Tumor and Large Cyst of the Cerebellum, with Symptoms Extending over Several Years J. T. ESKRIDGE, M.D. DENVER, COL. PROFESSOR OF NERVOUS AND MENTAL DISEASES IN THE MEDICAL DEPARTMENT OF THE UNIVERSITY OF COLORADO; NEUROLOGIST TO THE ARAPAHOE COUNTY AND ST. LUKE'S HOSPITALS BY Reprinted from the Medical Record, August 17, 1895 NEW YORK TROW DIRECTORY, PRINTING AND BOOKBINDING CO. 201-213 East Twelfth Street 1895 Reprinted from the MEDICAL Record, August 17, 1895. TUMOR AND LARGE CYST OF THE CERE- BELLUM, WITH SYMPTOMS EXTENDING OVER SEVERAL YEARS. By J. T. ESKRIDGE, M.D., DENVER, COL. PROFESSOR OF NERVOUS AND MENTAL DISEASES IN THE MEDICAL DEPART- MENT OF THE UNIVERSITY OF COLORADO ; NEUROLOGIST TO THE ARAPAHOE COUNTY AND ST. LUKE'S HOSPITALS. Lucy E , eleven years of age, white, of German parentage, was referred to me by Dr. McNaught, Feb- ruary 19, 1895, on account of convulsive seizures ex- tending over a period of several years, attended by almost complete blindness. Family history was nega- tive. The child at birth was apparently healthy, but when fourteen months old, while teething, began roll- ing the head from side to side, and when taken in the mother's arms the head would be retracted and turned from side to side. The child would frequently cry as if in pain. Several days after these symptoms first manifested themselves, she was seized with a convul- sion which lasted about five minutes, during which there was cyanosis of the face, frothing from the mouth, the eyes were fixed and the pupils dilated. One leg was flexed at the knee and thigh, but which the mother does not now remember, both hands were clinched, and she thinks the arms were extended and rigid. About this time, or February, 1885, a thick, offensive discharge took place from both ears. The otorrhoea continued for two or three months, and dur- ing most of this time the child was restless and threw its head from side to side. From June, 1885, to Janu- ary, 1888, the child was not really ill, but it remained 2 nervous and fretful. At the latter date it had a slight attack of mumps and afterward remained in its usual condition until May, 1889, when it suffered from mea- sles, but recovery from this seemed to be complete. In April, 1891, it fell from a wagon, striking the fore- head, just above the right eye, against a horse's hoof. There was a contused wound over the eye which con- tinued to suppurate for about two weeks. At this time complaint was made of pain in the back of the head. After this it remained in apparently good health until June, 1892, when it became very nervous, and vomited repeatedly. The vomiting was always attended with pain in the occipital region. These symptoms continued for two weeks, when it had its second convulsion, last- ing about thirty minutes. This seizure began by the child crying loudly, " Mamma, mamma ! " the head was retracted, and rolled from side to side, the spine was arched backward, and the arms and legs were extended and rigid. The child remained perfectly conscious throughout the seizure, moaned and called its mother, and gave evidence of great suffering. Six hours later it had a second and similar attack. The convulsions occurred two or three times every day, and on one day there were fourteen, each seizure lasting from thirty minutes to several hours. Her condition seemed to be uninfluenced by treatment, and, in fact, the mother stated that the bromides seemed to increase the seizures. In December, 1892, an abscess formed in the right groin, which was opened a few days later, giving vent to a free discharge of pus. About this time the child became almost totally blind, remaining so for three months, the convulsions continuing in the meantime. Early in 1893 she partially regained her vision, but the convulsive seizures, which were nearly all of the same character, continued until August, 1893. From this date until December, 1894, she was free from convul- sions, but she remained nervous and vision was very poor. At the latter date the convulsions recurred 3 again and were attended with increased pain in the back of the head and frequent vomiting. She remained in this condition until I saw her, February 19, 1895, when I placed her in St. Luke's Hospital, under a trained nurse, to have the convulsive seizures watched. Examination February 22, 1895.-She walks fairly well backward or forward and with eyes open or closed. There is no evidence of distinct ataxia in the legs, arms, or trunk muscles. The knee-jerks are slight, and ankle-clonus is absent. Plantar reflexes and tendo- Achillis are present. Lower abdominal, scarcely per- ceptible ; epigastric, about normal. Deep reflexes of the arms are not increased. Dyn. R. 22 ; L. 20. While the child is not strong for its age, there is no distinct paresis or paralysis of any group of muscles. The tongue is protruded in the median line. All the general sensory phenomena and the special senses, smell and taste, are normal. Hearing watch, R. % ; L. The tuning-fork is heard better in the left ear. There is no weakness of the external ocular muscles. She is able to perceive objects with the right eye, but vision is not sufficient to enable her to distinguish one face from another. In the left eye there is only slight light perception. There is an extreme degree of atrophy in both optic nerves. The retinae are unusually vascular, and present a red, velvety appearance. She remained in the hospital seven days, and during this time she had, on an average, from three to four seizures every day. Her temperature on admission and for a few days subsequently remained about nor- mal, or from a half to one degree subnormal. The pulse varied from 70 to 100, the respirations from 18 to 26. During the last days of her stay in the hospital the temperature was about one degree above normal, pulse varied from 80 to 102, the respirations most of the time from 20 to 34. Her appetite was good, she was well nourished, and when not suffering from pain was bright and cheerful. Her bowels were obstinately 4 constipated, requiring large enemas to effect a move- ment, as the ordinary purgatives were inefficient. The numerous seizures that were observed did not vary greatly in character, and a description of one, with a few additional remarks, will suffice for all. I witnessed two of these attacks. While I was talking with her, she seemed to be free from pain and was laughing, when suddenly she cried, " Oh, mamma, mamma ! " her face flushed, head was extremely re- tracted, spine arched backward, arms extended and rigid, right leg flexed at the knee and hip, the left straight, and all the muscles of the body seemed to be rigid. The hands were closed tightly. While in this condition, and apparently suffering intense agony, she was entirely conscious and would answer if spoken to ; respiration, pulse, and temperature were about normal. Pupils were dilated and did not respond to light. The spastic seizure which I observed lasted only two or three minutes, when the muscles began to re- lax. She seemed greatly exhausted and moaned for several minutes after the attack was over. She said that she was entirely conscious throughout the spasm and that the pain was very great, especially in the back of the neck and head. Several of the attacks observed by the nurse were twenty or thirty minutes in duration, and during some of them she would become almost frantic with the pain. On one occasion, while trying to bite her own hand, she begged the nurse to strike her head, punch out her eyes, as the pain in the front of the head was so intense. The prolonged spells ex- hausted her greatly, and the pain in the back of the head and neck following them would frequently con- tinue for several hours or until she would have another attack. If the pain did not continue long after a seiz- ure, each seizure would be followed by a period of moan- ing and restlessness, which would often end in sleep. About 8 a.m., Feburary 26, 1895, the day that I had promised her that her mother might take her home, 1 5 saw her while she was eating her breakfast, consisting of eggs, toast, and milk, which she seemed to relish very much. She was entirely free from pain, said she felt well, and was happy at the thought of going home that day. The nurse reported, however, that she had suffered from three attacks the previous night ; that while all had been longer in duration and greater in severity than those which she had previously witnessed, each seizure seemed to be worse than the one which preceded it. She remained in about the condition in which I saw her until noon, when suddenly she began to scream at the top of her voice, " Mamma, mamma, mamma ! " and immediately an intense rigidity seized every muscle, and the head was retracted to an ex- treme degree, the spine was arched backward so that she lay on her abdomen with head and feet in the air. The chest muscles and diaphragm were contracted, respiration ceased for a time, and the face became deeply cyanosed. Later, respiration became irregular, slow, and Cheyne-Stokes in character, and the pulse slow and full. This seizure lasted one and a half minutes, but she was no sooner out of one than she was seized by another of greater duration and intensity than the first. These attacks continued in rapid suc- cession, each one growing more severe until the fifth occurred about 2 p.m., when respiration ceased. The heart continued to beat for several minutes while arti- ficial respiration was kept up. When she died the face was deeply cyanotic. The autopsy was made three and a half hours after death by Drs. McNaught and Hopkins. The vessels and membranes of the brain presented a normal ap- pearance. The cortex and white substances of the brain showed nothing pathologic. The lateral ventri- cles were distended by about four ounces of a straw- colored, watery fluid. The left hemisphere of the cerebellum was apparently about twice as large as the right. The increase in size was apparently due to 6 fluid in the posterior portion of the left hemisphere, while the anterior portion seemed to be nearly normal, and no undue pressure appeared to be exerted on the pons or medulla. On cutting into the left hemisphere of the cerebellum a tumor was found about three- fourths of an inch in diameter in its extreme posterior tip. The growth was hard in one portion, while in another two or three small-sized cysts containing a gelatinous fluid were found. Connected with this growth was a large cyst two or three inches in diameter and extending from near the posterior portion of the cerebellum to within one-half or three-fourths of an inch of its anterior portion, containing a thin, straw- colored, watery fluid, with here and there, and espe- cially lying against the walls of the cyst, some rather thick gelatinous-like substance. The cyst-wall was quite thick, well defined, tough and apparently fibrous in character. Microscopic Appearance.1-"The tumor consisted of two portions-one, the external, hard and almost fibrous, and the other softer. The internal portion was toward the cyst. At the sides passing into the cerebellar tissue the growth was infiltrating in char- acter. The soft portion varied somewhat from the mere gliomatous and the more distinctly sarcomatous structure. In parts there was a marked proliferation both of the endothelium and the adventitia of the smaller blood-vessels, a new formation of capillaries, to such an extent as to give the appearance of an angioma. Many of the blood-vessels were widely di- lated, and there had been hemorrhages in parts, with breaking down of tissue and the formation of small cysts. The wall of the large cyst was distinctly fibrous in character." Simple non-parasitic cysts of the brain are most com- monly the result of acute destructive lesions, usually of 1 By Dr. H. C. Crouch, 7 vascular origin, such as hemorrhage or arterial occlu- sion ; the effused blood or disintegrated nerve elements having been removed, the cavity becomes a receptacle into which the watery portion of the blood finds access. Such cysts are often irregular in their outlines, and the wall may not be well defined. Distinct cysts of a regu- lar shape and with firm fibrous walls sometimes form in connection with traumatic injuries of the membranes- a beautiful example of which I have recently observed in consultation with Dr. McNaught. It occurred from a blow received on the head nearly thirty years before, and gave rise to headache, mental disturbance, and epileptic seizures, which were relieved on removal of the cyst. Tumor, especially the glioma or sarcoma, of the brain may contain several small cysts, or a large cyst, as in the case just reported, may be connected with a small tumor. Such cysts have their origin most probably in the results of changes which take place in the growth, and after they attain a certain size they become much larger than the tumor from the disten- tion of the cyst-wall by means of the watery portion of the blood. In the present instance careful microscop- ical search was made for granular and broken-down pus corpuscles, as trauma, distant suppuration, and otorrhoea, all causes of abscess of the brain, had been experienced by the little sufferer. The diagnosis was a most difficult and, as proved by the autopsy, an unattainable problem-for me, at least. The history of the seizures, preserved consciousness, talking, throwing the arms wildly around without dis- tinct clonic convulsive movements, and opisthotonus, at first suggested hysteria, but this was easily excluded by the optic-nerve atrophy and vascular changes in the fundi. If these had not been sufficient to enable me to diagnosticate an organic lesion, the attacks that I witnessed would have been ample evidence of organic change. They were typical of irritation in the pos- terior cerebral fossa, especially around the pons and 8 medulla. The posterior neck muscles were as rigid as cords, the head was retracted, the spine arched back- ward, the masseters were at times firmly contracted, so that when she attempted to speak, her teeth, which she could not separate, were simply uncovered by the lips. The arms, rigidly extended, were thrown wildly around with shock-like convulsive movements. Three morbid processes were considered in the diag- nosis, viz. : abscess, tumor, and the results of an old basilar meningitis. The history of the bilateral otor- rhcea, with a very offensive discharge, occurring about the time of her first nervous symptoms, and attended by a convulsion ; the suppuration in the right groin, the origin of which could not be determined, as there had never been any further evidence of caries of the spine ; and a blow to the head received just previously to the convulsive seizure becoming frequent, from an etiological stand-point were about as strong evidence in favor of cerebral suppuration as one expects to find. The subnormal temperature during the first few days of my observation of the case was also in favor of chronic cerebral abscess. The otorrhcea had ceased for a period of nearly ten years, and during this time the child had never complained of pain in the ears. It seemed to me that if the little patient was suffering from abscess of the brain the probabilities were against the ears having been the source of the septic poison, as a chronic abscess from ear infection rarely runs a very prolonged course. Headache is common to all of the pathological processes under discussion. The optic-nerve atrophy without any swelling of the disks was in favor of old meningitis and against the pressure of a growth or an abscess. I felt inclined to exclude chronic suppuration, but I admitted to the physician, Dr. McNaught, with whom I saw and studied the case, that I had not sufficient reason? to be positive in my opinion. Between meningitis and tumor I could not decide. 9 One of these morbid processes would have accounted for some of the symptoms, while certain ones were more common to the other. The almost total loss of sight with optic-nerve atrophy, and the seizures at- tended with opisthotonus, rigidity of the masseters, tonic extensor spasms of the extremities, especially the superior ones, and shock-like movements of the arms are found in meningitis in the posterior fossa when the sur- face of the pons is irritated, and not in tumor as a rule unless meningitis is also present. So far the symptoms and the theory of meningitis harmonized, but I was at a loss to account for the other cranial nerves than the optic escaping. That the child at times should feel quite well, be entirely free from headache or any dis- comfort, and that there should be no rigidity of the posterior neck-muscles except during the paroxysms, seemed to me inconsistent with an old meningeal exu- date in the posterior fossa. A tumor in the cerebellum so situated as to produce remittent pressure on the pons, with distinct paroxysms of increasing pressure, is common to the experience of everyone who has studied and observed cerebellar growths, but for the pressure symptoms to be apparently completely intermittent, and to return with as much regularity as epileptic seiz- ures, and to consist solely of epileptoid phenomena without mental dulness or loss of consciousness, was new in my experience. I confessed my inability to make even a problem- atic diagnosis, and advised the mother to take her child home, as I could not recommend an operation for her relief, but about an hour before the time the mother had set to leave the hospital the child was seized with a series of convulsions which proved fatal within two hours. While the body was on the post-mortem table I was asked by Drs. McNaught and Hopkins, before they began their examination, for my diagnosis. I said that a double lesion, a tumor in one hemisphere of the cere- 10 bellum, and an old meningeal exudate would explain all the symptoms observed during life, but not the ab- sence of others. In the light of the autopsy which revealed the pres- ence of a large cyst and a small tumor in the posterior three-fourths of the left cerebellar hemisphere, are the symptoms explicable ? It is possible that, owing to the watery contents of the cyst, the pressure exerted by the cyst, after it became chronic, on the adjacent structures was not as great as results from a more solid and unyielding tumor, so that, while there might have been a papillitis during the early stage of the growth, the lessened pressure on the formation of the cyst and the breaking down of the growth allowed this to sub- side with the resulting atrophy of the optic nerves, and time had permitted all intra-ocular exudates to be ab- sorbed. We know that a growth in the cerebellum, from its effects upon the circulation, especially in the veins of Galen and adjacent veins and sinuses, disturbs the function of the pons and medulla, usually to a slight extent continuously, and when it has reached sufficient size to exert pressure on the respiratory and neighbor- ing centres, by paroxysms, threatening life by increased pressure which probably results in temporary oedema of the parts. In the case of a cyst with yielding contents, which may vary in quantity from time to time, the pressure symptoms probably may become intermittent. The peculiar tetanic spasms were, most likely, due to tem- porary irritation of the pons and medulla. With the information gained from the clinical and pathological observations of this case, could error be prevented in the diagnosis of a somewhat similar one ? If a large cyst of the cerebellum is attended with more varying pressure-symptoms than a tumor, a problematic diagnosis might be ventured, and it might be correct or it might not. The only unilateral symptom in the case was the tendency of the right arm to become a little 11 more rigid than the left, with, at times, a turning of the head and eyes to the right, but this was inconstant and of no localizing value in determining which hemisphere of the cerebellum was affected. Had it been possible to localize accurately the lesions, the tumor, in all proba- bility, could have been removed, the contents of the cyst evacuated, and the life of the patient saved. A careful study of the case just reported demon- strates how meagre and unsatisfactory are the symp- toms of an extensive lesion in one lateral hemisphere of the cerebellum, and that ataxia from lesions of this portion of the brain probably only occur when they are situated so as to exert pressure on the pons, usu- ally through the medium of the middle lobe of the cerebellum.