Infantile paralysis, and its attendant deformities

Front matter

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{UNITED STATES OF AMElUCA.il

Preface

Notwithstanding the very great progress made of late years in medicine and surgery, there are still many forms of disease which have not yet received the careful study they require, and about which there is so little to be found in medical literature that the majority of physicians are practically excluded from profiting by the experience of others.

A hundred cases of Infantile Paralysis occurring in the practice of as many different physicians, in different localities, without knowledge of each other, and perhaps each the only case of a lifetime practice, afford very imperfect opportunity for that careful, comparative observation which positive science requires, Hence the meagreness of the literature on this subject. Only when some enthusiastic "specialist" calls them from their hiding-places — few in a given locality, but many in community — is there afforded sufficient opportunity for that consecutive and multiplied observation necessary for establishing facts and making deductions. As to the facts set forth in this book, there is scarcely chance for mistake ; if the deductions are erroneous,

IV PREFACE.

some keener mind will detect the error ; but, believing them to be true, the profession have a right to them. I only claim a faithful use of opportunity, and a careful, honest observation of facts.

I wish to express my indebtedness to F. O. Earle M.D., of Chicago, 111., lately assistant in this Institution for the accurate drawings illustrating the text.

If these pages are true, there should be, ten years hence, a smaller proportion of cripples than now distress our sight ; and that such may be the case is the fervent hope of

The Author.

N. Y. Orthopedic Institution, 1303 Broadway, Sept., 1866

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INFANTILE PARALYSIS

ATTENDANT DEFORMITIES.

STATEMENT.

Paralysis in early life is either the direct or remote cause of a large proportion of the distortions of the upper and lower extremities which afflict a certain number in every community.

Perhaps it is not always sufficiently realized that children of a tender age are as much more liable to loss of muscular power than persons of mature age, as they are to any other form of sickness. The young of every kind are always most susceptible and possess least power of endurance, and when not well guarded, suffer most from adverse influences. And this is especially true with regard to t> INFANTILE PARALYSIS, AND

affections of the nervous system. Hence there is sufficient reason for the fact, that paralysis is of common occurrence in early life.

DEFINITION.

By the term ''Infantile Paralysis" I do not mean simply a paralysis occurring in infancy or childhood ; but I desire to restrict the use of the term — and it is so used in this paper — to indicate that form of paralysis which occurs only in infancy. Or if the same form of paralysis be found to occur after maturity, then the term should indicate that which occurs principally and characteristically in children. In fact, as will be hereafter more fully understood, I use the term to express a paralysis peculiar to the growing period. Perhaps, more correctly, it might be called the paralysis of nutrition and development.

FREQUENCY.

There seems to be no doubt that this disease is much more frequent now, and in this country, than > formerly, and is rapidly increasing. Nor is it difficult to account for this state of tilings.

PROBABLE CAUSE.

Modern, and especially American, civilization is characterized by peculiar activity of the brain, and this is often carried to great excess.

The motive-force of American progress is brain-power. It is the ceaseless activity of directing mind that, in two centuries, has subdued the wilderness and peopled the continent ; that has built vast cities whose commerce reaches the remotest regions of the globe ; and that has proved itself capable of solving the most difficult political problems. The creative energy of the distinctively American intellect is recognized everywhere. But such vast results of this creative intelligence have not been accomplished without some sacrifices. It has diminished our physical endurance. As a people, we are dyspeptic, and weak in bodily vigor in the inverse ratio

8 INFANTILE PAEALYSIS, AND

of over-activity of brain. Our laborers have to be imported. We are predisposed to nervous derangements. As a people, we are over-worked. The nervous system becomes exhausted, and a constitution less strong than our own, but more excitable and impressible, is transmitted to our children.

WHY VIGOROUS PARENTS PRODUCE SICKLY OFFSPRING.

It is often asked, M How is it that, when both parents are well and vigorous, the children are often puny and nervous Y3 The reply must be, that the offspring partake of the parents' condition at the time they were hegotten. A man who is thoroughly engrossed in business calculations and cares, or even in the pleasures of society, will beget children with physical powers correspondingly subordinated to the nervous. To insure a perfect plant, it is not only requisite that the seed be produced by a strong and vigorous tree, but no drought must have absorbed its sap or untimely frosts have chilled it while the seed was maturing. We have imperfect grapes if an accidental cut lets the juice of the vine leak out.

How, then, can even naturally vigorous persons expect to bear children of equal vigor if begotten while they themselves are exhausted with intense mental labors and excitements? It is a physiological impossibility. Hence our children are born with a surprising degree of nervous irritability ; just the temperament for the production of infantile paralysis when favorable circumstances combine to produce it.

MOST COMMON AMONG THE RICH.

And, as may be supposed, this disease occurs most frequently in the families of active business men. Indeed, while strumous diseases abound in the lower classes, I have seen but very few cases of infantile paralysis among them. My whole experience has led me to regard infantile paralysis as being almost confined to the families of active, intelligent men, as above indicated.

ITS PATHOLOGY.

The definite pathology of infantile paralysis is not well understood. I have no theory to offer, and will only assume to present the facts connected with these cases which a pretty extensive, close, and prolonged observation of them may justify me in giving, with a considerable degree of explicitness.

SYMPTOMATOLOGY.

In many cases the paralysis is ushered in with symptoms apparently, in all respects, resembling inflammation of the membranes of the spinal cord. There may be every justification in applying the term spinal or cerebro-spinal meningitis to those cases. But there are many even among these severer cases, where the disturbance follows, rather than precedes, the paralysis, in such a relation as to suggest the inquiry, if the disturbance might not possibly have been the effect, rather than the cause of it? So great a shock as the paralyzing of a portion of the body might well be expected to produce a profound impression and serious disturbance.

CONSTITUTIONAL SYMPTOMS SOMETIMES TRIFLING.

There are other cases, however, in which the constitutional symptoms are so trifling, as to tax our minds to account for them in connection with so serious and dangerous a disease as inflammation of the spinal cord or its membranes. Is it possible for distinctive inflammation to be going on in the brain or spinal cord, and the patient exhibit no symptoms which might point to it? And yet there are many cases of infantile paralysis in which there is no noticeable variation from ordinary health, the only symptom to be noticed at the very time, being the paralysis itself.

CaseI. — K. G., at eleven months old, had learned to walk. Suddenly it was observed that she was unable longer to do so, and it was six or eight months later before she regained imperfect locomotive power. The right leg was partially paralyzed ; but there was no sickness or other indication of the cause, or even the time of its occurrence. At thirteen I operated on her for partial club-foot.

Case 2. — M. D., though a delicate, nervous child, had no special sickness before or after the paralysis. One day, when two years old, she started to walk across the floor, but became paralyzed when she got to the middle of the room. The paralysis is in the right leg — not complete, though attended with partial arrest of development.

Several other cases of paralysis without constitutional symptoms have come under my observation, though they embrace but a small proportion of the whole. But cases in which the symptoms are very mild are quite common.

In some instances the child is put to bed apparently well, and is found paralyzed in the morning, but without any indication of the time in the night when the paralysis happened, nor was the paralysis followed by any unusual bodily disturbance.

GENERAL HISTORY.

The majority of those cases, however, have about this history : the child is getting its first molar teeth — for the greatest number of cases occur when the child is from fourteen to twenty months old. Sometimes there has been a diarrhea, or other drain upon the S}7stem, or cause of irritation, accompanied with slight fever, though not often enough to keep the child abed. In a few days these symptoms abate and the child appears better ; but on making efforts to walk or use his limbs, it is found that he has lost the power to do so. In most cases, the exact time when the paralysis happened is never ascertained. -

H INFANTILE PARALYSIS, AND

SELDOM UNCONSCIOUS.

It is seldom that the accession of this form of paralysis is accompanied with unconsciousness. In one case, the child, in apparently perfect health, was sleeping quietly in its cradle, watched fondly by its father. It awoke with a scream, and was found to be completely paralyzed in its entire body. But neither then, nor at any subsequent time, was there any unconsciousness. It is true that there are occasional cases of convulsions followed by infantile paralysis,* but there are more cases of convulsions which are not followed by paralysis, and a careful study, when they do exist together, has failed to establish to my mind any direct connection between them. Any sufficient irritation may produce infantile convulsions ; but they do not seem to be caused by or to cause the paralysis, though the paralysis may happen at about the

° Infantile apoplexy, with convulsions and paralysis, is quite different from that which we are now considering.

same time. As a general rule, this paralysis in young children is accompanied with much less severe symptoms (though this is not always the case) than when the patient is older. With increase of years, the accompanying symptoms in general become more pronounced, and are neither so sudden in their accession, nor do they pass so speedily away. A child from ten to fourteen years old may be sick for several months with a more gradual and less uniform accession of paralysis. This is often accompanied with severe symptoms of what is generally called spinal or cerebro-spinal meningitis.

PECULIARITY OF INFANTILE PARALYSIS.

But the form, rather than the origin, of this paralysis is to engage our study.

I wish to call attention to the idea before alluded to, viz., that there is a form of paralysis peculiar to the growing period; that it is most likely to occur at the times of greatest organic or vegetative activity; and that it ceases to act after the organs have attained complete maturity. The periods of life during which it is most liable to occur are from six months to four years of age, and again from ten to fourteen. This form of childhood-paralysis has peculiarities not found in other forms, whether these other forms occur in children or adults. Children as w^ell as adults may have apoplexy, softening, inflammations, blows on the head, etc. ; but the resulting paralysis is always essentially different from that which we are now discussing.

NO EVIDENCE OF CENTRAL LESION.

One essential feature of infantile paralysis is. that there is uniformly perfect recovery, so far as we can discover, of whatever disease of the nervous center — if it is ever really caused by a local lesion — which may have caused it.

THE DISEASE PERIPHERIC.

Another essential characteristic of this paralysis is the entire absence of reflex irritability of the muscles, accompanied with a remarkable diminution of capillary circulation in the affected parts, and a correspondingly diminished muscular power. These, and the arrested growth of certain parts, are the only remaining evidence of previous disease. If we can conceive of a general constringing of the capillary vessels, and a shutting off of the capillary circulation, with such a physical condition as would result if the limb had for a long time been tightly bandaged, we should gain a very good idea of the actual condition in infantile paralysis. In fact, my own belief is that the disease is essentially peripheric, and that the great nervous centers are only indirectly and secondarily implicated. On no other hypothesis can I account for all the phenomena of these cases. But whether this suggestion be the true solution or not, the essential condition in infantile paralysis is a cutting off of the capillary circulation and a correspondingloss of muscular irritability ; the latter to a great extent, if not wholly, due to this deprivation of blood in the small vessels. And the limb withers as does a plant when the channels to its irrigation are dried up. It is a well-known physiological fact, that the function of any organ is diminished or destroyed by cutting off its supply of blood, and a muscle can no more act without its supply of blood than it can act without the nervous stimulus. In other words, you may paralyze a muscle as completely by stopping its circulation as by dividing its nerve. And it appears to me that the only satisfactory explanation of all the facts in these cases, is to suppose that the paralysis is nearly, if not wholly, due to the diminished capillary circulation rather than, as in other forms of paralysis, to the direct influence of a lesion in the brain or spinal cord. By what precise pathological condition this withdrawing of a large part of the sanguineous fluid is brought about, I have no theory to suggest. There is no doubt as to the fact, however.

TENDENCY TO RECOVERY.

There is another fact of immense importance, and that is, that these cases all tend toward recovery. Very few cases are to be met with where some portions of the originally paralyzed members have not entirely recovered. Of course, there are cases so bad that the sufferer must always remain without hope of essential improvement. Still, the large majority of all the cases I have seen had more than twice the paralysis when it occurred, or when it was first discovered, than they had at the end of the first year. One arm or leg may be paralyzed, but no other portion of the body affected. But more frequently the patient who comes to you a year after the attack, writh a single paralyzed arm or leg, was at first paralyzed in both legs or over the whole trunk and extremities, but has recovered except as to some single member. If the recovery is complete, it generally takes place within a few weeks, or at furthest a few months. And it is a curious circumstance, that this spontaneous recovery of a portion of the original paralysis is as much more complete in certain members as it is more imperfect in the remaining ones. That is, in cases of paraplegia for instance, if there is very great improvement or complete recovery in one leg, the other will improve but very little ; while, if both legs improve alike, the result will be more general power than in the worse leg, and less power than in the better leg in the first case ; as though nature possessed only reserve force enough for the complete recovery of one member, or a less relative improvement if this force be divided between the two.

But nature, immediately after such a shock, is seldom capable of repairing the whole damage which has been inflicted. She does with comparative rapidity what she can, and would often do much more if permitted. Many a theory has been triumphantly established by happening to be tried at a time when nature is sure to be using her reserve force to repair damages, and always with more or less success. If the apostles of "electricity," "movements," 11 strychnia," the "ice bag," etc., have no better data than partial recovery in a few cases within the period of first recuperative effort, their claims rest on a very unsatisfactory basis. I have said in substance, that there is a period of reaction during which time recuperative effort is carried forward with complete, or at least partial success. The partial recovery may be either as to the amount in all affected members, or complete in a portion, while recuperation has but slightly advanced in others apparently no worse originally than the restored portions. But why does the reparative process stop half way ? Evidently because nature may have exhausted her reserve force. But why should this reparative effort stop so entirely ? Why should we not expect, in accordance with the analogy of all recuperative processes, a continuous, though retarded improvement ? While the fact is, that after a certain time improvement is very feebly manifested and very slight in nearly all cases, while in many the condition of the remaining paralyzed limbs actually grows worse as time passes ; averaging perhaps one case with another, a total cessation of improvement after the first few months. But I think it can be very clearly established, that when patients do not continue to improve beyond a certain point, or when the improvement is so feeble as only to be recognized from year to year, as well as when there is no longer any improvement to be seen, that it is not always because nature has wholly exhausted herself, but because accidental circumstances have come in to interfere with her work, which would otherwise have gone on much further.

" CONTRACTIONS."

It is noticed that what are called " contractions" of various muscles, accompanied with Mieir inevitable results, distortions of joints — begin to appear early in the history of the case. And these so-called contractions go on till the distortions become permanent deformities. When the contractions have proceeded to a certain extent, all increase of muscular power ceases, and the case is generally considered fit only for the orthopaedic surgeon. The question arises, " What is the cause of these contractions ?" They occur with a preponderance of certain forms ; that is, talapes varus is more common than talapes valgus, and contraction of the hamstring muscles are more common than relaxation of the same ; but there is the absence of all uniformity or law in reference to it. Certain contractions are most frequent simply because circumstances more frequently favor them than others, and not at all because the disease itself predisposes particular muscles to contract. I have used, and shall continue to use, the word " contractions" in the ordinary way, because the term sufficiently conveys the idea of shortened muscles ; but I do not by its use intend to accept the common idea of active contractions of the muscles, in which sense it is generally used.

NO REFLEX CONTRACTION.

It is just here where this form of paralysis differs so radically from all others — from those forms where there is a known lesion in the nerve- center. When there is a rupture and clot in, or inflammation of, the brain or spinal cord or their meninges, the paralysis is always accompanied by reflex movements of the muscles, rigidity, spasm, clonic spasm, pain, numbness, and other indications of disturbance of innervation. But in the form of paralysis under consideration — except, possibly, during its incipiency — there is a total absence of the least variation of innervation ; no difference in the quality or amount of muscular force is ever to be noticed from one day to another, or varying with physical or physiological disturbances. In a word, Infantile Paralysis is peculiar, in that it is characterized by unvarying want of irritability , and this want of irritability is probably the cause of, as well as the extent of, the paralysis. What it is that has deprived the muscles of their irritability, or has deprived the nerves of their power of imparting their stimulus to the muscles ; whether the origin was in the nervous centers or in the nervous periphery where the only evidence exists of anything wrong — are questions regarding which I am not prepared to express an opinion. My only object at present is to direct attention to the fact of the unvarying absence of irritability in the muscles themselves, and the want of the ordinary evidence of local disease anywhere in the brain or spinal cord, as the essential characteristic features of this form of paralysis.

SHORTENING OF CERTAIN MUSCLES.

11 What, then, causes the muscular contractions, and consequent deformities, which so regularly follow as a seeming necessary consequence of infantile paralysis ?" I reply, without hesitation, that contractions and distortions are not necessary consequences of this paralysis ; and, moreover, that these unhappy results are always and entirely preventable. Indeed, what seem to be contractions of certain muscles — generally the flexors — are not contractions at all, but simply a mechanical shortening of muscles when their attachments are for a length of time brought nearer together. Furthermore, this tendency of muscles to adapt their length to the position in which they are for a time placed, is found to be much less in these cases than where there has been no paralysis at all. And, as might be supposed, the greater the paralysis, the less the shortening of flexor and other habitually relaxed muscles ; and, on the other hand, the less the paralysis, the more readily do these muscles assume a permanently shortened condition when the limb is kept for a time in such an attitude that the ends of the muscles approach each other ; and when muscles are not paralyzed, they become shortened or elongated according to the accidental position of the limb much more readily than when paralyzed. In case of hip-joint disease, for instance, a very short time will often suffice to cause certain muscles to become so much shortened as to require tenotomy. But in infantile paralysis, the shortening of certain muscles is not the first or principal damage done by improper and careless positions of the paralyzed limbs.

LENGTHENING OF MUSCLES.

The shortening, though the most noticeable, is not the first or worst complication which arises to arrest the progress of improvement and to set in train a series of conditions favoring the formation of distortions and deformities. Any position of a limb which allows the extensor muscles to become shortened must inflict a worse damage on the flexors by keeping them extended till they lose their remaining irritability and become degenerated. Now, we have another fact connected with these cases when they have arrived at the stage of deformity, viz., this: the difficulty of treatment consists much less in relaxing the shortened muscles than in giving tone and strength to their antagonists — the lengthened and weakened ones. Indeed, it is this, in the destruction of all remaining muscular irritability, and in many instances the destruction and entire loss of the substance itself of the expanded muscle, which constitutes the principal anxiety in treating this class of deformities. As this important consideration has been many times neglected, if not entirely lost sight of, let us consider the effect of simple extension upon the power and functions of muscular tissue in its healthy state.

PROPOSITIONS.

In reference to this, the following are believed to be true, viz. :

1st. To retain a healthy muscle in an expanded state for a certain length of time is to diminish or destroy its irritability and contractile force.

ITS ATTEMDANT DEFORMITIES. 29

2d. To extend a muscle while in the act of contracting, that is, to overcome it, is to, at once, destroy its irritability and force.

A moment's reflection will call up ample ^ illustrations of the correctness of the foregoing ' statements.

1. — A few months ago, Dr. Wm. H. Van Buren read a paper before the New York Academy of Medicine, on the successful treatment of spasm of the sphincter ani, by means of this principle of overcoming muscular action. His treatment for this most distressing disease is as efficacious as it is simple and speedy. By inserting the thumbs within the sphincter and placing the fingers on each haunch as fulcrums, by a powerful effort he stretches the hypertrophied muscle to the fullest extent between the ischii. The irritability and hypertonicity of the sphincter is destroyed and the cure is thus effected. I have seen several cases of relaxed muscles, and one case of real club-foot, which had been caused by a violent extension of muscles while in the act of contracting, which had from that moment caused paralysis of those muscles.

THE " INDIA-RUBBER MAN."

* " There is a curious illustration of the effect of extension on the ligaments and muscles now in this city. At one of the minor theaters there is exhibiting what is called the "india-rubber man" — an individual whose business it is to contort himself in the most unheard-of and inconceivable manner — dislocating his joints, laying his leg up along the spinal column, and otherwise defying ordinary anatomical laws. How did he attain this remarkable flexibility of joints and relaxation of muscles ? Muscles and ligaments are for the purpose of holding the frame together, rather than of facilitating its separation. I have been informed that every day this man subjects himself to a process of continuous

* From my paper on " Tenotomy," in the N. Y. State Medical Society's Transactions for 1865. • stretching of the muscles. A heavy weight is tied to the foot, which he holds pendent, slowly swinging in the air. The same is done to the arms and the whole body. The result is an almost complete destruction of the tonicity of the muscles and a corresponding relaxation of all the tissues. The muscular power is so overcome, and he is so nearly paralyzed, that he can hardly walk ; he is wholly incapable of a firm embrace ; the spinal column can be made to reverse the natural curves, and, altogether, he is an illustration on a large scale of what must and does occur in a single muscle or group of muscles when subjected to continued or violent traction or extension. The only possible results, loss of irritability and degeneration, are sure to follow. It might be inferred, and such is the fact, that the effect of extension is greater in the paralyzed muscle than in the healthy tissue, and that a slight degree of continuous or comparatively feeble sudden extension, which might be harmless in healthy muscles, may be capable of destroying altogether the feeble irritability and power of a recently paralyzed muscle.

1 ' The fact that continuous extension has the power of diminishing, and ultimately destroying, the contractility of muscles, may be explained without supposing rupture of the fiber-cells, when we remember that the course of the muscular fibrillae is not straight but zigzag, and that in contraction, this eccentricity is increased, the fibers being thrown up into irregular zigzags. In prolonged extension, these eccentrics may be destroyed and the spaces closed up by the presence and pressure of other straightenedout fibers. Instead of a wavy mass, the fibers may approximate straight lines with a loss of some of the most essential features of muscular tissue and a corresponding loss of contractility.

"It is no doubt already correctly inferred, that I place much more importance on the loss of power in certain muscles as explained above (a relative loss of power beyond the primary effect of the original paralysis), than on the shortened condition of certain other muscles — their antagonists — which is usually referred to as the cause of these deformities."

DIFFERENT DEGREES OF PARALYSIS IN DIFFERENT MUSCLES.

Nothing is more noticeable than the varying degrees of paralysis in different extremities of the same individual, and indeed in the same limb. It is true of these cases, that there may be complete loss of contractility in muscles receiving their nervous supply from the same nerves, and even in contact with other muscles evincing considerable muscular power. There is no other explanation of this apparent defiance of all known physiological laws than the one I have given. But as a matter of fact, these variations — this relative losing or gaining of force — correspond exactly in all cases with the position of the muscles,

whether they have been kept in extension or

2* relaxation ; for muscles allowed to relax — all other things being equal — gain as much as the extended ones lose. The muscles kept in a shortened position, recover irritability more readily than if kept extended to their natural length ; but having no extension, become in time inelastic and tendonous ; while those kept unnaturally extended, lose their irritability and become thin and degenerated.

And having thus destroyed the harmonious action of the muscles, as well as their length and mechanical adaptation, we have the first elements of a deformity.

POSITION THE SOLE CAUSE OF SHORTENING AND RELAXATION OF MUSCLES.

Having shown that position of the limbs alone is sufficient to cause deformity even in the healthy subject, it only remains to inquire, "Is this actually the case in infantile paralysis ?" The reply must be affirmative.