Miss N. D——, æt. 15, paresis in both legs, first at age of nine, increased at age of twelve, when weakness of vision first noted. At fourteen both feet in rigid pes equinus, and both tendons achilleis cut, without benefit. Hands became tremulous, without paresis. On examination at age of fifteen found moderate atrophy of muscles of both legs. Tendo Achillis united on both sides, and equinus persists. Voluntary movement exists, both in anterior tibial and in gastrocnemius muscles, but diminished in anterior tibial. Faradic contractility diminished in both sets of muscles; examination difficult from extreme sensibility of patient. In both hands interossei, muscles of thumb, and little finger show tremors and fibrillary contractions. Thenar eminences small, abductor pollicis nearly absent, not reacting to faradic current. Optic nerves slightly atrophied. Mind enfeebled, memory poor; articulation not affected. Five years later the motor paralysis and mental enfeeblement had still further progressed, but no exact notes exist of this period.
139Loc. cit.(ed. 1877).
Erb140relates a case that he considers unique at the time in a girl of six. The paralysis began insidiously in the right foot in July; a fortnight later had extended to the left foot; complete motor paralysis existed in August, without any lesion of sensibility: after electrical treatment, then instituted, first return to motility to peroneal muscles in November; by January child able to walk again and electrical reactions nearly normal.141
140Brain, 1883.
141In the same number ofBrain, A. Hughes Bennett quotes cases of so-called chronic paralysis in very young children which are evidently cases of general paresis from congenital cerebral atrophy. The children were defective in intelligence, could not sit up nor hold up the head; the electrical reactions were preserved. I have seen a great many such cases: they are indeed not at all uncommon. Much more so is Bennett's diagnosis.
COMPLICATION WITHPROGRESSIVEMUSCULARATROPHY.—Raymond142and Seeligmüller describe some rare cases where progressive muscular atrophy declared itself in persons previously affected with infantile paralysis in other limbs. Both observers infer a gradual and chronic extension along the cord of the originally acute anterior poliomyelitis.143Similar cases have much more recently (1884) been quoted by Ballet as tending to modify the prognosis which has usually been pronounced favorable quoad life and further spinal accidents. (See infra.)
142Gaz. méd., 1875. No. 17.
143It seems to me that Seguin's case, above quoted, might be an example of such complication(?). But I have not seen the patient myself, and describe the case according to the views of the author.
PROGNOSIS.—The prognosis of atrophic paralysis, quoad vitam, is, as is well known, extremely good. The prospect of recovery from the paralysis is variable. It cannot be estimated either by the extent of the initial paralysis or by the severity of the fever or attendant nervous symptoms. The electrical reactions alone are of value in the prognosis, and their value is very great. Duchenne first formulated their law: “All the cases of infantile paralysis which I have seen where the faradic contractility was diminished but not lost, and which could be treated by faradic electricity within two years after the onset of the paralysis, have completely recovered.”144This encouraging statement must be read as applying rather to individual muscles than to cases as a whole. Fewcomplete recoveries of patients are claimed even by so enthusiastic an electrician as Duchenne; who nevertheless affirms his not unfrequent success in re-creating entire muscles out of a few fibres saved from degeneration.
144Loc. cit.
The persistence of galvanic irritability in muscles which fail to contract to the faradic current has been shown by Erb to belong to the degenerative reactions. Hammond, however, without alluding to the qualitative changes in the galvanic contractions, sees in them the elements of a relatively favorable prognosis, even when faradic contractility is lost. Thus, out of 87 cases, in 39 of which the paralyzed muscles contracted to the galvanic but not the faradic current, 14 were entirely cured, 28 greatly improved, 30 slightly improved, 15 discontinued treatment very early.145
145Loc. cit., p. 482.
Examination of fragments of living muscle obtained by Duchenne's harpoon, though useful, should not be allowed to exaggerate an unfavorable prognosis. Much fat may be found in such fragments when the muscle is as yet by no means completely degenerated and can be made to contract to one or the other current. Erb, however, admits that the results of treatment have not, in his hands, been brilliant; but adds that he has had no opportunity to treat any cases which were not of long standing.146
146Loc. cit.
Volkmann147considers the paralysis entirely hopeless, and advises the concentration of all effort upon the prevention or palliation of deformities.
147Loc. cit.
It seems probable that at the present moment sufficient data do not exist for formulating a fair prognosis; nor will they until a much larger number of cases than hitherto have been submitted to all the resources of a complex and persevering system of therapeutics from the earliest period of the disease.
SPECIALPARALYSES.—Among the paralyses, some exercise a more unfavorable influence on locomotion than others. Thus, paralysis of the muscles of the trunk is more difficult to palliate, either by apparatus or by the efforts of the patient, than any paralysis of the limbs. Similarly, paralysis of the upper segments of a limb is more crippling than when confined to the lower. Partial paralysis of the muscles surrounding a joint is often (but not always) more liable to lead to deformity than total paralysis.
Influence of Neglect.—Apart from the influence of treatment in curing the paralysis, must be estimated in the prognosis the effect of care and watchfulness in limiting the disease and in averting many consequences, even of those which are incurable. The rescue of muscles only partially degenerated may often serve to compensate the inaction of those which are irretrievably ruined.
Ballet148has recently called attention to the fact that in certain cases persons who had been attacked with an anterior poliomyelitis in childhood became predisposed to different forms of spinal disease. Four have been observed: (1) transitory congestion of the cord, causing paralysis of a day or two's duration; (2) an acute spinal paralysis of the form usually seen in adults; (3) subacute spinal paralysis; (4) progressive muscular atrophy. The author relates cases under each of these heads, and further quotes onerelated by Dejerine in 1882.149The patient, a carpenter aged fifty-five and with an atrophic deformity of the foot, became suddenly paralyzed in the four limbs, trunk, and abdomen. The paralysis was complete in a month, was stationary for three months, then began to improve, and at the end of six months from the onset of the disease recovery was complete.
148Revue de Médecine, 1884.
149Revue de Médecine, 1882.
The observations of progressive muscular atrophy in persons bearing the stigmata of an infantile paralysis are quite numerous.150
150Charcot,Soc. Biol., 1875, andGaz. méd.;Seeligmüller (4 cases), inGerhardt's Handbuch, 1880; Hayem,Bull. Soc. de Biol., 1879; Vulpian,Clinique méd. de la Charité, 1879; Pitres, new observation, quoted by Ballet in 1884.
The prognosis cannot be the same for cases where everything is done to avert malpositions and for those where all precautions are neglected. Thus, prolonged rest in bed favors pes equinus; the use of crutches necessitates flexion of the thigh and forced extension of the foot; locomotion without support tends to displace articulations by superincumbent weight, causing pes calcaneus, genu-recurvatum. Finally, compensatory deformities must be averted from sound parts, as scoliosis from shortening of the atrophied leg, equinus from passive shortening of the gastrocnemii through flexion of the leg, etc.
ETIOLOGY.—Concerning the etiology proper of infantile paralysis little definite is known. It is probable, as has been already noticed, that traumatisms have a much more decided influence than is generally assigned to them. Leyden particularly insists on this influence, and on the facility with which a traumatism relatively severe for a young child may be overlooked, because it would not be recognized as such for an adult. It must be noticed, however, that children are much more liable to have the arms wrenched and pulled violently than the lower extremities; yet in a great majority of cases the lesion is situated in the lumbar cord.
It has been shown that the myelitis, though so limited transversely, is often far more diffused in the longitudinal axis of the cord than might be supposed from the permanent paralyses. This fact corresponds to the initial generalization of the motor disturbance. It seems possible that the traumatic irritation, starting from the central extremity of the insulted nerve, diffuses itself through the cord until it meets with its point of least resistance, and here excites a focal myelitis. That this point should most frequently be found in the lumbar cord would be explained by its relatively less elaborate development, corresponding to the imperfect growth and function of the lower extremities.
A second cause of anterior poliomyelitis is, almost certainly, the presence of some poison circulating in the blood. The frequent occurrence of the accident in the course of one of the exanthemata is one indication of this; other indications are found in such cases as that related by Simon, where three children in one family were suddenly attacked—two on one day, one, twenty-four hours later.151The same author relates a case of motor paralysis in an adult, followed by atrophy of left lower extremity, and which occurred during a fit of indigestion caused by eating mussels.152The acute ascending paralysis of Landry,with its absence of visible lesion, has been said to strikingly resemble the effects of poison. Hydrophobia and tetanus are again examples of the predilection exhibited by certain poisons for the motor regions of the cord.
151Journal de Thérap., 7, vii., 1880, p. 16. These children belonged to an American family, but were seen by several distinguished French physicians.
152P. 357.
The evidence that infectious diseases may constitute the immediate (apparent) causal antecedent of acute poliomyelitis has led, not unnaturally, to the theory that all cases of acute infantile paralysis are due to a specific infecting agent, some as yet unknown member of the great class of pathogenic bacteria. It may be noticed, however, that the occurrence of the spinal accidents after the ordinary infectious diseases, as scarlatina and measles, should as well indicate that a specific agent proper to itself was at least not essential to its development.153
153Perhaps the occurrence of diphtheria in the course of scarlatina and typhoid should indicate a similar lack of real specificity in the morbid agent of the former disease.
The influence of exposure to cold, which seems to have been sometimes demonstrated, must probably be interpreted, as in the case of rheumatism and pneumonia, as effective by means of some poison generated in the organism when cutaneous secretion, exhalation, or circulation has been suddenly checked.
DIAGNOSIS.—The diagnosis of the acute anterior poliomyelitis of childhood is usually easy, but unexpected difficulties occasionally arise.
Typical cases are markedly different from typical cases of cerebral paralysis, but in exceptional cases these differences disappear. This is shown in the following table:
Rather singularly, the diagnosis from transverse myelitis is less liable to error than that from cerebral paralysis:
The diagnosis from hæmatomyelitis is almost impossible, and practically useless. For if the hemorrhage be severe, the child dies at once, as in Clifford Albutt's case. If less severe, it excites a myelitis, and the history becomes identical with that of the disease we are considering; or if the clot beyond the anterior cornua, it is identified with a vulgar myelitis of traumatic origin.
Progressive muscular atrophy is extremely rare in childhood, but is occasionally seen under hereditary influence (Friedreich's disease). In adult cases confusion is not only easy to make, but often difficult to avoid, especially with the rare, chronic form of poliomyelitis. The basis of distinction is as follows:
Paralysis from lesion of a peripheric nerve closely imitates anterior spinal paralysis.154It is distinguished by closely following the distribution of the injured nerve, and, usually, by concomitant lesions of the sensibility and of cutaneous nutrition.
154The importance of this fact has been shown in the section on Pathogeny. (See also quotations from Leyden and remarks on lesions of peripheric nerves.)
The pseudo-paralysis sometimes observed in syphilitic children as a consequence of a gummatous infiltration of the bones at the junction of the epiphysis and diaphysis155might easily be mistaken for a spinal paralysis. But it is an affection peculiar to the new-born; the electrical reactions of the paralyzed muscles are intact; careful examination will show that the movements of the muscles are not impossible, but restrained by pain; often other syphilitic affections are present.
155Parrot, Wagner.
The diagnosis from diphtheritic paralysis is embarrassed, from the fact that true anterior poliomyelitis may develop in the course of diphtheria as of other infectious diseases. The paralysis of the soft palate, preservation of faradic reaction, absence of atrophy, and the usually rapid recovery must establish the differentiation.
In spinal paralysis there is loss of the reflexes,156and also of faradiccontractility, both of which are preserved in hysteria. In hysterical paralysis, also, there is no wasting of the affected muscles.
156See Gowers's monograph on “Spinal-Cord Diseases” for an excellent summary of the spinal reflexes.
Various diseases of the bony skeleton or articulations may simulate spinal paralysis. Congenital club-foot, caused by unequal development of the bones and cuticular surfaces, is to be distinguished from the paralytic variety by the date of its appearance,157by the deformity of the tarsal bones, and by the extreme difficulty of reduction.
157Though in some cases paralysis of the muscles of the foot seems to take place during fœtal life, and a club-foot result which is both congenital and paralytic.
Caries of the calcaneum, leading the child to walk on the anterior part of the foot to avoid pressure on the heel, may leave after recovery such a retraction of the plantar fascia as to cause a degree of equinus and varus, with apparent paralysis of the peroneal muscles. I have seen one such case.
Congenital luxation of the hip may simulate paralysis; indeed, by Verneuil, it has been attributed to an intra-uterine spinal paralysis. There is, however, no change in the electrical reactions of the muscles surrounding the joint.
In coxitis, however, Newton Shaffer158has demonstrated a moderate diminution of faradic contractility in such muscles, and a corresponding degree of atrophy; and this fact might complicate the diagnosis of paralysis from arthritis of the hip-joint. Gibney159has called attention to the facility with which this confusion may arise, and Sayre160relates cases of infantile paralysis mistaken for coxitis.
158Archives of Medicine.
159Am. Journ. Med. Sci., Oct., 1878.
160Orthopædic Surgery.
In a case observed by myself, which had been previously diagnosed as coxitis, the mistake was all the more interesting as the paralysis which really existed seemed to have been caused by a meningitis rather than primary myelitis of the cornua.161It thus corresponded to the meningo-myelitic case related by Leyden.
161The details of this case are as follows: C. P——, aged 11, ten months previous to consultation suffered from febrile attack, accompanied by retraction of head, severe pains diffused through body and intense at nape of neck; unconsciousness for thirty-six hours; vomiting; no convulsions. Case diagnosed as cerebro-spinal meningitis by attendant physician. Convalescence in a week, but with pain in lumbar region of back, predominating on right side, so aggravated by standing or walking that both acts impossible. Coincidently, pain in right calf; exquisite tenderness to pressure even from stocking. No complaint in recumbent position. Child could not get from floor to bed, nor raise right leg from ground. As pain subsided walking became possible, but right leg dragged. Chronic twitchings on left side, face, arm, leg. These symptoms lasted ten or twelve weeks, but at end of nine weeks patient could walk up stairs. In ten months power of walking almost recovered, but there remained a certain amount of lordosis and oscillation of pelvis, which is jarred on the left side while the right leg is swung forward. Recumbent, all movements executed equally well on both sides and passive motion of the hip-joint perfectly free. Circumference of right thigh and leg diminished from one-half to one inch as compared with the left. Faradic contractility diminished on the right side in the gluteal muscles, vastus externus, and rectus, and in the gastrocnemii. The sacro-lumbalis muscle was, unfortunately, not examined, but from the lordosis was probably affected. The remaining muscles were intact. Pain on pressure persisted over right side of second, third, and fourth lumbar vertebræ. Diagnosis was made of a limited meningeal exudation, with compression of anterior part of cord or of a portion of the lumbar and of the sacral plexus.
Scoliosis, which may be caused by the relatively rare unilateral paralysis of some of the muscles of the trunk, may also be simulated by paralysis with shortening of one lower extremity. To compensate theshortening, the trunk is bent over on the paralyzed side; hence a lateral curvature, easily reducible, but easily leading into error.
It would seem easy to distinguish traumatic cases of subluxation of the humerus from those due to paralysis of the deltoid. Yet sometimes only the history will serve to establish, and that somewhat doubtfully, the diagnosis.162
162A child of four was brought to me with a stiffness and rigidity of the shoulder-joint which could only very partially be overcome by passive motion, and not at all by voluntary effort. The mother stated that several months previously the child had, without apparent cause, become suddenly unable to move the arm. After two months' delay it was taken to a dispensary, and told that the arm was out of joint, and had it reset under ether. From this date the stiffness had gradually developed. The deltoid was atrophied, with marked diminution of the faradic contractility. Question: Were these signs merely symptomatic of an arthritis consequent on a dislocation, or was the latter the result of a spinal paralysis of the deltoid?
THERAPEUTICS.—The treatment of anterior poliomyelitis embraces two stages. In the first it is directed against inflammation of the spinal cord and the paralysis of the muscles; in the second period the spinal lesion has run its course and the paralysis is considered incurable. Treatment is then directed to the prevention or palliation of deformities or toward facilitating the functions of the limb in spite of them.
These two periods are not, however, rigidly separated from each other in chronological order. From the very outset it is important to take certain precautions to prevent deformities, and while palliating these with orthopædic apparatus it is important for years to continue treatment of the paralyzed muscles in the hope that at least a remnant of them may be saved. To abandon the case to the orthopædic instrument-maker, or to neglect the problem of dynamic mechanics while applying electricity and studying the progress of fatty degeneration, are errors greatly to be condemned.
The treatment of the initial stage is necessarily purely symptomatic for the fever and convulsions, since the diagnosis cannot be made out until these have subsided.
As soon as the diagnosis is clear, however, certain measures should be adopted to diminish the hyperæmia of the spinal cord. Dally163recommends the ventral decubitus; almost all modern authorities advise ice to the spine and ergot internally or subcutaneously. Thus, Althaus164makes hypodermic injections of ergotin in doses of one-fourth of a grain for a child between one and two years old; one-third of a grain between three and five; and one half grain from five to ten; and these doses repeated once or twice daily. The only objection to this treatment is the degree of local irritation it can hardly fail to occasion. Hammond, who “affirms ergot to be of great service, the only medicine capable of cutting short the disease or of limiting its lesions,” recommends the internal administration of the fluid extract—ten drops three times a day for infants of six months, half a drachm for children between one and two years.165
163Journ. Thérap., t. viii., 1880.
164On Infantile Paralysis.
165I have elsewhere quoted one case of early recovery under the use of ice and ergot; or was this a case of temporary paralysis?
The belladonna treatment, at one time so warmly praised by Brown-Séquard, retains to-day few adherents.
Simon advises cutaneous revulsives to divert the circulation to thesurface; thus, hot-air baths, mustard powder sprinkled on cotton enveloping the limbs. Ross advises mercurial inunction along the spine, followed by iodine and blisters. At the same time, iodide of potassium should be given internally in large doses. The action of this drug upon inflammations of the nerve-centres seems, within certain limits, to be indisputable, but its mode of action is certainly very obscure. Where the lesion can be attributed to a meningo-myelitis,166the iodide may be expected to facilitate the absorption of the exudation. In these cases it should be continued for a long time.167
166As in Leyden's first case, and my own.
167Binz explains the local action of iodine by an exudation of leucocytes which follows the dilatation of blood-vessels. These elements break down the exudation into which they are poured, and thus facilitate its absorption.
Electrical treatment may be begun by the end of the first week after the paralysis. At this stage Erb recommends central galvanization as an antiphlogistic remedy for the myelitis. For this purpose a large anode must be placed over the spine at the presumed seat of the lesion, while the cathode is applied over the abdomen. By a slight modification of the method the cathode is placed over the paralyzed muscles. The application is stabile, and, according to Erb, should last from three to ten minutes; according to Bouchut, several hours daily. Erb's method is intended exclusively as a sedative to the local inflammation. When the cathode is placed on the muscles it is hoped that the descending current, replacing the lost nervous impulses, may avert the threatening degeneration of the muscle and nerve.
Faradization cannot modify the inflammatory lesions of the cord. As a means of averting degeneration in completely paralyzed muscles it is inferior to galvanism, and should not therefore be used in those muscles which refuse to contract under its stimulus. Its immense utility, however, is as a stimulus to muscles imperfectly paralyzed, but liable to degenerate from inaction and to be overborne by their antagonists. The excitation of contractions in such muscles is a powerful local gymnastic, helping to maintain nutrition by artificially-excited function.
For the same purpose, muscles inexcitable to the faradic current should be, when this is possible, made to contract by the interrupted galvanic current. After this treatment has been prolonged during several months, the faradic contractility often returns, and the current then should be changed (Seguin).
The value of electrical treatment has been very differently estimated. Erb remarks that “its results are not precisely brilliant.” Roth, whose testimony perhaps is not above suspicion, since evidently prejudiced, insists that numerous cases fall into his hands which have submitted for months to electrical treatment without the slightest benefit. On the other hand, Duchenne, as is well known, has expressed almost unbounded confidence in the therapeutic efficacy of faradization, declaring that it was capable of “creating entire muscles out of a few fibres.”
The sensitiveness of children to the electrical current, and their terror at its application, seriously interfere with its persistent use; as, if the patience of the physician is maintained, that of the parents is very likely to fail in the presence of the cries and resistance of the child.
It is very probable that some of the failures of electrical treatment aredue to the attempt to rely upon it exclusively, instead of suitably combining both electrical methods with each other and with other remedial measures. With our present knowledge it is safe to assert the desirability of persistent electrical treatment during at least the first two years following the paralysis. The currents must never be too strong—the faradic, at least, never applied for longer than ten minutes at a time. The muscles should be relaxed by the position of the limbs (Sayre). If the muscles continue to waste, and especially if they become fatty, the electrical response will grow less and less, and finally cease altogether.168In the contrary case the galvanic contraction will become normal in quality, and the faradic contractility will return and increase, while the atrophy is arrested and the muscle regains its bulk and voluntary powers. Sometimes, as already stated, the latter is regained, while faradic contractility remains greatly diminished.169
168Passing through three stages: faradic contractility diminished, galvanic contraction increased; faradic response lost, galvanic degenerative; absence of contraction to either current.
169Sayre (loc. cit.) has noticed cases in which the muscle would contract several times under faradism, then refuse to do so for a day or two. This observation, if valid and not due to unequal working of the battery, is a most curious one.
A succedaneum to electricity that is highly prized by some authorities is strychnia, especially when subcutaneously administered. Pelione170relates the cure of two cases in children of four and five years, after three and four years' duration of the paralysis, by strychnia—one-half milligramme daily. None should be given to children under six months, but over that age one-ninety-sixth of a grain may be given (Hammond). It should not be given subcutaneously more than two or three times a week (Seeligmüller).171
170L'Union médicale, 1883.
171Duchenne relates a case of a paralysis general at the outset and remaining so for six months. It was then treated by strychnine for five or six months, and at the end of that time had become limited to the lower extremities (Elect. local., ed. 1861, p. 278).
The incidental action of electricity in attracting blood to the paralyzed muscles may be sustained by several other methods.
Among these the external application of heat, either dry or in the form of hot douches, alternating with cold, is an adjuvant remedy of real importance. Beard has suggested tubing, malleable to the limbs, for the conduction of hot water. It is desirable to employ massage immediately after cessation of the hot applications.
On the value of massage and passive gymnastics opinion is even more variable than in regard to electricity. Roth, a specialist in orthopædics, places it at the head of all remedial measures, and denounces electricity in comparison. Many professional manipulators, ignorant of medical science, continually claim wonderful triumphs over regular physicians obtained by means of systematized massage. Volkmann, on the other hand, dismisses the pretensions of the Heilgymnastik with considerable contempt, declaring that faradization is the only method which can really secure exercise to paralyzed muscles.
The Swedish movement cure consists in passive movements imparted to a limb by the manipulator, at the same time that they are strenuously resisted by the patient. From the nature of this method, and its aim in stimulating the voluntary innervation of the muscles, itis admirably adapted to hysterical paralysis. Theoretically, it is difficult to perceive the applicability of this method in organic atrophic paralysis, especially in young children, whose voluntary efforts cannot be commanded. There are, however, several real indications for passive gymnastics in the treatment of infantile paralysis. Surface friction and deep massage have some influence in dilating the blood-vessels and causing an afflux of blood to the cold and wasting muscles. A probably more important effect may be produced upon the contraction caused by malposition and adapted atrophy of certain groups of muscles. It is these contractions which formerly constituted the special objection of the orthopædist, and were treated almost universally by tenotomy. They are in any case the proximate cause of deformities; and, generally existing on the side of the joint opposite to the most severely paralyzed muscles, they keep these over-stretched and prevent them from receiving the benefit of the electrical treatment. Muscles which will not contract to the faradic current while thus stretched will often begin at once to do so when the rigidity of their antagonists has been overcome.
Persevering stretching by the hands will often overcome this rigidity as completely, and even more permanently, than will the tenotomy-knife. It is in this part of the treatment that entirely ignorant and even charlatan manipulations do, not unfrequently, achieve remarkable results.172
172Of course many of those on record, and to some of which I have been a witness, relate to hysterical contractions, hysterical scoliosis, etc.
It is the retracted tendo Achillis and plantar fascia which most frequently require this manipulation. In the paralytic club-foot of young children all authorities agree in the value of repeated manipulations and restorations of the foot as nearly as possible to a position where it may be retained by simple bandaging. While turning the foot out it becomes perfectly white, but on releasing hold of it the circulation is restored, after which the manœuvre may be repeated (Sayre).
This principle of intermittent stretching by seizure of the segments of the limb above and below the joint applies to all forms of paralytic contraction. In the trunk the pelvis should be held by the mother, while the manipulator, seizing the thorax of the child between both hands, moves it gently but forcibly to and fro in the required direction. Great care is required in these manipulations—not merely to avoid exhausting the muscles, but even to avoid fracturing atrophied bones.
It may be laid down as a positive rule that tenotomy should never be performed in the contractions of spinal paralysis until the resources of manipulation have been exhausted. It is to be remembered that the rigidity depends on no active contraction of the muscle, but on its elastic retraction. The manœuvre of stretching does not appeal to the force of contractility, which may have been lost, but to the force of elasticity, which remains and can be made to act in a reverse direction. Finally, in the cases where the retracted muscles have not been originally paralyzed, but have lost the power of contracting during the process of shortening, this power may be restored if the muscle regain its normal length.
The operation of tenotomy, apparently a far more heroic measure, is often a less efficacious means of arriving at the results. Unless followed by the application of apparatus which permits motion in the joint, section of contracted tendons is only of brief utility.
Though the edges of the cut tendon have been kept apart until the intervening space is filled by new tissue, union is finally effected by the latter, and retraction through elasticity is again imminent. Often, therefore, the deformity is repeated in spite of repeated operations; when it is not, the happy issue is due to the fact that, with increased freedom of locomotion immediately after the tenotomy, the patient has been enabled to bring the influence of weight to bear in such a manner as to fix the limb in a new and more convenient position. Thus, after section of the tendo Achillis for pes equinus, if the patient begins at once to walk on the paralyzed foot, the weight of the body, pressing down the heel, may keep the tendon stretched. So walking immediately after section of the hamstring muscles will have a tendency to produce genu-recurvation by the same mechanism which produces it in total paralysis, and the original deformity will not recur.
Besides the tendo Achillis, the parts which may be occasionally submitted to tenotomy are the plantar fascia, the peroneal muscles, very rarely the anterior tibial and extensors, the hamstrings, the thigh adductors. Section of the external rotators of the thigh or of the tensors of the fascia lata could hardly ever be required, and among these operations Hueter173rejects that on the plantar aponeurosis as inadequate. The excavation in the foot it is designed to remedy depends upon alteration in the form of the tarsal bones, and can only be cured by means of forcible pressure exerted on their dorsal surface. Section of the peroneal muscles, often recommended by Sayre, is considered by Hueter to be superfluous after section of the tendon achilleis. Paralytic contraction of the hamstrings or of the hip flexors is rarely sufficiently severe to demand tenotomy.
173Loc. cit., p. 416.
From what has preceded it is evident that maintenance of locomotion is of great importance, in order to avoid the deformities which are threatened by prolonged repose. Locomotion, however, can only be safely permitted with the assistance of apparatus capable of restraining the movements liable to be produced by the weight of the body. The supporting instrument which restrains movement in certain directions must, however, facilitate it in others: immovable apparatus, such as is not infrequently applied after tenotomy, is always injurious.
In young children unable to walk, the development of pes equinus may often be prevented by drawing down the foot to a sole splint made of thin wood, gutta-percha, or felt, and fastening it with a flannel bandage. The point of the foot may be drawn up toward the tibia by a strip of diachylon plaster. If the equinus has already developed, a splint of gutta-percha or of felt (Sayre) may be modelled to the leg and foot while the latter is held forcibly in dorsal flexion. The splint is attached by means of strips of adhesive plaster. It should extend as far as the knee, and be suitably padded (Seeligmüller).
In children able to walk a sole splint of thin metal, to which the foot had been previously attached by a flannel band, should be inserted in a stout leather boot. On the outer side of this boot should run a metallic splint, jointed at the ankle and extending to a leather band surrounding the leg just below the knee. A broad leather band, attached to the outer edge of the sole anterior to the talo-tarsal articulation, also passes up onthe outside of the foot, gradually narrowing until, opposite the ankle, it passes through a slit in the side of the shoe, to be attached to the leg-splint. This band tends to draw the point of the foot outward, and thus correct the varus (Volkmann). Sayre174has improved on this shoe by dividing the sole at the medio-tarsal articulation, in which lateral deviation takes place, and uniting the anterior and posterior parts by a ball-and-socket joint, permitting movement in every direction.
174Loc. cit., p. 88.
The orthopædic boot for the treatment of calcaneo-valgus is constructed on the same principle. But the splint runs up the inner side of the leg, and the leather strap passing to it from the edge of the sole draws the point of the foot inward and raises its depressed inner border (Volkmann). Essential to the treatment of this deformity, however, is the elevation of the heel. This is effected by means of a gutta-percha strap which is attached below to a spur projecting from the heel of the shoe, and above to a band encircling the leg. If, by rare exception, a paralytic calcaneus exists in a child unable to walk, a simple substitute may be found for the shoe in a board sole-splint projecting behind the heel, attached to the foot by a strip of adhesive plaster, which finally passes from the posterior extremity of the board up the back of the leg, and is there secured by a roller bandage.
The device of the gutta-percha elastic band to replace the gastrocnemius muscle illustrates a principle of wide application in orthopædic apparatus. The suggestion to replace paralyzed muscles by artificial ones was first made by Delacroix175in an apparatus designed for the hand. The suggestion was repeated by Gerdy;176and in 1840, Rigal de Gaillac proposed to exchange the metallic springs hitherto used for India-rubber straps. Duchenne elaborated the suggestion in a remarkable manner,177using delicate spiral springs as a substitute for the lost muscles, and taking the greatest pains to make the insertion-points of these to exactly correspond with the insertions of the natural muscles. This was effected by means of sheaths, imitating natural tendinous sheaths, sewed to a glove or gaiter in which the hand or foot was encased.
175Article “Orthopédie,”Dict. des Sciences médicales, quoted by Duchenne.
176Traité des Bandages, 2d ed., Paris, 1837, quoted by Duchenne.
177See chapter on “Prothetic Apparatus” in his treatiseDe l'Électrisation localisée.
At the present day the prothetic apparatus the most employed is that contrived by Barwell.178The principle is the same as Duchenne's, but the artificial muscles are made of India-rubber, to which a small metallic chain is adjusted, and they are attached to the limb by means of specially-devised bands of adhesive plaster and pieces of tin bearing loops for the insertion of the muscle. In this apparatus the artificial muscles do not attempt to imitate the situation of the natural muscles with the precision which Duchenne claimed for his. Barwell's own dressing for talipes valgus consists of two rubber muscles which pass from the inner border of the foot, one to the inner, the other to the anterior, part of a band which encircles the leg just below the knee. For talipes calcaneus another band is required behind the leg, passing to the heel, as in Volkmann's apparatus, already mentioned. For talipes varus a rubber bandshould pass on the outside of the foot; for equinus, one or more from the anterior part of the leg to the sides of the anterior part of the foot.
178A tolerably minute account of the Barwell dressing is given by Sayre,loc. cit., p. 84.
Sayre endorses Barwell's dressing as entirely adequate for the treatment of any form of club-foot, but modifies it by substituting a ball-and-socket shoe for the adhesive plaster which should encircle the foot. The artificial muscles are then passed from the sides of the shoe to a padded leather girdle encircling the leg. A straight splint, jointed opposite the ankle, runs up from each side of the foot to this girdle, and from it two lateral upright bars, jointed at the ankle, pass to the heel of the shoe; and from below the joint passes forward on each side a horizontal bar reaching the point of origin of the artificial muscles and giving attachment to them.
In equinus it is necessary to bind the heel of the foot down firmly in the heel of the shoe; and this is accomplished by means of two chamois-leather flaps which are attached to the inside walls of the shoe and lace firmly across the foot.179
179“The aim of the dressing or instrument is simply to imitate the action of the surgeon's hand; accordingly, any apparatus combining elastic force is far superior to any fixed appliance; and, moreover, that is to be preferred which is the most readily removable. Shoes, therefore, are better than bandages or splints. A proper shoe must have joints opposite the ankle and the medio-tarsal articulation; it must permit the ready application of elastic power; and it must not so girdle the limb as to interfere with the circulation” (Sayre,loc. cit., p. 91).
Sayre places so much confidence in the power of this elastic tension to overcome contractions that he rarely resorts to tenotomy in the treatment of paralytic talipes. Hueter, however,180considers tenotomy much the speediest, and therefore the most desirable, way of removing contractions.181
180Loc. cit.
181Loc. cit.
Seeligmüller quotes with approval Böttger's method for the treatment of deformities, where the weight of the body is utilized to stretch the retracted tendons. Thus, for talipes equino-varus an over-reduction is effected under ether, and the foot forced into a position of moderate calcaneo-valgus. In this position it is retained by the immediate application of a plaster or silica bandage. After this has hardened the child should be encouraged to walk in the mould, with the addition of felt shoes having a slanting sole that is thickened like a wedge at the inner side of the foot and strapped on like a skate. Then, during the act of walking the body tends to constantly force down the heel and thus stretch the retracted tendo Achillis, while the bandage and felt sole (acting like a splint) prevent the inner side of the foot from slipping up.
For talipes valgus the method is analogous, but the foot is forced into an equino-varus position, so that the tendo Achillis is artificially shortened, and ultimately becomes a rigid band, capable, in spite of the sural paralysis, of sustaining the heel.
A cause of relapse in talipes not unfrequently overlooked is the presence of even slight contractions of the hip- and knee-joints. These by shortening the limb tend to the production of equinus, since the foot points itself in order to reach the ground. These contractions, whose rigidity is far inferior to that induced by chronic arthritis, may be overcome by forced extension under ether or gradually by manipulations, or by the weight-and-pulley apparatus, applied in the recumbent position, as in morbuscoxarius. The obvious objection to the latter method is the confinement in bed which it necessitates in a child enjoying at the time perhaps robust general health.
The contraction once overcome, the limb must be placed in apparatus which shall both maintain suitable extension and assist in supporting the trunk during station and locomotion. The latter purpose is effected, as in apparatus for chronic joint diseases, by transferring the weight of the body to steel splints running up each side of the limb, the outer one as far as a girdle which encircles the hips; the inner to a band surrounding the upper part of the thigh. Thus is extended the support which in paralysis limited to the leg-muscles is given by the steel splints inserted in the side of the club-foot shoes.
In the simplest form of apparatus locomotion is expected to be accomplished by the action of muscles inserted above the seat of the paralysis. Thus, when the muscles passing over the ankle-joint are paralyzed, the foot is moved as a dead weight by means of the quadriceps extensor, popliteus, and hamstring muscles inserted at the upper extremity of the leg. If the quadriceps cruris is paralyzed, the rotators of the thigh, ilio-psoas, sartorius, and adductor muscles, passing from the pelvis to the thigh, and which are so frequently intact in atrophic paralysis, are enabled to move the limb if the weight of the body is borne by steel splints, if these be light and properly jointed at the hip, knees, and ankle.182
182Or the joint of the knee may be kept locked while the patient walks, when extension of the limb is mainly required, during both the active and passive movements of locomotion, the necessary flexion being supplied at the hip and ankle. By means of a key the knee-joint can be flexed during the sitting positions.
But an important aid to locomotion may be obtained from the artificial muscles, whose elastic tension is of such value in overcoming contractions. The quadriceps extensor, the most frequently paralyzed, may be supplemented by an India-rubber band and chain passing down the front of the thigh from a point on the pelvic girdle corresponding to the anterior iliac spine to a point on a leg-band, imitating the tibial insertion of the quadriceps tendon. Analogous bands stretched on the posterior aspect of the thigh simulate the hamstring muscles. When the external rotators are paralyzed, the artificial muscle must stretch from the pelvic girdle to a band encircling the upper part of the thigh.
The action of these muscles, apart from their elastic tension of repose, is thus explained by Duchenne: When any effort is made to move a paralyzed limb, the intact antagonists to the paralyzed muscles contract; thus, the flexors of the leg. But this contraction, being constantly opposed by the elastic tension of the artificial quadriceps, is restrained and gradual, instead of being brusque, jerking, and excessive, as it otherwise would be. This is the first result obtained. In the second place, contraction of the antagonist having ceased, the artificial muscle which has been stretched returns upon itself in virtue of its elasticity, and restores the limb to the position of normal equilibrium.
For the act of walking, however, the artificial quadriceps would require to be made tense enough to resist flexion, and thus keep the limb in extension. An artificial anterior tibial muscle, however, would require to yield to the intact gastrocnemius while the heel was being raised from the floor; then its elastic force should be sufficient to retract the point ofthe foot in dorsal flexion during the pendulum movement which passively swings the leg forward. The tension of the artificial muscle should therefore be so adjusted that it can only be overcome by the active contraction of the gastrocnemius, and at the moment of greatest tension, immediately after stretching, it should be able to quite overcome the gastrocnemius, then relatively183relaxed.
183We say relatively, believing that the simultaneous contraction of antagonist muscles has been well established as a constant normal phenomenon.
The anterior tibial, gastrocnemius, and many other of the artificial muscles devised by Duchenne are still in use in the modified form given to them by Barwell. On the other hand, the action of the long peroneus in pronating the foot, and which Duchenne imitated by an elaborate artificial tendon following the exact course of the natural one, is to-day generally supplemented by the jointed shoe and laced bandage.
In paralysis of all the muscles surrounding a joint, when the limb is placid and no retractions by adapted atrophy have taken place, the artificial muscles can only serve to oppose the malpositions which are threatened from mechanical influences.
In the upper extremities prothetic apparatus has been principally used for progressive muscular atrophy. Paralysis of the wrist extensors is perhaps the only case in which the artificial muscle is required in anterior poliomyelitis. A string may be necessary to support the arm in paralysis of the deltoid, to avert luxation of the humerus.
Duchenne's ingenuity did not shrink from the difficult task of supplementing the muscles of the trunk. This he did by inserting the elastic spirals in corsets in a direction following that of the muscles paralyzed. Thus, a unilateral paralysis of the sacro-lumbalis may be met by a spiral splint running up one side of the spine; below, to the lateral posterior portion of a pelvic girdle. In bilateral paralysis two springs are used to antagonize the action of the abdominal muscles.
In Barwell's apparatus for the trunk184India-rubber bands are again substituted for spiral springs. No attempt is made to imitate the direction of muscles, but the force is applied in any direction required to antagonize the pressure producing the deformity.185
184Especially designed for habitual scoliosis, but applicable also to the paralytic deformity.
185Volkmann (loc. cit., p. 778) thinks that the force of Barwell's India-rubber straps, whether for scoliosis or club-foot apparatus, is inadequate, and much inferior to metallic springs.
It is always important to remember the rarity of scoliosis caused by spinal paralysis of the trunk-muscles, and the much greater frequency with which this deformity occurs as a consequence of the paralytic shortening of a leg. A high shoe, equalizing the length of the lower extremities, is then the simple and efficient remedy.
In cases of long standing, even when the scoliosis is due to this cause, certain muscles on the concave side of the curve may become so retracted and rigid as to require tenotomy. Before this operation it is necessary to put the rigid muscles on the stretch as much as possible; and this may be done, if necessary, by means of Sayre's hanging apparatus. After this operation the spine may be straightened out with ease—an important distinction from advanced habitual scoliosis, where the alteration in the shape of the vertebræ defeats all attempts at rectification. The positionmay be maintained by elastic straps or corsets and by removing the condition which has led to the deformity.
Seeligmüller criticises too unfavorably the entire system of elastic tension in the prophylaxis and treatment of paralytic deformities. He quotes Duchenne's admission, that in certain cases traction upon rigidly-retracted tissues becomes insupportably painful, and must be abandoned. It is in these cases that tenotomy becomes an indispensable preliminary to the use of apparatus. Sayre insists that the necessity for tenotomy is indicated when pressure on the rigid muscle is followed by instantaneous spasmodic contraction in the affected or neighboring muscles. He declares that such contractions indicate reflex irritations, show that the muscle has undergone structural change, and that any attempt to stretch or lengthen it would be followed by an excess of irritation and pain.
This explanation can hardly be accepted, since muscles, whether imperfectly or not at all paralyzed, which from position and adapted atrophy have become retracted, have necessarily undergone structural changes. The greater these changes, the greater the diminution of reflex excitability; and in any muscle completely paralyzed and degenerated this is entirely lost. If, however, the afferent nerves retain enough vitality, if the muscle be slightly paralyzed or altogether intact, then irritation of its tendon by stretching may serve to excite contractions in the belly of the muscle. The possibility of such spinal reflexes is demonstrated by the now familiar phenomenon of the tendon reflex in various spinal diseases.186The contractions must be painful from the impediments offered to the progress of the contracting nerve, and from the exaggeratedly vicious position into which they tend to force the limb. Under these circumstances prothetic apparatus must be deferred until section of the tendons has been made.
186“Passive muscular tension excites tonic contraction in a muscle, and this action may, in abnormal conditions, be excessive, as in the myelitic contractions (so-called tendon reflexes).... The afferent nerves commence in the fibrous tissues of the muscle, and seem to be especially stimulated by extension” (Gowers,On Epilepsy, 1881, p. 97).