. Irregularities of the pulse or respiration did not occur, so that there was no stimulation of medullary centers such as is frequently encountered in tonic spasms.