CEREBRAL PALSY OF CHILDHOOD FOLLOWING DIPHTHERIA. BY CASPAR W. SHARPLES, M.D., OF SEATTLE, WASH. FROM THE MEDICAL NEWS/ August 4, 1894. [Reprinted from The Medical News, August 4, 1894] CEREBRAL PALSY OF CHILDHOOD FOLLOWING DIPHTHERIA) By CASPAR W. SHARPLES, M.D., OF SEATTLE, WASH. Comparatively recently a group of cases has been recognized as the cerebral palsies of childhood, having definite relations one to another and separate from ordi- nary infantile palsy. The condition often makes its appearance as a sequel of some acute infectious disease, such as scarlet fever, measles, etc. Most of the cases offered for study are those of months' or years' duration, and when seen they present as the one characteristic a spastic state of the muscles of the affected side, associ- ated, if of long standing, with a decrease in the size and length of the limbs. Many present various evidences of cerebral disturbance, as epilepsy, idiocy, etc. The occur- rence of cases of this class of hemiplegia after diphtheria is rare. Among one hundred and sixty cases of spastic hemiplegia tabulated by Wallenberg only three cases followed diphtheria or croup; nine followed measles ; thirteen, scarlet fever ; and six, epidemic cerebro-spinal meningitis. Of Osler's one hundred and twenty cases none was reported to be due to diphtheria; and if I re- call correctly none of the cases tabulated by Dr. Sarah McNutt, in her paper on " Spastic Hemiplegia, " followed diphtheria. To say just what has taken place in the brain in these 1 Read at the annual meeting of the State Medical Society ol Washington, May 2 and 3, 1894. 2 cases is difficult, for autopsies in cases of recent date are rare, because most of them survive the immediate dan- gers of the primary condition, and new processes obliter- ate to some extent the original state of affairs. Sclerosis, then, is the most common condition found, with poren- cephalon, simple cysts, and softening as rarer conditions. A recent German journal contained the report of a case very much like the present one. About six days after the diphtheric membrane had disappeared the boy suddenly became comatose; on emanating from this condition he was found completely paralyzed on his right side, and was also aphasic. After the lapse of five months the aphasia had to a great degree disappeared and motion was returning in the arm and leg, although he still pre- sented the hemiplegic gait. This is the only case of the kind of which I have seen any record. I venture to report this case on account of the infre- quent occurrence of similar ones. I am indebted to Dr. L. R. Dawson for the privilege of seeing the case and reporting it. J. C., a boy, thirteen years old, had an attack of diph- theria a short time after his father recovered from an attack of erysipelas of the face following a scratch from a fall. Before the membrane had disappeared, and although he was apparently doing nicely, his heart be- gan to be irregular and rapid. About midnight, Decem- ber 4, 1893, he was suddenly paralyzed upon the entire right side. Coma was not deep or long, for in a half- hour the boy realized what was going on around him. No convulsions accompanied the onset. Preceding the attack, during the evening, the child frequently cried out, making a peculiar sort of noise. This was continued during the day and especially if he was disturbed in any way. I first saw the patient twenty-four hours after the onset of the trouble. His mental state was improving. The right side of the body was the seat of tremors that shook 3 the entire body. These would last off and on for a half- hour and would recur at irregular intervals during the day. The skin was hypersensitive. Touching the leg would cause the boy to cry out as if in pain. The arm did not present these phenomena to the same extent as did the leg. The tendon-reflexes at the time were absent. Speech was wanting, although the child appeared to understand what was said to him, and was able to make his displeasure known. His heart was behaving very poorly; its action was tumultuous, irregular, and weak, so that death would not have been a surprise at any time, and it so continued for three or four days, gradually coming down to normal. No heart-murmur was de- tected. The urinary secretion was scant and passed in- frequently. The bowels only moved after the adminis- tration of drugs or if injections were used. This report is made four months after the onset. There has gradually been a return of the power of speech and of motion. Motion began to return in about five weeks, when the boy made an effort to walk with assistance, and now he gets along by himself very nicely, with the ordinary hemiplegic gait. For a time, to obtain any motion in the right hand and arm, he had to use the left to the same purpose, when the right would coordinate; yet the use of the left arm did not always excite motion in the right. Two weeks ago when he attempted to squeeze my hand, he made a very strong left-handed contraction and a scarcely perceptible right-handed one. To-day he could raise his arm a short distance from his body, and could extend the little and ring fingers so that they were nearly straight. Athetosis has not been present, though at times while moving the left arm the fingers of the right would assume a position approaching athetosis. This now has disap- peared. As is common in ordinary hemiplegia and as is char- acteristic of hemiplegia in children, the tendon-reflexes have been increased. They are more marked in the leg than in the arm. Ankle-clonus could not be elicited. The skin-reflexes are still exaggerated. Electric exami- nation does not show any reaction of degeneration. In both the arm and the leg the galvanic current causes a contraction of the limb-acting, I would say, through the skin, and producing an exaggerated skin-reflex. The size of the limb has decreased, as shown by the following measurements : The right calf measures 10.6 inches; the left calf 10.9 inches; the right thigh 13.1 inches ; the left thigh 13.5 inches ; the right forearm 6.5 inches; the left forearm 7 inches; the right biceps 7 inches; the left biceps 7.5 inches. The aphasic condition has improved and no doubt will continue to improve until the boy can talk fairly well. At first he could not say a word. After commen- cing to talk he would use the wrong word. " Yes'' was the first thing he could say. After naming an object incorrectly, he would recognize his mistake, but could not correct it, and often could not repeat what he was told. He now occasionally joins a couple of words coherently. To-day he said " Papa-Sharples,'' mean- ing that his father had brought him to the office. Occa- sionally he says something " hurts there." So far there has been no mental deterioration, unless it be that the boy is more irritable than he was. He adds, multiplies, etc., as well as ever. For a month the galvanic current has been applied, and within this period he has made more rapid im- provement than at any previous time; yet I would not attribute too much to electricity. There are two other points of interest in the family. His mother had paralysis of the palate, so that degluti- tion was uncertain and speech poor. The sister, a couple of years older than the boy, who was the last of the family afflicted, and had had her feelings much worked 4 5 upon by her brother's paralysis, had two hysterical seizures in which right hemiplegia was quite well insu- lated. It is a matter of common occurrence to find palsies of different types or locations after an attack of diph- theria. These palsies depend on certain local condi- tions, the most common being a neuritis involving indi- vidual nerves, though separate nerves situated in different parts of the body may be affected at the same time. The motor nerves show more profound changes than the sensory ones. Yet we are too liable in looking at a case of post-diphtheric palsy to consider it a peripheral neuritis and to look no deeper. In any lesion of the peripheral portion ot a nerve, de- generative changes travel up as well as down, and if they enter the cord they do so by means of the posterior roots. In a few cases of post-diphtheric palsy the degenerative changes have been found in the anterior roots, indicating beyond a doubt that the lesion has destroyed some of the cells of the gray matter of the cord. One observer has found in the substance of the cord colonies of the same germs as exist in the membrane of diphtheria, and the nerve-sheaths have also been found infiltrated with the same germs. In one case in which the latter condition existed, the brain contained a number of infarcts. These facts have been mentioned to show that if diph- theria can be productive of such conditions in the nerves, and more especially in the cord, it would be only reason- able to attribute a similar causative relation to diphtheria followed by cerebral lesions. Yet it may be justly said that if this were the fact cerebral lesions would be more common after diphtheria than they are. It may be true that diphtheria did not have any more relation to this condition in the case here reported than any other septic or infectious process would have had; and the reason for this would appear the stronger when it is considered 6 that the essentially diphtheric palsies are peripheral and due to an inflammation of the nerves. As to the question of diagnosis, there is no doubt now, nor was there at any time a reasonable one. The case differed from common infantile palsy in the manner of onset, being sudden and immediately complete, while the other develops gradually and progressively. The time of occurrence, in relation to the attack of diphthe- ria, was as would be expected for both conditions. The loss of consciousness was complete for only a short time, while if there had been an extensive hemorrhage into the brain-substance, this loss would have lasted longer. It was such as comes on from cerebral embolism. Some observer has stated that these cases of hemiplegia may be caused by colonies of germs plugging a vessel. Apha- sia indicated the central origin of the lesion, yet if there had been a neuritis of the inferior laryngeal nerves alone, there would have been aphonia. The subsequent state of ataxic aphasia offsets the possibility of any local con- dition. All reflexes were gone at first on the affected side, but have returned. For diagnostic purposes at the time of onset they were of no aid. In diphtheric palsy they only return. In our case they have returned and have also become exaggerated. This change indicates the central origin. The tremor and involuntary movements of the right side indicated that some central irritation was taking place. In one case in which an autopsy was made sopn after the hemiplegia, a very small hemorrhage was found. Porencephalon is attributed to plugging of a cerebral vessel, and as so many cases of cerebral palsy of child- hood show porencephalon, the embolic origin of these cases must be quite frequent. One more point I want to mention, and that is that erysipelas occurred in the house just preceding these cases of diphtheria. The same causes evidently act to 7 produce these two conditions, as they are often found associated in the same houses. Note.-This boy has materially improved in speech and motion, and the size of his limbs has also in- creased. The Medical News. Established in 1843. A WEEKL Y MEDICAL NEWSPAPER. Subscription, $4.00 per Annum. The American Journal OF THE Medical Sciences. Established in 1820 A MONTHLY MEDICAL MAGAZINE. Subscription, $4.00 per Annum. COMMUTA TION RA TE, $7.30 FER ANNUM. LEA BROTHERS A- CO. PHILADELPHIA.