On The Pathology and Treatment of Cerebral Disease
190 r Art. IV.?O :Author: Robert Hunter Semple, M.D., Fellow of the Royal College of Physicians of London. In a paper on Cerebral Disease written many years ago, and read before the Westminster Medical Society, I remarked that the only true basis of the classification of the diseases of the nervous system was to be sought in Pathology, and that in all fatal cases a diligent post-mortem examination should be in- stituted. In making this remark, however, I admit that in the present state of our knowledge the seat of disease in the nervous centres cannot always be precisely determined by the scalpel of the anatomist, even when aided by the magnifying powers of the microscope. Nevertheless, the attempt should always be made, and it is almost a truism to affirm that no person can be qualified to give a positive opinion upon a fatal case of cerebral disease unless such an investigation has taken place.
In accordance with these views, I have, in all cases where I found it practicable, made post-mortem examinations of the patients I have attended during life, as well as of many others where, to satisfy my own mind, I have examined the bodies of those who had died suddenly before medical aid could be obtained. From a diligent series of enquiries of this descrip- tion, I have collected a large amount of materials, some of which are of a negative character, and others are in contra- diction to nosological systems and theories; but others, again, have thrown so much light upon cerebral disease, and have often cleared up so much obscurity, that I feel grateful that such opportunities for enquiry have been presented to me, and I earnestly advise others to pursue a similar course, and to record the phenomena they observe.
To one who has been taught by the practical lessons of the dead-house, the artificial distinctions laid down and insisted upon by nosographers are comparatively unimportant; and although it is of course necessary to retain such terms as apoplexy, epilepsy, &c., yet these and similar words merely help to define certain groups of symptoms, which often depend upon causes wholly distinct from one another, and sometimes opposite in their nature. Thus, convulsions may arise from dentition, from inflammation of the cerebral membranes, from the pressure of exostoses or of specula of bone, from the pre- sence of undigested or indigestible matter in the alimentary canal, from worms, from uterine irritation, from toxaemia, from excess of blood in the system or from a deficiency of the same fluid, or from the action of certain poisons. The abolition of sense or motion, or of both?or, in other words, the condition usually called apoplexy?may be caused by narcotic agents, by the inhalation of deleterious gases, by mechanical pressure, by drunkenness, by congestion of the cerebral vessels, by hysteria, by effusion of blood or of serum within the cranium, by softening of the brain, by intracranial tumours, or by hydatids.
In many cases it must be fairly and honestly admitted that a correct diagnosis during life is simply impossible, and in such cases a post-mortem examination is the only mode of solving the difficulty. As I intend, as far as possible, to illustrate the remarks made in this paper by a reference to facts observed by myself, I adduce the following example of the observation just made. A year or two ago a young woman, of slender make, without any hereditary disease, was a patient under my care at the Bloomsbury Dispensary; she complained of violent pain in the head, but otherwise did not seem seriously ill, and was manifestly hysterical. The senses and the intellect were not impaired. She died rather un- expectedly, and it was totally impossible to assign any cause for the fatal event. But on making a post-mortem examina- tion, a large abscess, of about the size of a hen’s egg, was found on the right side of the brain, and connected with, and indeed caused by, a carious condition of the petrous portion of the temporal bone on that side. I made a diligent examina- tion of the course of the disease, but could not find any com- munication towards the exterior of the body, and no discharge from the ear had ever occurred during life. It was certainly most extraordinary that so serious and extensive a disease should have caused during life such comparatively slight sym- ptoms, but the case is far from being an uncommon one in the records of cerebral pathology.
The case now to be recorded is even more curious; and although it occurred many years ago, I have never seen a similar instance. There was, indeed, evidence at the last of some brain-disease, but what that disease was must have re- mained a mystery but for the revelation made by the post- mortem examination. The patient was forty-six years old, and was an inmate of an infirmary which I at the time attended. She had a bloated, leuco-phlegmatic appearance, and was of rather torpid intellect, but she had not suffered from any remarkable ailment imtil the period of the fatal attack. She was sensible at first, but complained only of cough, difficulty of breathing, and general uneasiness. She was, however, anasarcous, and the face was swollen and the lips were livid, and she gradually sank into a state of coma, her breathing being stertorous, her pulse very feeble, and her extremities cold; and in this state she died. The post-mortem examination showed that the kidneys were healthy, although the anasarcous condition of the body might have led to the suspicion of renal disease, and the abdominal viscera, heart, and lungs were likewise healthy, with the exception of some evidence of chronic bronchitis, which had been detected and pre- scribed for during life. The brain, however, to my surprise, presented the following remarkable appearances. As I was examining the surface beneath the arachnoid membrane, I observed some semi-transparent masses in one or two places, and at first imagined them to be local effusions of serum ; but on investigating them more closely, I found that they could readily be detached from the surrounding tissues, and were in fact hydatids, each consisting of a flask-shaped mass, having a thick skin and a gelatinous interior. I now instituted a diligent search for these hydatids in all parts of the brain, and found many of them on the exterior lying between the sulci, and overlapped and hidden by the convolutions?all being beneath the arachnoid membrane. One of them was found lying, free and unattached, in the left ventricle ; it was much softer than the others, and resembled a small oval mass of jelly of a yellow colour. Another, much smaller, and rather hard, was found lying upon the optic nerve, on the right side, at the part where the nerve winds round the crus cerebri. Altogether I collected about eighteen of these hydatids, varying in size from that of a bean to that of a small pea, and there were also a number of others scattered about in various parts of the brain, but they were so small that I could not extract them entire. They did not exist so abundantly in the white as in the cineritious substance, and they were wholly wanting in the cerebellum. None were found in any other part of the body. The hydatids were taken by me to Professor Sharpey, who recognised them as examples of the cysticercus cellulosce, and they are now in the Museum of University College, London. I have never seen another case of this kind, either before or since, and I believe the occurrence to be very rare. I, how- ever, at the time investigated the literature of the subject, and saw a coloured plate in the University College Museum, and executed by the late Sir Robert Carswell, representing a number of subarachnoid cysts, similar to those I have described. Dr Copland, in liis Dictionary, mentions the fact of hydatids being found on the surface of the brain, but gives no case from his own experience; Dr Abercrombie, in his work on Diseases of the Brain and Spinal Cord, also mentions the fact, but gives no case of his own. Mr. Solly, in his work on the Brain, alludes to the existence of hydatids in that organ, but gives no case from his own practice; and the French pathologist Cruveilhier states that he has met with hydatids beneath the arachnoid membrane, but gives only one illustration, which appears in the plate more like a large cyst than a true example of a cysticercus.
In determining the causes of sudden death, post-mortem examination is absolutely essential in arriving at any conclu- sion; and although I fully admit that many such cases are attributable to disease of the heart, yet cerebral disturbance accounts for a far greater number than is usually sup- posed. I agree with Trousseau, however, in the statement he makes, that haimorrhagic apoplexy does not occur suddenly, but gradually; and lest I should be accused of presumption in placing my own experience in juxtaposition with that of so distinguished an observer, even for the purpose of confirming it, I may state that for many years of my life I had peculiar opportunities of watching the mode of death in apoplexy, for I attended a large infirmary, in which there was a great number of old people, and I was often present when the apoplectic attacks took place, or attended very soon after they occurred. I had, therefore, good means of observing the progress and termination of the disease, and as I have before remarked, I made post-mortem examinations in every case where it was possible to do so?that is to say, whenever the violent opposi- tion of the relatives did not forbid such an enquiry. I may also state that I had opportunities, which the medical officers of large hospitals seldom enjoy, of watching the cases which recovered for many years afterwards, and also of knowing for many years beforehand the history and habits of those who were attacked.
Although, therefore, many of these cases of cerebral disease died suddenly, they were not instances of hemorrhagic apoplexy, but of the congestive form of the malady; and in those who died of the former variety, although I frequently witnessed the first attack, yet there was an appreciable interval of some hours, or of some days, between the onset and the fatal termination. As the cases of sudden death from apoplexy of any kind are cer- tainly somewhat rare, though not so rare as is generally supposed, I subjoin the following as an instance :?A girl, aged 17, an inmate of the infirmary alluded to, had been under medical treatment for some time, on account of suppression of the menses; she was stout, healthy-looking, and of a florid com- plexion. The menstrual discharge was not restored, but one night, after eating her supper, she complained when in bed of some pain and uneasiness, but not of so severe a character as to induce her to ask for medical assistance. The next morning she was found dead in her bed, with her face downwards. A coroner’s inquest was held on the body, and, as there were no circumstances of suspicion attaching to the case, a verdict of ” Natural Death” was returned. After the inquest I made a post-mortem examination, and found the vessels of the scalp turgid with blood; and on removing the skull, the brain was seen to be in a high state of congestion, the veins and sinuses being greatly distended with fluid blood. Beyond this general congestion, however, the brain presented no morbid appearance, and no extravasation could be detected in any part. The heart and lungs were quite healthy, and so were the stomach, in- testines, and liver. The bladder was empty, and the uterus was small, of healthy structure, and with its cavity empty. In this case there can be little doubt that death was due to cerebral congestion, and in all probability this congestion was connected with the suppression of the menses, and might have been relieved or averted if that natural evacuation of blood had taken place.
The following is another case of sudden death from cerebral congestion, or congestive apoplexy, although in a very different subject:?A gentleman, about 55 years of age, of a stout and plethoric configuration, of perfectly strict habits, and in easy circumstances, but fond, for amusement, of making abstruse mathematical calculations, had always enjoyed perfectly good health up to the time of the fatal seizure. He lived next-door to me, and I was well acquainted with him, but for the reason just mentioned I had never been requested to attend him pro- fessionally. One morning, about 10 o’clock, I was requested to see him immediately, as he had fallen down in a fit. I lost not a moment in going to him, but before I arrived he was dead. There were no circumstances of suspicion attaching to any person, and a coroner’s inquest returned a verdict of ” Natural Death.” I made a post-mortem examination, and found great congestion of the scalp, and, on removing the skull, the veins and sinuses on the surface of the brain were all turgid with blood, which flowed freely when they were cut, but on the most careful examination no extravasation could be detected in any part of the brain. All the other parts of the body were minutely investigated, but, beyond congestion of some of the organs, and a large quantity of fluid blood in the right side of the heart, no morbid appearances could be anywhere detected. This case was, I think, undoubtedly one of congestive apoplexy, or apoplectiform cerebral congestion.
Among the anomalous diseases which sometimes simulate apoplexy, softening of the brain is one of the most prominent and remarkable. Many attempts have been made to establish the diagnosis of this mysterious malady during life, but none have hitherto been entirely successful. The distinguishing- features of it after death are plain enough, and its pathology, or rather its pathogeny, in many instances is quite intelligible; but it is still a desideratum in practical medicine to determine the symptoms which clearly denote its presence. I myself’ have long been convinced of the inutility and, indeed, the pre- sumption of attempting to form any dogmatic views on the subject; and Trousseau was evidently of the same opinion, for in one of his lectures he declares, in answer to enquiries from his pupils, that the diagnosis between cerebral hemorrhage and softening of the brain is ” one of the most difficult problems in pathology.” He, however, agrees with Recamier in thinking- that the sudden occurrence of hemiplegia (he insists on the suddenness of the attack), without loss of consciousness, is cha- racteristic of softening of the brain; and Dr Todd, in his- ” Clinical Lectures on Nervous Diseases,” entertains a similar opinion. Notwithstanding these high authorities, however, I maintain that the diagnosis is still exceedingly obscure, as the following case of undoubted softening of the brain will amply demonstrate.
A man, aged 62, of middle size, stout, and with short neck, said to have been of intemperate habits, was attended by me some years ago. He had a bewildered air, though he was quite able to answer questions put to him, but his appearance was dull and heavy. The head was rather hot, and he complained of great pain; the pupils were fixed between contraction and dilatation. Pulse 100, full and strong; tongue clear; bowels rather confined. There was no paralysis, convulsion, or spasm of any part of the body. My impression was that the man was on the point of having an apoplectic attack, and I strongly advised his wife to place him under careful medical supervision; and in the meantime I directed him to be bled, which was done. Although I now admit, after reviewing the whole history of the case, that this step did no real good, it certainly did no harm, for the patient experienced great relief from the pain in his head, and, in fact, left the infirmary and went home. He was, however, soon brought back again, as he had not materially improved. He did not present any peculiar appearance, but his intellect seemed rather dull; he, however, answered questions quite rationally. He was not paralysed, and bad no rigidity or convulsions in any part. My notes, made at the time, state that “2 ivas satisfied of this from my own observation; and al- though I frequently sought for and expected some of these symptoms, none made their appearance” The head -was cool and moist; tongue clean; pulse 80, weak, and occasionally intermittent. The urine was albuminous. The hair of the head was cut close, and a blister was applied to the nape of the neck. The other symptoms were very anomalous, and he was rest- less and irritable, tearing off the blister, which, however, had risen previously. Rather more than a week after his re- admission under medical treatment, this patient was seized with violent epistaxis from the left nostril, which was therefore plugged, and he died soon afterwards somewhat unexpectedly.
The post-mortem examination revealed an extraordinary amount of softening of the brain, together with extensive disease of the cerebral arteries, to which the softening was of course due. The internal surface of these vessels was lined with an atheromatous and calcareous deposit, giving them the feeling and consistence of hard rigid tubes. The surface of the brain was almost uni- versally healthy in appearance, and the colour pale ; but on removing the upper portion of the cerebral mass on a level with the corpus callosum, and then examining the ventricles, I found that the fornix, the optic thalami, the corpora striata, and the substance adjoining, were all in a softened state and of about the consistence of clotted cream; but the colour of these struc- tures was not changed. Some portions of the softened mass were examined by the microscope, and found to present a number of fat and pus-globules, oil-cells, and disintegrated nerve-cells and fibres. As to the other organs of the body, there was found hypertrophy of the left ventricle of the heart, but without valvular disease, and the kidneys were contracted and cirrhotic. This is, perhaps, as striking an instance of softening of the brain as can be adduced, and its cause, the rigidity and calci- fication of the arterial coats, is clearly manifested. In reference to treatment, my present remarks apply only to the subject of apoplectiform seizures.
The treatment of the diseases usually called apoplexy? which, according to the views already expressed in the preceding pages, is a rather vague term?is a subject still involved in considerable doubt, and one on which great difference of opinion is now entertained. At no remote period in the history of Medicine, the principles of treatment of this disease were clearly laid down, and the practice followed logically from the principles. It was supposed that apoplexy was a disease especially and uniformly caused by a superabundance of blood in the cranial cavity, that this blood pressed upon and so impaired the functions of the brain, and that the remedy therefore was to withdraw blood from the current of the circulation. Nor can it be alleged that these principles and this practice were incapable of being defended by strong arguments. A patient meets with an accident, by which a portion of the skull is depressed upon the surface of the brain, and apoplectic phenomena necessarily and almost constantly ensue; but when this pressure is artificially removed, by the operation of trephining, the functions of the brain are restored. Equally striking are the beneficial changes which often ensue from a spontaneous discharge of blood from some of the natural outlets of the body in cases of cerebral congestion?as, for instance, where a threatened apoplexy is warded off, or an apoplectic condition is relieved, in a female by the flow of the menses, or in a man by the hemorrhage from piles. Examples, too, were by no means wanting (and I have often seen such cases myself), where a prompt bleeding, practised in the case of a patient who had fallen down in an apoplectic fit, was attended with immediate relief, and followed by a disappearance of all the symptoms. Such a person, in fact, was overpowered by the pressure of blood on his brain, and experienced the same partial abolition of the vital powers as he would have done if some extraneous body had been artificially introduced between the skull and the cerebral mass, or as if some depressed portion of the skull had produced a similar effect; and the removal of the pressure, whether caused by mechanical means, or by abnormal fulness of the cerebral vessels, would be followed by immediate relief.
Such are the modes of reasoning which may be adopted in favour of the abstraction of blood from the system in cases of apoplexy; and if it were proved that the disease always de- pended upon mere fulness of blood within the cranial cavity, the practice might still perhaps be amply justified. But, unfortunately, the progress of pathological enquiry has shown that mere superabundance or deficiency of blood has but little connection with the causation, or the prevention, or the cure of many cases of apoplexy; and that the conditions of the disease are to be sought very often in diathetic and molecular changes in the composition of the tissues, rather than in any excess in quantity, or abnormality in composition, of the circulating fluid itself. The view formerly entertained as to the nature of apoplexy assumed the integrity of the vessels, and attributed the morbid phenomena to the force exercised upon those tubes by blood, superabundant in quantity and rich in solid con- stituents ; but recent researches have compelled the physician to regard the whole question from a totally different point of view. There are, it is true, some human beings so happily constituted that, during a long life, no diathetic malady has sapped the foundations of their health, or led to degeneration of any of their tissues; and such persons attain to a tranquil old age, and at last die from sheer wearing-out of the bodily framework. But in too many instances, either latent maladies, hereditary or acquired, or more open and obvious visitations of disease, are exerting their baneful influence among the tissues, are causing hypertrophy in some and atrophy in others, are softening one set of organs and hardening another set, are converting elastic vessels into brittle or rigid tubes, or are “blocking up those tubes with solid plugs, or are introducing oil-globules in the place of healthy cells and fibres and sarcous elements; and, in short, like the trap-doors lying concealed on the bridge rof life, as seen in the vision of Mirza,* they are stealthily undermining the integrity of the organs and tissues ?of the body, and a ” plurima mortis imago,” as Virgil expresses it, is leading the victims, by multiform aspects of disease, into the gulf of eternity.
When, therefore, the essence of apoplexy is proved to consist of a brittleness of the coats of arteries, leading to their easy rupture and the extravasation of their contents, or when the same disease is induced by the bursting of a cerebral aneurism, it is idle to expect that blood-letting, or indeed any other measure, will remedy the primary source of the mischief. But, on the other hand, it must be recollected that, during life, it is often impossible to distinguish with certainty the cases due only to congestion from those in which there is disease of the arterial coats; and it is still more important to bear in mind that, even when extravasation has ensued from the latter condition, the case is not hopeless. The effused blood may be, and very often is, absorbed, and the patient is restored to health, perhaps to undergo subsequently another seizure, or even a succession of seizures.
I cannot, therefore, altogether agree with Trousseau in his indiscriminate condemnation of blood-letting, in any form, in every case of apoplexy; and looking back to three of the cases recorded in the present paper, I cannot help thinking, if the menses had returned (or, in other words, if there had been a loss of blood), in the case of the girl who died of congestive apoplexy, and if, in the case of the gentleman, who died suddenly from the same cause, some blood could have been abstracted, that in both cases life might have been pre- served. In the case of softening of the brain, too, although I have admitted that the bleeding did no good, it clearly did no harm, and, indeed, Nature made a spontaneous effort to relieve the symptoms, a copious epistaxis taking place a very short time before death. Nor, while I agree with Trousseau that blood- letting is often useless in sanguineous apoplexy, can I admit that such a measure is always injurious; and I altogether disagree with him in regarding congestive apoplexy and epilepsy as identical diseases, a view which he clearly sets forth in his lectures.*
The subject of treatment in cerebral diseases is a very wide and a very difficult one, and one on which I have no dogmatic opinions to offer. For the present, I must repeat the observa- tions which I made and published many years since?namely, that the treatment must depend, not upon the name given to the disease, but upon its pathology, as indicated by its diagnosis.
The diagnosis is often very difficult, and this difficulty should inspire great caution in the therapeutical means employed. Still there are positive and negative indications which should guide our treatment, and which are often sufficiently plain when carefully considered. I fully indorse the opinion of Trousseau, that blood-letting has too often been performed in an unnecessary and injurious manner in cerebral disease; but, nevertheless, I still think that this mode of depletion is very valuable when cautiously performed, and when the state of the pulse admits of its adoption. As to other measures, they are, and must be, of the most varied description, for one case may be benefited by emetics and cold affusion; a second shall yield to opium and brandy; a third shall require the cautious abstraction of blood; a fourth shall demand the use of calomel and purgatives; a fifth shall be relieved by generous diet; a sixth shall imperatively necessitate a system of rigid abstinence; and a seventh shall with propriety be left to take its course. Nullius addictus jurare in verba magistri, Quo me cunque ferat tempestas deferer hospes. * Lectures on Clinical Medicine, Lecture II.
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