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PATHOGNOMONIC SYMPTOMS EXAMINED--TREMOR COACTUS--SCELOTYRBE FESTINANS 19

SHAKING PALSY DISTINGUISHED FROM OTHER DISEASES WITH WHICH IT MAY BE CONFOUNDED 27

CONSIDERATIONS RESPECTING THE MEANS OF CURE 56

AN ESSAY ON THE SHAKING PALSY.

DEFINITION--HISTORY--ILLUSTRATIVE CASES.

SHAKING PALSY.

Involuntary tremulous motion, with lessened muscular power, in parts not in action and even when supported; with a propensity to bend the trunk forwards, and to pass from a walking to a running pace: the senses and intellects being uninjured.

HISTORY.

So slight and nearly imperceptible are the first inroads of this malady, and so extremely slow is its progress, that it rarely happens, that the patient can form any recollection of the precise period of its commencement. The first symptoms perceived are, a slight sense of weakness, with a proneness to trembling in some particular part; sometimes in the head, but most commonly in one of the hands and arms. These symptoms gradually increase in the part first affected; and at an uncertain period, but seldom in less than twelvemonths or more, the morbid influence is felt in some other part. Thus assuming one of the hands and arms to be first attacked, the other, at this period becomes similarly affected. After a few more months the patient is found to be less strict than usual in preserving an upright posture: this being most observable whilst walking, but sometimes whilst sitting or standing. Sometime after the appearance of this symptom, and during its slow increase, one of the legs is discovered slightly to tremble, and is also found to suffer fatigue sooner than the leg of the other side: and in a few months this limb becomes agitated by similar tremblings, and suffers a similar loss of power.

Hitherto the patient will have experienced but little inconvenience; and befriended by the strong influence of habitual endurance, would perhaps seldom think of his being the subject of disease, except when reminded of it by the unsteadiness of his hand, whilst writing or employing himself in any nicer kind of manipulation. But as the disease proceeds, similar employments are accomplished with considerable difficulty, the hand failing to answer with exactness to the dictates of the will. Walking becomes a task which cannot be performed without considerable attention. The legs are not raised to that height, or with that promptitude which the will directs, so that the utmost care is necessary to prevent frequent falls.

At this period the patient experiences much inconvenience, which unhappily is found daily to increase. The submission of the limbs to the directions of the will can hardly ever be obtained in the performance of the most ordinary offices of life. The fingers cannot be disposed of in the proposed directions, and applied with certainty to any proposed point. As time and the disease proceed, difficulties increase: writing can now be hardly at all accomplished; and reading, from the tremulous motion, is accomplished with some difficulty. Whilst at meals the fork not being duly directed frequently fails to raise the morsel from the plate: which, when seized, is with much difficulty conveyed to the mouth. At this period the patient seldom experiences a suspension of the agitation of his limbs. Commencing, for instance in one arm, the wearisome agitation is borne until beyond sufferance, when by suddenly changing the posture it is for a time stopped in that limb, to commence, generally, in less than a minute in one of the legs, or in the arm of the other side. Harassed by this tormenting round, the patient has recourse to walking, a mode of exercise to which the sufferers from this malady are in general partial; owing to their attention being thereby somewhat diverted from their unpleasant feelings, by the care and exertion required to ensure its safe performance.

But as the malady proceeds, even this temporary mitigation of suffering from the agitation of the limbs is denied. The propensity to lean forward becomes invincible, and the patient is thereby forced to step on the toes and fore part of the feet, whilst the upper part of the body is thrown so far forward as to render it difficult to avoid falling on the face. In some cases, when this state of the malady is attained, the patient can no longer exercise himself by walking in his usual manner, but is thrown on the toes and forepart of the feet; being, at the same time, irresistibly impelled to take much quicker and shorter steps, and thereby to adopt unwillingly a running pace. In some cases it is found necessary entirely to substitute running for walking; since otherwise the patient, on proceeding only a very few paces, would inevitably fall.

In this stage, the sleep becomes much disturbed. The tremulous motion of the limbs occur during sleep, and augment until they awaken the patient, and frequently with much agitation and alarm. The power of conveying the food to the mouth is at length so much impeded that he is obliged to consent to be fed by others. The bowels, which had been all along torpid, now, in most cases, demand stimulating medicines of very considerable power: the expulsion of the faeces from the rectum sometimes requiring mechanical aid. As the disease proceeds towards its last stage, the trunk is almost permanently bowed, the muscular power is more decidedly diminished, and the tremulous agitation becomes violent. The patient walks now with great difficulty, and unable any longer to support himself with his stick, he dares not venture on this exercise, unless assisted by an attendant, who walking backwards before him, prevents his falling forwards, by the pressure of his hands against the fore part of his shoulders. His words are now scarcely intelligible; and he is not only no longer able to feed himself, but when the food is conveyed to the mouth, so much are the actions of the muscles of the tongue, pharynx, &c. impeded by impaired action and perpetual agitation, that the food is with difficulty retained in the mouth until masticated; and then as difficultly swallowed. Now also, from the same cause, another very unpleasant circumstance occurs: the saliva fails of being directed to the back part of the fauces, and hence is continually draining from the mouth, mixed with the particles of food, which he is no longer able to clear from the inside of the mouth.

As the debility increases and the influence of the will over the muscles fades away, the tremulous agitation becomes more vehement. It now seldom leaves him for a moment; but even when exhausted nature seizes a small portion of sleep, the motion becomes so violent as not only to shake the bed-hangings, but even the floor and sashes of the room. The chin is now almost immoveably bent down upon the sternum. The slops with which he is attempted to be fed, with the saliva, are continually trickling from the mouth. The power of articulation is lost. The urine and faeces are passed involuntarily; and at the last, constant sleepiness, with slight delirium, and other marks of extreme exhaustion, announce the wished-for release.

Almost every circumstance noted in the preceding description, was observed in a case which occurred several years back, and which, from the particular symptoms which manifested themselves in its progress; from the little knowledge of its nature, acknowledged to be possessed by the physician who attended; and from the mode of its termination; excited an eager wish to acquire some further knowledge of its nature and cause.

The subject of this case was a man rather more than fifty years of age, who had industriously followed the business of a gardener, leading a life of remarkable temperance and sobriety. The commencement of the malady was first manifested by a slight trembling of the left hand and arm, a circumstance which he was disposed to attribute to his having been engaged for several days in a kind of employment requiring considerable exertion of that limb. Although repeatedly questioned, he could recollect no other circumstance which he could consider as having been likely to have occasioned his malady. He had not suffered much from Rheumatism, or been subject to pains of the head, or had ever experienced any sudden seizure which could be referred to apoplexy or hemiplegia. In this case, every circumstance occurred which has been mentioned in the preceding history.

The subject of the case which was next noticed was casually met with in the street. It was a man sixty-two years of age; the greater part of whose life had been spent as an attendant at a magistrate's office. He had suffered from the disease about eight or ten years. All the extremities were considerably agitated, the speech was very much interrupted, and the body much bowed and shaken. He walked almost entirely on the fore part of his feet, and would have fallen every step if he had not been supported by his stick. He described the disease as having come on very gradually, and as being, according to his full assurance, the consequence of considerable irregularities in his mode of living, and particularly of indulgence in spirituous liquors. He was the inmate of a poor-house of a distant parish, and being fully assured of the incurable nature of his complaint, declined making any attempts for relief.

The next case was also noticed casually in the street. The subject of it was a man of about sixty-five years of age, of a remarkable athletic frame. The agitation of the limbs, and indeed of the head and of the whole body, was too vehement to allow it to be designated as trembling. He was entirely unable to walk; the body being so bowed, and the head thrown so forward, as to oblige him to go on a continued run, and to employ his stick every five or six steps to force him more into an upright posture, by projecting the point of it with great force against the pavement. He stated, that he had been a sailor, and attributed his complaints to having been for several months confined in a Spanish prison, where he had, during the whole period of his confinement, lain upon the bare damp earth. The disease had here continued so long, and made such a progress, as to afford little or no prospect of relief. He besides was a poor mendicant, requiring as well as the means of medical experiment, those collateral aids which he could only obtain in an hospital. He was therefore recommended to make trial if any relief could, in that mode, be yielded him. The poor man, however, appeared to be by no means disposed to make the experiment.

The next case which presented itself was that of a gentleman about fifty-five years, who had first experienced the trembling of the arms about five years before. His application was on account of a considerable degree of inflammation over the lower ribs on the left side, which terminated in the formation of matter beneath the fascia. About a pint was removed on making the necessary opening; and a considerable quantity discharged daily for two or three weeks. On his recovery from this, no change appeared to have taken place in his original complaint; and the opportunity of learning its future progress was lost by his removal to a distant part of the country.

In another case, the particulars of which could not be obtained, and the gentleman, the lamented subject of which was only seen at a distance, one of the characteristic symptoms of this malady, the inability for motion, except in a running pace, appeared to exist in an extraordinary degree. It seemed to be necessary that the gentleman should be supported by his attendant, standing before him with a hand placed on each shoulder, until, by gently swaying backward and forward, he had placed himself in equipoise; when, giving the word, he would start in a running pace, the attendant sliding from before him and running forward, being ready to receive him and prevent his falling, after his having run about twenty paces.

In a case which presented itself to observation since those above-mentioned, every information as to the progress of the malady was very readily obtained. The gentleman who was the subject of it is seventy-two years of age. He has led a life of temperance, and has never been exposed to any particular situation or circumstance which he can conceive likely to have occasioned, or disposed to this complaint; which he rather seems to regard as incidental upon his advanced age, than as an object of medical attention. He however recollects, that about twenty years ago, he was troubled with lumbago, which was severe and lasted some time. About eleven or twelve, or perhaps more, years ago, he first perceived weakness in the left hand and arm, and soon after found the trembling commence. In about three years afterwards the right arm became affected in a similar manner: and soon afterwards the convulsive motions affected the whole body, and began to interrupt the speech. In about three years from that time the legs became affected. Of late years the action of the bowels had been very much retarded; and at two or three different periods had, with great difficulty, been made to yield to the action of very strong cathartics. But within the last twelvemonths this difficulty has not been so great; perhaps owing to an increased secretion of mucus, which envelopes the passing faeces, and which precedes and follows their discharge in considerable quantity.

About a year since, on waking in the night, he found that he had nearly lost the use of the right side, and that the face was much drawn to the left side. His medical attendant saw him the following day, when he found him languid, with a small and quick pulse, and without pain in the head or disposition to sleep. Nothing more therefore was done than to promote the action of the bowels, and apply a blister to the back of the neck, and in about a fortnight the limbs had entirely recovered from their palsied state. During the time of their having remained in this state, neither the arm nor the leg of the paralytic side was in the least affected with the tremulous agitation; but as their paralysed state was removed, the shaking returned.

At present he is almost constantly troubled with the agitation, which he describes as generally commencing in a slight degree, and gradually increasing, until it arises to such a height as to shake the room; when, by a sudden and somewhat violent change of posture, he is almost always able to stop it. But very soon afterwards it will commence in some other limb, in a small degree, and gradually increase in violence; but he does not remember the thus checking of it, to have been followed by any injurious effect. When the agitation had not been thus interrupted, he stated, that it gradually extended through all the limbs, and at last affected the whole trunk. To illustrate his observation as to the power of suspending the motion by a sudden change of posture, he, being then just come in from a walk, with every limb shaking, threw himself rather violently into a chair, and said, "Now I am as well as ever I was in my life." The shaking completely stopped; but returned within two minutes' time.

He now possessed but little power in giving a required direction to the motions of any part. He was scarcely able to feed himself. He had written hardly intelligibly for the last three years; and at present could not write at all. His attendants observed, that of late the trembling would sometimes begin in his sleep, and increase until it awakened him: when he always was in a state of agitation and alarm.

On being asked if he walked under much apprehension of falling forwards? he said he suffered much from it; and replied in the affirmative to the question, whether he experienced any difficulty in restraining himself from getting into a running pace? It being asked, if whilst walking he felt much apprehension from the difficulty of raising his feet, if he saw a rising pebble in his path? he avowed, in a strong manner, his alarm on such occasions; and it was observed by his wife, that she believed, that in walking across the room, he would consider as a difficulty the having to step over a pin.

The preceding cases appear to belong to the same species: differing from each other, perhaps, only in the length of time which the disease had existed, and the stage at which it had arrived.

It has been seen in the preceding history of the disease, and in the accompanying cases, that certain affections, the tremulous agitations, and the almost invincible propensity to run, when wishing only to walk, each of which has been considered by nosologists as distinct diseases, appear to be pathognomonic symptoms of this malady. To determine in which of these points of view these affections ought to be regarded, an examination into their nature, and an inquiry into the opinions of preceding writers respecting them, seem necessary to be attempted.

It is necessary that the peculiar nature of this tremulous motion should be ascertained, as well for the sake of giving to it its proper designation, as for assisting in forming probable conjectures, as to the nature of the malady, which it helps to characterise. Tremors were distinguished by Juncker into Active, those proceeding from sudden affection of the minds, as terror, anger, &c. and Passive, dependant on debilitating causes, such as advanced age, palsy, &c. But a much more satisfactory and useful distinction is made by Sylvius de la Bo? into those tremors which are produced by attempts at voluntary motion, and those which occur whilst the body is at rest. Sauvages distinguishes the latter of these species by observing, that the tremulous parts leap, and as it were vibrate, even when supported: whilst every other tremor, he observes, ceases, when the voluntary exertion for moving the limb stops, or the part is supported, but returns when we will the limb to move; whence, he says, tremor is distinguished from every other kind of spasm.

A small degree of attention will be sufficient to perceive, that Sauvages, by this just distinction, actually separates this kind of tremulous motion, and which is the kind peculiar to this disease, from the Genus Tremor. In doing this he is fully warranted by the observations of Galen on the same subject, as noticed by Van Swieten. "Binas has tremoris species Galenus subtiliter distinxit, atque etiam diversis nominibus insignivit, tremor enim facultatis corpus moventis et vehentis infirmitate oboritur. Quippe nemo, qui artus movere non instituerit tremet. Palpitantes autem partes, etiam in quiete fuerint, etiamsi nullum illis motum induxeris palpitant. Ideo primam modo descriptam tremoris speciem, quando quiescenti homini involuntariis illis et alternis motibus agitantur membra, palpitationem dixit, posteriorem vero, quae non fit nisi homo conetur partes quasdam movere tremorem vocavit."

Under this authority the term palpitation may be employed to mark those morbid motions which chiefly characterise this disease, notwithstanding that this term has been anticipated by Sauvages, as characteristic of another species of tremor. The separation of palpitation of the limbs from tremor, is the more necessary to be insisted on, since the distinction may assist in leading to a knowledge of the seat of the disease. It is also necessary to bear in mind, that this affection is distinguishable from tremor, by the agitation, in the former, occurring whilst the affected part is supported and unemployed, and being even checked by the adoption of voluntary motion; whilst in the latter, the tremor is induced immediately on bringing the parts into action. Thus an artist, afflicted with the malady here treated of, whilst his hand and arm is palpitating strongly, will seize his pencil, and the motions will be suspended, allowing him to use it for a short period; but in tremor, if the hand be quite free from the affection, should the pen or pencil be taken up, the trembling immediately commences.

This affection, which observation seems to authorise the being considered as a symptom peculiar to this disease, has been mentioned by few nosologists: it appears to have been first noticed by Gaubius, who says, "Cases occur in which the muscles duly excited into action by the impulse of the will, do then, with an unbidden agility, and with an impetus not to be repressed, accelerate their motion, and run before the unwilling mind. It is a frequent fault of the muscles belonging to speech, nor yet of these alone: I have seen one, who was able to run, but not to walk."

Sauvages, referring to this symptom, says, another disease which has been very rarely seen by authors, appears to be referable to the same genus ; which, he says, "I think cannot be more fitly named than hastening or hurrying Scelotyrbe ."

SHAKING PALSY DISTINGUISHED FROM OTHER DISEASES WITH WHICH IT MAY BE CONFOUNDED.

Treating of a disease resulting from an assemblage of symptoms, some of which do not appear to have yet engaged the general notice of the profession, particular care is required whilst endeavouring to mark its diagnostic characters. It is sufficient, in general, to point out the characteristic differences which are observable between diseases in some respects resembling each other. But in this case more is required: it is necessary to show that it is a disease which does not accord with any which are marked in the systematic arrangements of nosologists; and that the name by which it is here distinguished has been hitherto vaguely applied to diseases very different from each other, as well as from that to which it is now appropriated.

Palsy, either consequent to compression of the brain, or dependent on partial exhaustion of the energy of that organ, may, when the palsied limbs become affected with tremulous motions, be confounded with this disease. In those cases the abolition or diminution of voluntary muscular action takes place suddenly, the sense of feeling being sometimes also impaired. But in this disease, the diminution of the influence of the will on the muscles comes on with extreme slowness, is always accompanied, and even preceded, by agitations of the affected parts, and never by a lessened sense of feeling. The dictates of the will are even, in the last stages of the disease, conveyed to the muscles; and the muscles act on this impulse, but their actions are perverted.

Anomalous cases of convulsive affections have been designated by the term Shaking Palsy: a term which appears to be improperly applied to these cases, independent of the want of accordance between them and that disease which has been here denominated Shaking Palsy. Dr. Kirkland, in his commentary on Apoplectic and Paralytic Affections, &c. cites the following case, related by Dr. Charlton, as belonging, he says, to the class of Shaking Palsies. "Mary Ford, of a sanguineous and robust constitution, had an involuntary motion of her right arm, occasioned by a fright, which first brought on convulsion fits, and most excruciating pain in the stomach, which vanished on a sudden, and her right arm was instantaneously flung into an involuntary and perpetual motion, like the swing of a pendulum, raising the hand, at every vibration higher than her head; but if by any means whatever it was stopped; the pain in her stomach came on again, and convulsion fits were the certain consequence, which went off when the vibration of her hand returned."

Another case, which the Doctor designates as 'A Shaking Palsy,' apparently from worms, he describes thus, "A poor boy, about twelve or thirteen years of age, was seized with a Shaking Palsy. His legs became useless, and together with his head and hands, were in continual agitation; after many weeks trial of various remedies, my assistance was desired.

Whether these cases should be classed under Shaking Palsy or not, is necessary to be here determined; since, if they are properly ranked, the cases which have been described in the preceding pages, differ so much from them as certainly to oppose their being classed together: and the disease, which is the subject of these pages, cannot be considered as the same with Shaking Palsy, as characterised by those cases.

The latter appears to be referable to that class of proteal forms of disease, generated by a disordered state of primae viae, sympathetically affecting the nervous influence in a distant part of the body.

Before making the attempt to point out the nature and cause of this disease, it is necessary to plead, that it is made under very unfavourable circumstances. Unaided by previous inquiries immediately directed to this disease, and not having had the advantage, in a single case, of that light which anatomical examination yields, opinions and not facts can only be offered. Conjecture founded on analogy, and an attentive consideration of the peculiar symptoms of the disease, have been the only guides that could be obtained for this research, the result of which is, as it ought to be, offered with hesitation.

Uncertainty existing as to the nature of the proximate cause of this disease, its remote causes must necessarily be referred to with indecision. Assuming however the state just mentioned as the proximate cause, it may be concluded that this may be the result of injuries of the medulla itself, or of the theca helping to form the canal in which it is inclosed.

The great degree of mobility in that portion of the spine which is formed by the superior cervical vertebrae, must render it, and the contained parts, liable to injury from sudden distortions. Hence therefore may proceed inflammation of quicker or of slower progress, disease of the vertebrae, derangement of structure in the medulla, or in its membranes, thickening or even ulceration of the theca, effusion of fluids, &c.

But in no case which has been noticed, has the patient recollected receiving any injury of this kind, or any fixed pain in early life in these parts, which might have led to the opinion that the foundation for this malady had been thus laid. On the subject indeed of remote causes, no satisfactory accounts has yet been obtained from any of the sufferers. Whilst one has attributed this affliction to indulgence in spirituous liquors, and another to long lying on the damp ground; the others have been unable to suggest any circumstance whatever, which, in their opinion, could be considered as having given origin, or disposed, to the calamity under which they suffered.

Cases illustrative of the nature and cause of this malady are very rare. In the following case symptoms very similar are observable, so far as affecting the lower extremities. That the medulla spinalis was here affected, and in its lower part, is not to be doubted: but this, unfortunately, was never ascertained by examination. It must be however remarked, that this case differed from those which have been given of this disease, in the suddenness with which the symptoms appeared.

It must be acknowledged, that in the well-known cases, described by Mr. Potts, of that kind of Palsy of the lower limbs which is frequently found to accompany a curvature of the spine, and in which a carious state of the vertebrae is found to exist, no instructive analogy is discoverable; slight convulsive motions may indeed happen in the disease proceeding from curvature of the spine; but palpitating motions of the limbs, such as belong to the disease here described, do not appear to have been hitherto noticed.

This gentleman died about four years after the accident, when the body was examined by Dr. Bellett and Mons. Sorbier, who made the following report:

"We first examined the muscles of the tongue, which were found extenuated and of a loose texture. We observed no signs of compression in the lingual and brachial nerves, as high as their exit from the basis of the cranium and the vertebrae of the neck; but they appeared to us more compact than they commonly are, being nearly tendinous. The dura mater was in a sound state, but the pia mater was full of blood and lymph; on it several hydatids, and towards the falx some marks of suppuration were observed. The ventricles were filled with water, and the plexus choroides was considerably enlarged, and stuffed with grumous blood. The cortical surface of the brain appeared much browner than usual, but neither the medullary part nor cerebellum were impaired. We chiefly took notice of the Medulla Oblongata, this was greatly enlarged, surpassing the usual size by more than one third. It was likewise more compact. The membranes, which, in their continuation, inclose the spinal marrow, were so tough that we found great difficulty in cutting through them, and we observed this to be the cause of the tendinous texture of the cervical nerves. The marrow itself had acquired such solidity as to elude the pressure of our fingers, it resisted as a callous body, and could not be bruised. This hardness was observed all along the vertebrae of the neck, but lessened by degrees, and was not near so considerable in the vertebrae of the thorax. Though the patient was but nine and thirty years old, the cartilages of the sternum were ossified, and required as much labour to cut them asunder as the ribs; like these they were spungy, but somewhat whiter. The lungs and heart were sound. At the bottom of the stomach appeared an inflammation, which increased as it extended to the intestines. The ileum looked of that dark and livid hue, which is observed in membranous parts tending to mortification. The colon was not above an inch in diameter, the rectum was smaller still, but both appeared sound.--From these appearances, we were at no loss to fix the cause of this gradual palsy in the alteration of the medulla spinalis and oblongata."

Dr. Bellett offers the following explanation of these changes. "I conceive, that, by this accident, the head being violently bent to the right, the nervous membranes on the left were excessively stretched and irritated; that this cause extended by degrees to the spinal marrow, which being thereby compressed, brought on the paralytic symptoms, not only of the left arm, but at last in some measure also of the right. This induration seems to have been occasioned by the constant afflux of the nutritive juices, which were stopt at that place, and deprived of their most liquid parts; the grosser ones being unable to spread in the boney cavity, by which they were confined, could only acquire a greater solidity, and change a soft body into a hard and nearly osseous mass. This likewise accounts for the increase of the medulla oblongata, which being loaded with more juices than it could send off, swelled in the same manner as the branches of trees, which will grow of a monstrous size, when the sap that runs into them is stopt in its progress. The medulla oblongata not growing so hard as the spinalis, was doubtless owing to its not being confined in an osseous theca, but surrounded with soft parts, which allowed it room to spread. The obstruction from the bulk of this substance must have affected the brain, and probably induced the thickening of the pia mater, the hydatids, and the beginning of suppuration, whereas the dura mater, being of a harder texture, was not injured."

In some of the symptoms which appeared in this case, an agreement is observable between it and those cases which are mentioned in the beginning of these pages. The weakened state of both arms; the power first lessening in one arm, and then in a similar manner in the other arm; the affection of the speech; the difficulty in chewing and in swallowing; as well as of retaining, or freely discharging, the spittle; the convulsive motions of the body; and the unimpaired state of the intellects; constitute such a degree of accordance as, although it may not mark an identity of disease, serves at least to show that nearly the same parts were the seat of the disease in both instances. Thus we attain something like confirmation of the supposed proximate cause, and of one of the assumed occasional causes.

Whilst conjecturing as to the cause of this disease, the following collected observations on the effects of injury to the medulla spinalis, by Sir Everard Home, become particularly deserving of attention. It thence appears, that none of the characteristic symptoms of this malady are produced by compression, laceration, or complete division of the medulla spinalis.

"Pressure upon the medulla spinalis of the neck, by coagulated blood, produced paralytic affections of the arms and legs; all the functions of the internal organs were carried on for thirty-five days, but the urine and stools passed involuntarily.

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