Volume 5 — DISEASES OF THE NERVOUS SYSTEM. (continued 38)

³⁸ Archiv Psychiatrie, Bd. vi.

Finally, as in cerebral paralysis, the muscles of the trunk, though often paralyzed at the outset, rarely remain so in children—much more often in adults. Eulenburg³⁹ relates one interesting case of complete paralysis and atrophy of the extensors of the back. Even the interspinous muscles were involved, as shown by the divergence of the spinous processes. The paralysis was observed in a girl of fifteen affected since the age of three, and was completely cured in five months by daily faradizations of ten minutes each, and two gymnastic séances, each lasting two hours.

³⁹ Arch. Virch., Bd. xvii., 1859.

Birdsall⁴⁰ has described one case of unilateral paralysis of the abdominal muscles.

⁴⁰ Journal of Nervous Diseases.

Study of the precise combinations of the muscles paralyzed has recently acquired peculiar interest in connection with the localization in the spinal cord of the motor or trophic nuclei of their nerves.⁴¹ Several facts have been ascertained: 1st, that, in notable contrast with progressive muscular atrophy, atrophic paralysis tends to involve definite groups of muscles; 2d, that this grouping is not effected in accordance with the proximity to each other of the muscles on the limb, but with their functional association. Remak affirms that Charles Bell had already called attention to the fact that in cases of local muscular paralysis of the extremities the paralysis does not spread by muscular continuity, but in accordance with the functional association of muscles. Thus, paralysis of the thumb is more often associated with that of the forearm than with paralysis of the other muscles of the hand. 3d. From such grouping may often be inferred a different localization of certain nerve-nuclei than would be supposed from the position of the muscles alone. 4th. That the fibres contained in a single nerve-trunk, but distributed to different muscles, probably separate from each other within the cord, to be there distributed to variously-situated nuclei.⁴²

⁴¹ Ernst Remak, “Localis. der Atroph. lahmung,” Archiv f. Psych., ix., 1879; Ferrier, Brain, vol. iv. No. 3; also, Proceedings Royal Society, No. 212, p. 12.

⁴² The theory of course assumes the truth of the demonstration by which atrophic paralysis is rendered symptomatic of disease of the spinal cord, and the nutrition of a muscle dependent on the integrity of the muscles of origin of its nerves.

In the arm two mutually correlative cases are observed: (a) Immunity of the supinator longus during paralysis of the forearm muscles; (b) paralysis of the supinator in association with paralysis of the deltoid, biceps, and brachialis anticus. The latter constitutes Remak's upper-arm type of localization, and is exhibited in his first case.⁴³

⁴³ Loc. cit.; also, cases 1st and 2d by Ferrier, in which, however, other shoulder-muscles were involved.

Ferrier has experimentally confirmed this muscular association by means of isolated irritation of the fourth cervical nerve, which threw into contraction the supinator longus, together with the deltoid, biceps, and brachialis internus. At the same time, in the experiment the flexors and extensors of the wrist were excited, while in the special form of paralysis noted they were exempt. This electrical method is a less precise mode of analysis than the pathological, for the double reason that (a) fibres whose nuclei are dissociated may pass together in the same root; (b) because the same muscles receive fibres from more than one root: thus the flexors and extensors of the wrist from the fifth as well as the fourth cervical. Thus when the nucleus of the latter was destroyed paralysis would be averted by means of the fibres coming from the fifth root.

The experiment and the pathological observation, however, concur in indicating that the fibres innervating the supinator longus, though passing to it in the path afforded by the radial nerve, afterward ascend in the cord to a ganglionic nucleus in close proximity to those of the upper-arm muscles specified—liable, therefore, to be affected with them. The purpose effected by such association is the supination of the arm.

It is excitation of the fourth root in Ferrier's experiment which gives results most closely corresponding to Remak's observations. Excitations of the fifth and sixth root reveal other combinations, which Ferrier has found realized in adult's spinal paralysis. Thus in his second case, in addition to the group of muscles already mentioned, the rhomboid, infraspinatus, and serratus magnus were paralyzed, the last muscle indicating complication with the fifth root. In the third and fourth cases muscles supplied from the sixth root were joined to those innervated by the fourth and fifth—namely, the pectoralis major and latissimus dorsi.

In this upper-arm type the muscles affected are supplied by three different nerve-stems—the axillary, musculo-cutaneous, and the radial. In the forearm type the most common variety consists in paralysis of the extensors of the wrist, thus exactly imitating lead palsy.⁴⁴ The supinator remains intact, the intrinsic muscles of the hand are sometimes intact, sometimes paralyzed. Sometimes, however, the extensors are relatively intact; the interossei are atrophied, and a clawed hand, resembling that characteristic of cervical hypertrophic pachymeningitis, is developed.⁴⁵

⁴⁴ It is on this fact, indeed, that Remak has been led to argue the spinal nature of saturnine paralysis (“Zur Pathogenie der Blei lahmung,” Archiv für Psych., Bd. vi., 1876).

⁴⁵ The march of this disease, together with that of tabes dorsalis, furnishes data for localizing the nervous nucleus for the wrist extensors. In both diseases the lesion is ascending: in tabes disturbance of sensibility occurs first in the distribution of the sensory fibres of the ulnar nerve; in cervical pachymeningitis the flexors and intrinsic muscles of the hand are first paralyzed. Hence it is to be inferred that the central nucleus for the latter muscles lies in the lower, that for the extensor muscles in the middle, segment of the cervical enlargement of the cord.

The much greater frequency of extensor paralysis in the forearm type of anterior poliomyelitis indicates that the lesion of this disease begins about the middle of the cervical enlargement (see note).

The foregoing groupings have been made out almost entirely from cases of adult spinal paralysis or else of lead palsy. In the lower extremity it is much more difficult to establish such definite muscular association. Certain laws, however, can be made out: 1st. The liability to paralysis increases from the thigh toward the foot; thus, the muscles moving the thigh on the pelvis are the least liable to paralysis, then those moving the leg on the thigh, while the muscles moving the foot and leg and thigh are the most frequently paralyzed of any in the body. 2d. Of the upper thigh-muscles, the glutæi are not infrequently paralyzed, the ilio-psoas hardly ever, the adductors rarely except in total paralysis. 3d. Of the muscles moving the leg on the thigh, the quadriceps extensor is very frequently paralyzed—the most often, indeed, after the foot-muscles: the sartorius is almost always exempt; the liability of the hamstring muscles corresponds to that of the thigh adductors. 4th. At the foot the tibialis anticus often suffers from isolated paralysis, sharing in this respect the fate of the deltoid in the upper extremity—a fact already noticed by Duchenne. On the other hand, (5th) the tibialis anticus often remains intact while the other muscles supplied by the perineal nerve, the perineus longus and brevis, are completely paralyzed.⁴⁶

⁴⁶ Thus Buzzard relates a case of paralysis involving the quadriceps extensor and peroneal muscles, while the anterior tibial were intact.

The remarkable contrast in the morbid susceptibility of the quadriceps on the one hand, and the sartorius on the other, suggests dissociations of their nuclei. Remak relates one interesting case (Obs. 13) where the sartorius was paralyzed—coincidently with the quadriceps, it is true, but also with partial paralysis of the ilio-psoas muscle, which is as rarely attacked as the sartorius itself. The two facts, taken together, would indicate that the nucleus of the sartorius lies high in the lumbar enlargement, in proximity to that of the ileo-psoas. The inference, continues Remak, is reinforced by functional considerations, since the sartorius, obliquely flexing the leg on the thigh, is generally in action at the moment that the psoas flexes the thigh on the pelvis.

Again: according to Remak the tibialis anticus is generally paralyzed together with the quadriceps extensor, although supplied by a different nerve.⁴⁷ And this should be expected from the necessity of exciting dorsal flexion of the foot by means of the tibialis anticus at the moment of extending the leg for the act of walking.⁴⁸

⁴⁷ Obs. 14, 15, 16, 17, from Remak's essay.

⁴⁸ At the moment that the foot is thus flexed, however, to allow the leg to be swung forward, the thigh and leg are both slightly flexed.

Ferrier, from his experiments on the roots of the lumbar plexus, is inclined to doubt this association of the tibialis anticus with the quadriceps, and he adduces Buzzard's case, already quoted, to show coincident paralysis of the quadriceps and peroneal muscles. It is not improbable, however, that fibres associated together in nerve-roots may again diverge in the cord, and thus the discrepancy would be explained.

DIAGNOSIS OF SPECIAL PARALYSIS.—Paralysis of isolated muscles may sometimes be concealed by the vicarious action of their synergists: thus of the extensor communis for the tibialis anticus. Paralysis of both legs and feet may even be partly concealed by the energy of the thigh-muscles, which, using the paralyzed segments of the limbs as inert supports, succeeds in effecting locomotion.⁴⁹ On the other hand, in limbs apparently abandoned to total paralysis persevering search will often discover some muscles or parts of muscles which respond to faradic electricity: these must be considered as susceptible of ultimate recovery.

⁴⁹ Thus in Cornil's famous case, Soc Biol., 1863.

The following table sums up some special diagnostic marks for the different paralyses⁵⁰ afforded by the position of the limb and loss of movements:

Upper Extremity. Deltoid. Absence of deformity, which is averted by weight of arm. Inability to raise arm. Sometimes subluxation. Frequent association with paralysis, biceps, brachialis anticus, and supinator longus.

Lower Extremity. Ilio-psoas. Rare except with total paralysis. Associated with paralysis, sartorius. Loss of flexion of thigh. Limb extended (if glutæi intact).

Glutæi. Thigh adducted. Outward rotation lost. Lordosis on standing. Frequent association with paralysis of extensors of back.

Quadriceps extensor. Flexion and adducting of leg (if hamstrings intact). Loss of extension of leg. Frequent association with paralysis of tibialis anticus.

Tibialis anticus. Often concealed if extensor communis intact. If both paralyzed, then fall of point of foot in equinus. Dragging point of foot on ground in walking. Big toe in dorsal flexion (if extensor pollicis intact). The tendons prominent. Hollow sole of foot (if perineus longus intact).

Extensor communis. Nearly always associated with that of tibialis anticus. Toes in forced flexion.

Peroneus longus. Sole of foot flattened. Point turned inward. Internal border elevated.

Sural muscles. Heel depressed. Foot in dorsal flexion (calcaneus). Sole hollowed if perineus longus intact; flattened if paralyzed. Point turned outward (calcaneo-valgus).

Extensors of back. Lordosis on standing. Projection backward of shoulders. Plumb-line falls behind sacrum (unilateral). Trunk curved to side. Trunk cannot be moved toward paralyzed side.

Abdominal muscles. Lordosis, without projection backward of shoulders.

⁵⁰ See Duchenne, loc. cit., and also Roth, On Paralysis in Infancy, London, 1869.

After the paralysis the most remarkable symptom of anterior poliomyelitis is the rapid wasting of the paralyzed muscles. The atrophy begins within a week after the paralysis, and its progress is even more rapid than that following the section of a nerve. Sometimes all the flesh on a limb is shrivelled down to the bone; at other times the muscular atrophy is concealed by an abnormal development of fat, constituting a pseudo-hypertrophy. When all the muscles surrounding a joint are equally paralyzed and atrophied, no deformity develops,⁵¹ unless, indeed, the segment of a limb is used by means of the non-paralyzed proximate segment. In this case deformities may be produced by the effect of weight quite irrespective of muscular action, or in directions opposed to what we should expect from that.

⁵¹ Except talipes equinus.

The weight of the limb or a portion of it, by stretching paralyzed muscles, often aggravates their atrophy. This is most likely to occur with the paralyzed deltoid when the arm is unsupported, and with the anterior tibial muscles when the foot is allowed to drop.

Muscular atrophy occurs in the spinal paralysis of adults as well as in children; but in the latter alone does the atrophy extend to the bones and cartilages, tendons, fascia, ligaments, and blood-vessels. The osseous projections to which the muscles are attached waste; so do the epiphyses.⁵² The long bones are thinner and shorter, the foot is shorter, and the hand is shortened in paralysis of the upper extremity, even where this is limited to the upper arm, and the forearm is scarcely affected (Seeligmüller).

⁵² Seeligmüller, Centralbl. f. Chirug., No. 29, 1879.

In exceptional cases the limb may become even elongated from passive extension of the ligaments of the articulation. The bones may become soft and flexible, and break if pressure be applied.⁵³

⁵³ Ch. Salomon, “Des Lesions osseuses et articulaires lieés aux Maladies du Système nerveux,” Revue mensuelle, No. 8, 1878.

Atrophy of the bones stands in no fixed relation to that of the muscles, now exceeding, now falling short of that in intensity. This naturally progresses more slowly; still, within seven or eight months there may be a centimeter of difference between two limbs.

In marked contrast with this profound trophic disturbance of the bones is the intact nutrition of the skin. The absence of decubitus is indeed an important diagnostic mark from ordinary myelitis. The subcutaneous fat, however, wastes so completely that the skin seems to be closely adherent to the subjacent tissues, and cannot be pinched up into folds.

The temperature of the skin always falls; the limb is perceptibly colder to the touch than its fellow, and is often bluish and cyanotic. Heine has observed that the temperature diminishes gradually from the centre to the periphery, and at the coldest point may sink to 14° R.⁵⁴ Hammond relates a case where the local temperature was 75° in an atmosphere of 72°. The author says that exact measurements of surface temperature should be taken with Lombard's differential calorimeter, especially when convalescence is expected, as then a rise of temperature, however slight, is of most favorable augury.

⁵⁴ Loc. cit., p. 16. This is not a difference of 14 degrees between the sound and paralyzed limbs, as is erroneously quoted by Seeligmüller (loc. cit., p. 67).

General factors contribute to the fall of temperature: diminished blood-supply from shrinkage of blood-vessels, or even atrophy of a certain number among these; loss of nerve-influence upon the oxidation processes; loss of muscular contractions, which should attract an afflux of blood. Among these factors the loss of nerve-supply is probably the most important, since the others exist in cerebral paralysis without causing the remarkable coldness characteristic of anterior poliomyelitis.

The atrophy of the blood-vessels is not always confined to the terminal twigs. The entire iliac artery, and even the lower part of the aorta, have been found markedly diminished in calibre.⁵⁵ This shrinkage is unaccompanied by any change in the walls of the blood-vessels: it is a simple arrest of development. It strikingly illustrates the dependence of the blood-vessels on the vascular demands of the tissues they are destined to supply.

⁵⁵ Charcot and Joffroy, Archives de Phys., 1870, case by Séguin, loc. cit., p. 9.

The changes which take place in the electrical reactions of the paralyzed limbs rank in importance with their paralysis and their atrophy. They serve to establish the diagnosis, to decide, to a large extent, the prognosis, and to measure the degree of nervo-muscular degeneration.

It is well known that the early diminution, and even entire loss, of faradic contractility was first emphasized by Duchenne as pathognomonic of infantile spinal paralysis. Contractility is diminished in from three to five days after the occurrence of the paralysis, and by the end of a week is completely lost in those muscles in which the paralysis is to be permanent. The muscles which recover spontaneously during the period of early regression recover their faradic with their voluntary contractility. In others, persistently but less profoundly paralyzed and susceptible of cure, the faradic contractility remains simply diminished and in unequal degrees. Progress to recovery under treatment is usually marked by progressive increase in the faradic response; but sometimes the power of voluntary contraction is fully regained, while the faradic response is still permanently lessened. The loss of faradic contractility is more complete and permanent in muscles irretrievably paralyzed by anterior poliomyelitis than in any other disease. These laws have been generally accepted by late observers.⁵⁶

⁵⁶ Simon disputes their validity, and declares that the importance of electricity in the diagnosis of spinal paralysis may easily be exaggerated (Union médicale, 7, 28, p. 942, 1879).

In 1868, Salomon discovered that muscles in which faradic contractility had been completely lost were nevertheless capable of contracting under the stimulus of galvanism—that this contraction is exaggerated, and sometimes occurs at the opening as well as at the closing of the circuit.⁵⁷ The author remarks that the persistence of galvanic reaction after the complete loss of faradic contractility is completely analogous to an observation of Brucke's on muscles poisoned by woorara,⁵⁸ where the intramuscular termination of the nerve is paralyzed. It is to be inferred, therefore, in both cases that the muscular contraction results from direct irritation of the muscle, and implies the entire loss of influence from the nerve.

⁵⁷ Jahrb. f. Kinderheilkunde, N. F. i., 1868. According to Erb (loc. cit., p. 984) and to Ross (loc. cit., p. 111), Salomon was the first to make this observation. Seguin, however, attributes priority to Lobb (Lond. Med. Times and Gaz., 1863), to Hammond (New York Med. Journal, 1865), and to J. Netten Radcliffe between 1863 and 1865. These dates precede that of the publication of Salomon's paper, but the latter seems to have been written without knowledge of earlier observations. (See also Onimus, Soc. de Biol., 1878, who argues that muscle-termination of nerve is partly destroyed.)

⁵⁸ “Ueber den Einfluss der Stromes dauer auf die Elektrische Bewegung der Muskeln,” Sitzber. d. k. Akad. d. Wissensch. in Wien, 1867, Bd. lxi., quoted by Salomon, loc. cit., p. 388.

Erb has greatly extended these observations, and shown that the galvanic reactions of paralyzed muscles indicate their structural degeneration, and are identical with those observed after section of a peripheric nerve. There are three characteristic peculiarities in the contractions thus obtained: 1st, they are slow, tonic, long drawn out; 2d, they are more painful than in normal muscles submitted to an equal amount of electricity; 3d, in complete degeneration the contraction obtained at anode closure equals or exceeds in intensity that excited by cathode closure [AnSZ = or > KSZ]. The excitability of the muscle to the galvanic current remains increased for several months, then gradually diminishes, and finally falls below normal. The qualitative alterations persist somewhat longer: finally, the muscle fails altogether to contract.

Spinal paralysis differs markedly from progressive muscular atrophy in the absence of constant correlation between the degree of paralysis or atrophy and of electrical changes.

The last positive symptom to be noted in the paralytic stage of infantile paralysis is the diminution and ultimate loss of reflex excitability. This is correlative in time and extent with the loss of faradic contractility. This seems to be an exception to the usual rule, which associates loss of tendon reflex with lesion of the posterior columns or nerve-roots. This is a proof that interruption of the reflex arc at any point suffices to abolish the tendon phenomena.⁵⁹

⁵⁹ Buzzard tested the tendon reflex in the zygomaticus major in a patient in whom the sensory branch of the fifth nerve had been stretched, and therefore, to a certain extent insulted. The reflex response was decidedly lower than on the opposite side (Lancet, Nov. 27, 1880).

Negative Symptoms.—The negative symptoms of atrophic paralysis are as important for the diagnosis and pathogeny as are the positive characters, which have now been sufficiently detailed. The absence of decubitus or other nutritive lesions of the skin has been already mentioned. The absence of anæsthesia, or, as a rule, of any marked degree of hyperæsthesia, is most important as indicating immunity of the sensory tracts in the cord. Some diffused hyperæsthesia is sometimes noted during the febrile stage: pain is by no means rare in adults. But in children this is altogether absent, or else slight and transitory. On the other hand, the complete preservation of sensibility constitutes, in children, a serious obstacle to electrical investigation.

After subsidence of the cerebral symptoms, if any, of the initial stage, the functions of the brain are always intact⁶⁰ and the disposition of the children apt to be remarkably lively. The general health is often remarkable for its vigor. The worst, because the most neglected, cases are naturally most often seen among the poor: the ranks of professional beggars are largely recruited from among the victims of infantile paralysis.

⁶⁰ Practically, it may often be of importance for the physician to ascertain that an intellectual enfeeblement, or even idiocy, existing at the time of examination had preceded the onset of the paralysis by months or years.

The chronic stage is marked by the development of a new set of symptoms—contractions of certain muscles surrounding one or more joints and deformed positions of the limbs. These symptoms do not always appear. If all the muscles surrounding a joint are completely paralyzed and extensively atrophied, and if no weight is imposed on the limb by the action of a non-paralyzed upper segment, and if the paralyzed segment be so supported that its own weight does not approximate the insertion-points of muscles, and thus cause their passive retraction, then there is no deformity, but a dangling limb, a membre de Polichinelle.

Laborde states that contractions appeared at the earliest about two months after the paralysis. Seeligmüller, however, has seen pes equinus and pes calcaneus develop in four weeks. The date is partly, at least, determined by the time at which the children try to walk or otherwise to use the paralyzed limbs; and the deformities are very much more marked in the lower extremities, proportioned to the much greater weight which they are obliged to sustain.

All varieties of club-foot, and most frequently equino-varus, knock-knees, rigid flexions at the knee and hip, cyphosis, lordosis, and colossal scoliosis may develop as manifold consequences of atrophic paralysis. That subluxation of the humerus and the claw-hand may occur in the upper extremities has already been mentioned.

In Seeligmüller's 75 cases, 53, or 71 per cent., exhibited some kind of deformity. Among these, 43, or 56 per cent., were of the foot; 6 were cases of subluxated humeri; 5, easily-reducible luxation of the fingers.

The following table contains a summary of the deformities observed as a consequence of atrophic paralysis. They are distinguishable from congenital deformities dependent on altered relations of articular surfaces through defective development⁶¹ by being easily reducible. This remark especially applies to paralytic club-foot:

Foot. Equinus. Equino-varus (varus hardly ever alone). Calcaneus. Calcaneo-valgus. Valgus.

Knee. Genu-recurvation. Genu-incurvation. Permanent flexion.

Hip. Luxation. Permanent flexion. Permanent adduction.

Hand. Flexion fingers or wrist (rare). Extension of wrist. Claw-hand.

Elbow. No deformity (Seeligmüller).

Shoulder. Subluxation humeri.

Trunk. Dorsal scoliosis. Lateral incurvation lumbar region. Cyphosis. Lordosis with backward projection of shoulders. Lordosis without backward projection of shoulders.

⁶¹ Volkmann's Handbuch, Billroth und Pitha.

Mechanism of Deformities.—From what has been said on the cases in which deformities are absent it is evident that one at least of three conditions are required for their production: the paralysis must be unequally distributed in the muscles surrounding a joint; pressure must be exerted by the weight of the body or traction by the weight of the limb; effort must be made to utilize the maimed part of the limb by means of other parts, or even by the muscles of the trunk.

The share taken by these different factors in the production of deformities has been differently estimated by different observers. The French surgeon Delpech was the first to explain the phenomenon on the theory of muscular antagonism. The same theory has been most minutely elaborated by Duchenne.⁶² According to it, the intact or less paralyzed muscles, in virtue of their tonus, constantly tend to draw the segment of the limb on which they act in a direction opposed to that in which it should be drawn by the paralyzed muscles. Since this action is unantagonized, its influence persists; the insertion-points of the contracting muscle being permanently approximated, the nutrition of the muscle is modified: it grows shorter (adapted atrophy). There results finally shortening and retraction of the muscles on one side of the joint, over-stretching of those on the other.

⁶² De l'Électrisation localisée, 1861.

Duchenne used to illustrate this theory by means of a skeleton supplied with artificial muscles, whose successive section would cause the appearance of the corresponding deformity. Werner⁶³ first protested against this theory, and the protest has been further developed and a different theory built up through the efforts of Hueter⁶⁴ and Volkmann.⁶⁵ The latter observes that the muscles and ligaments surrounding a joint normally receive a large amount of the weight falling upon its articular surfaces. Removal of this elastic resistance exposes these surfaces to the full force of the pressure, and thence to almost certain danger of deformity—a danger, therefore, always incurred after paralysis of the muscles. Thus, the weight of the body, pressing, unresisted, on the arch of the foot, is able to displace the bones of the arch from their normal relations and completely flatten the arch.

⁶³ Reform der Orthopædie, 1845.

⁶⁴ Gelenkkrankheiten.

⁶⁵ Sammlung klin. Vort., No. 1.

A position of ease is that in which the movement of the joint has been pushed as far as possible until limited by the passive resistance of the ligaments or the conformation of the articular surfaces. The weight of the body must then be so placed that the line of gravity falls on the side of the open angle, while the limiting bands stretch across the base. Thus, a tired man sits with a curved back; the muscles which may extend the spinal column in a straight line are relaxed; the column falls forward until arrested by the anterior vertebral ligaments. Thus, in standing at ease the thigh presses against the leg, so as to form a wide angle open anteriorly. When the quadriceps extensor is paralyzed, this position is inevitable and exaggerated, since the force which might counteract it, contraction of the thigh extensor, has been removed.

Formerly, the rôle of muscles in this elastic resistance was under-estimated and that of ligaments exaggerated.

CRITICISM OF THE THEORY OF MUSCULAR ANTAGONISM.—Three considerations have been urged in objection to the theory of muscular antagonism: First, deformities may develop even in limbs totally paralyzed, provided these limbs be subjected to weight and pressure. Pes equinus, the most common form of paralytic club-foot, develops with total paralysis of the muscles of the leg where the child does not walk, but is carried on the arms of a nurse with its foot dangling. The part of the foot anterior to the ankle-joint being longer and heavier than that behind, the point falls; the tendo Achillis is passively shortened, and by nutritive adaptation to this position may become permanently retracted. Long persistence in this position accustoms the dorsal surface of the bones to a less degree of pressure than the plantar surface: as a consequence, the growth of the bone becomes more active above, while it is arrested below; the arch of the foot is increased until the sole is curved into a deep hollow; and the plantar aponeurosis is correspondingly shortened.

Volkmann relates a case where this same deformity appeared without the least paralysis, but simply from prolonged passive extension of the feet in bed. The patient was an adult, and suffered from a severe typhoid with a double relapse. After recovering from the fever a year of orthopædic treatment was required to restore the feet to their normal position.

The second objection is the absence of any proof of such constant tonus in the muscles as may be sufficiently powerful to determine the position of a limb. Such tonus exists in the involuntary muscles, especially in those of the blood-vessels, but there is no evidence that it exists in the voluntary muscles. To this Seeligmüller has replied by admitting the objection to the theory as thus proposed, but substituting the more plausible influence of repeated contractions on the part of the non-paralyzed muscles. Each contraction draws the limb in a certain direction, and there it tends to remain, because there is nothing to antagonize the force which it has obeyed.

The third objection is that examination of individual cases not infrequently shows displacements in directions opposed to that which should be determined by muscular antagonism. Volkmann has especially illustrated the latter assertion by the mechanism of genu-recurvation. In paralysis of the quadriceps extensor of the tibia the weight of the body is exercised, not merely from above downward, but from without inward, falling, therefore, on the inner malleolus. It thus tends to press the anterior part of the foot outward,⁶⁶ and a valgus finally complicates the calcaneus. Seeligmüller, however, quotes two cases of pes calcaneus developed in children who had never walked: in one paralysis of the sural muscles had occurred at the age of four weeks, and the other case was observed at the age of fourteen weeks. Seeligmüller remarks that only early examination of the faradic contractility of a group of paralyzed muscles can decide whether any among them preponderate during a time sufficient to fix the limb in a vicious position. Thus in one case of pes calcaneus he found two years after the occurrence of the paralysis that some contractility still persisted in the dorsal flexors, but six months later this had quite disappeared. Had the examination then been made for the first time when all the muscles were equally paralyzed, it would have seemed impossible to explain the deformity by muscular antagonism.

⁶⁶ Sayre asserts that lateral rotation cannot take place at the ankle-joint, but at the medio-tarsal articulation. Hueter also refers pes varus and pes valgus to the talo-tarsal articulation.

RELATION OF WEIGHT AND MUSCULAR FORCES.—The influences of weight and of muscular action sometimes concur, sometimes are opposed to each other. Thus, the weight of the foot alone always tends to produce equino-varus; it acts therefore to intensify the action of the sural muscles when the anterior tibial are paralyzed, but to diminish the influence of paralysis of the gastrocnemius when the foot is being drawn into dorsal flexion. Hence one reason for the comparative rarity of pes calcaneus.

Paralytic deformities at the hip and knee are much rarer than those of the foot. At the hip this immunity is partly due to the relative rarity of paralysis in the muscles surrounding the joint—still more to the fact that the weight of the limb tends to correct excessive flexions. These are therefore more likely to occur in children allowed to remain in bed than in those who are encouraged to walk by means of suitable apparatus. The use of crutches, however, favors the development of deformity, because, since with paralysis of the thigh- or leg-muscles pes equinus nearly always exists, the thigh is unduly lengthened. To palliate this inconvenience the patient instinctively flexes the knee or hip, or both, and the position tends to become permanent.

When the flexion is rigid and extension becomes impossible, the gastrocnemii are relaxed until they lose their power of tension, and thence of fixing or raising the heel. Further, as by the flexion the limb is moved in front of the body, it is necessary to project the body forward again over the support. Hence a lordosis is developed, to be distinguished from that caused by paralysis of the vertebral extensors (a) by the rigid flexion of the thigh; (b) by the facility with which the patient can extend the back as soon as he is placed in a sitting position.

Although the quadriceps extensor is so frequently paralyzed, rigid flexion at the knee—such as on the theory of muscular antagonism might be expected from the action of the hamstring muscles—is very rare. As already observed, it occurs, if at all, in neglected children allowed to lie or sit with the leg partly flexed. In those who attempt to walk the leg is not flexed, but forced into hyper-extension by the following mechanism: The muscles inserted on the upper part of the thigh swing the leg forward like a passive support. Afterward the body bends forward over the support, and its weight, pressing from above downward and from before backward, and pressing the articular surfaces of the joint together, forces the head of the tibia backward until the movement is checked by the posterior ligaments. The deformity is the same whether the quadriceps or one or all the muscles surrounding a joint are paralyzed (Volkmann).

DISLOCATIONS.—Dangling limbs are, however, much more frequently the consequence of total paralysis, with extraordinary relaxation of the ligaments of the joint permitting dislocation. Reclus⁶⁷ has published several such cases. Verneuil has even suggested that congenital luxation of the hip-joint always depends on an intra-uterine spinal paralysis.⁶⁸<

⁶⁷ Revue mensuelle de Méd. et de Chir., Mars, 1878.

⁶⁸ Quoted from Seeligmüller.

In the upper extremities rigid contractions are much less frequent, even in proportion to the number of paralyses. Seeligmüller has seen five cases of reducible flexion of the fingers, and one of permanent extension of the wrist and fingers, associated with paralysis of all the flexors. In this case, if the arms were so suspended that the hand hung freely, its weight gradually overcame the action of the extensors and the fingers fell into flexion. Upon any attempt at exertion the hyperextension was reproduced.

DEFORMITIES OF THE TRUNK.—Scoliosis will be caused when, with unilateral paralysis of the extensors of the vertical column, the lower part of the trunk is drawn to the non-paralyzed side, and the upper half is bent over the paralyzed side in order to restore the balance. In bilateral paralysis of the extensors both shoulders are projected backward, so that a plumb-line dropped from between them falls behind the sacrum, and lordosis is developed, although the lumbar column is not projected forward. The same form of lordosis occurs when the glutæi are paralyzed. When, however, the abdominal muscles are paralyzed, the lumbar column is really projected forward, and then a plumb-line dropped from the shoulders passes over the sacrum.⁶⁹

⁶⁹ Duchenne, loc. cit., 1861.

PARALYSIS OF THE ABDOMINAL MUSCLES.—Unilateral paralysis of the extensors of the back is often difficult to detect in young children. Seeligmüller recommends that the child be laid across the mother's knees and told to move the trunk from one side to the other while the pelvis is held firm. If too young to obey the direction, the movement can be excited by pricking or by electric irritation. It will be seen that the trunk can be turned only to one side.

In these paralyses of the trunk it is clear that the deformity does not develop under the influence of muscular antagonism alone, but only when the non-paralyzed muscles attempt to sustain the superincumbent weight of the body or a portion of it.

SUMMARY OF MECHANISMS OF THE DEFORMITIES.—We may indeed conclude, as stated at the beginning, that this complex etiology exists in almost all cases. When the limb is at rest in bed or the weight of the body is transferred to crutches, then repeated contraction of the flexus will suffice to bring the limb into a vicious position (contraction at knee- and hip-joint). When the foot or hand hangs unsupported, its weight is sufficient to cause deformity, even when all the muscles are paralyzed, and sometimes in opposition to the direction of intact muscles. Thus the weight which is passively borne by the limb, and the efforts of intact muscles to effect the function of the limb in spite of the paralysis, both concur in the production of the deformity.

ANATOMICAL LESIONS.—The theory of the anatomical basis of infantile paralysis constitutes one of the most interesting portions of its history. It is indeed one of the most instructive chapters of modern pathology from the rapidity with which in a short time precise knowledge has accumulated, and for the degree in which this has revolutionized previous ideas.

Autopsies.—Until 1863 only five autopsies had been made upon persons affected with infantile paralysis.⁷⁰ As the disease was never, of itself, fatal, opportunity for pathological investigation did not present itself, while attention was still vividly attracted to the paralysis. When this had become a chronic infirmity, and patients had succumbed to intercurrent disease, the opportunity must have occurred, but was not then utilized.

⁷⁰ I believe the first list of modern autopsies was made by myself in a paper read before the New York County Medical Society, Dec. 22, 1873. The paper was not published until May, 1874, and in the mean time Seguin's lecture on “Infantile Paralysis” was published in the N. Y. Med. Record, Jan. 15, 1874, with a tabulated list of 25 autopsies, necessarily almost identical with mv own—cases 9 and 10 are really identical. A case by Roth is included; 4 negative autopsies are omitted. In 1880, Seeligmüller, in his elaborate essay in Gerhardt's Handbuch, published a list of 32 cases, including the above, and adding to them the following: Case by Müller, 1871; 4 cases by Leyden, 1875 (Arch. de Psych.); 1 case by Raymond, 1874 (Gaz. méd. de Paris); 1 case by Demme, 1876 (B. med. Bericht über das Jennerischt Kinderspital zu Bern); 1 case by Eisenlohr, 1876 (Tageblatt des Hamburger Naturforscher Versammlung); 1 case by Schultze, 1877 (Virch. Arch., Bd. lviii.).

To this list we may now add 3 cases by Turner, Humphrey, Taylor, 1881 (Path. Trans. London); 2 cases by Dejerine, 1878 (Progrès méd.), giving a total of 37 cases with detailed histories and autopsies.

The first cases affording autopsies in which the spinal cord was thoroughly investigated, belonged, however, precisely to this class of extremely chronic lesions, which should be regarded as showing rather the results of the morbid process than that process itself. Omission to observe this distinction has been the occasion of several misinterpretations of the pathological appearances.

Premature Theories.—In the entire absence of evidence it is a little remarkable that such high authorities as Rilliet and Barthez,⁷¹ West,⁷² Vogel,⁷³ Eulenburg,⁷⁴ should have pronounced dogmatically that the disease was essential—i.e. unaccompanied by any structural lesion whatever; and that Bouchut,⁷⁵ on the strength of most incomplete examination, should have built up a theory of myogenic paralysis. It is still more remarkable, after the published autopsies of Rinecker,⁷⁶ Laborde,⁷⁷ Cornil,⁷⁸ Prévost,⁷⁹ Charcot and Joffroy,⁸⁰ Parrot and Joffroy,⁸¹ Vulpian,⁸² Roger and Damaschino,⁸³ that Politzer in 1866,⁸⁴ Brown in 1871,⁸⁵ Barwell in 1872,⁸⁶ Kétli,⁸⁷ Adams in 1873,⁸⁸ should still adhere to this doctrine. They are, however, entirely in the minority, and all recent monographs and works published with a view to presenting the state of science assume the spinal nature of infantile paralysis to be established beyond possibility of controversy.⁸⁹

⁷¹ Traité des Maladies des Enfants.

⁷² Diseases of Children, Am. ed., 1860.

⁷³ Diseases of Children, trans. from 4th ed., 1870.

⁷⁴ Arch. Virchow, Bd. xvii. 1859.

⁷⁵ Deutsche Klinik, 1863.

⁷⁶ Gaz. méd., 1864, Soc. de Biol., 1864.

⁷⁷ De la Paralysie de l'Enfance.

⁷⁸ Gaz. méd., 1866, Soc. de Biol., 1866.

⁷⁹ Arch. de Phys., 1870, p. 134.

⁸⁰ Ibid., p. 310.

⁸¹ Ibid., p. 316.

⁸² Bouchut is said by Simon (loc. cit.) to be alone in his theory; Kétli, however, agrees with him. Eulenburg, in 1872, in his systematic treatise, assigns a central origin to infantile paralysis, but offers no opinion in regard to its nature.

⁸³ Gaz. méd., 1871.

⁸⁴ Jahrbuch für Kinderkrankheiten, 1866.

⁸⁵ Compend. für Kinderkrank., p. 161.

⁸⁶ Lancet, 1872.

⁸⁷ Jahrbuch für Kinderkrank., 1873.

⁸⁸ Treatise on Club-foot.

⁸⁹ See Seguin, loc. cit., 1874; Erb, Ziemssen's Handbuch, Bd. xi.; Seeligmüller, Gerhardt's Handbuch der Kinderkrankheiten; Ross, Treatise on Diseases of Nervous System, vol. ii.; Hammond, Diseases of Nervous System, 6th ed., 1881, etc. etc.

The following table contains a summary of the seven autopsies in which the spinal cord is said to have been examined with negative results. Of these, the only really important case is the third, in which a microscopic examination, made by so competent an histologist as Robin, was said to have discovered no lesion of the cord.

The foregoing autopsies may be tabulated as follows:

TABLE I.—NEGATIVE AUTOPSIES.

No. Year. Name of Author and Patient. Age at time of Paralysis. Age at time of Autopsy. Nature of Symptoms. Limbs Paralyzed. Electric Reaction. Appearances at Autopsy. Reference in Literature.

1 1850 or '51? Rilliet and Barthez. ? ? ? l. o. ? Negative. Gaz. méd. de Paris, 1850 (or '51?) p. 681.

2 1850 or '51? Rilliet and Barthez. ? ? ? b. u. ? Negative. Ibid.

3 1867 Bouchut and Robin. (Angélique Lermain). 1½ 3 Suddenly after 3 days' fever. both u. Negative micro- scopically. Union méd., 1867, No. 130, p. 187.

4 1873 Adams. — — — — — Negative. Treatise on Club-foot, p. —.

5 1873 Elischer and Kétli. — — — — — Negative. Jahrb. Kinderheilk., 1873.

6 1873 Elischer and Kétli. — — — — — Negative. Ibid.

Heine, in the absence of autopsies, but arguing from clinical symptoms alone, already inferred the existence of a spinal lesion as cause of the paralysis, and believed that it consisted in congestion, or even in hemorrhagic exudation, capillary or massive, which should compress the cord and result in partial atrophy. The same opinion is advanced in 1844 by Brunnière,⁹⁰ also by Vogt,⁹¹ in 1868 by Salomon⁹² and Radcliffe.⁹³ The autopsies contained in the following table, in all of which vascular lesions are prominent, might be invoked in support of this view:

TABLE II.—AUTOPSIES SHOWING VASCULAR LESIONS OF CORD.

No. Year. Name of Author and Patient. Age at time of Paralysis. Age at time of Autopsy. Nature of Symptoms. Limbs Paralyzed. Electric Reaction. Appearances at Autopsy. Reference in Literature.

7 1829 Klein. 5 5 Persistent cerebral symptoms. l. o. ? Congestion of pia around roots of left brachial plexus. Quoted by Heine.

8 1855 Brund. 1 5 Meningitis? r. u. ? Chronic spinal klepto-meningitis.

9 — Hammond. 4 yrs. stand. — l. u. ? Encysted clot in left ant. column, lower dorsal cord. Journ. Psych. Medicine, 1867.

⁹⁰ Krankheiten des Gehirns und Ruckenmarkes.

⁹¹ Lahmung der Kinder, p. —.

⁹² Jahrb. f. Kinderheilk., 1865.

⁹³ Reynolds's Syst. of Medicine.

The first really modern autopsy, that made by Cornil in 1863, agrees with the two earliest on record in disclosing only an atrophy of the cord. Some of the other cases, contained in Table III., note in addition sclerosis of the lateral columns.

In all the remaining autopsies on record are noted atrophy of the anterior gray cornua and more or less extensive destruction of the ganglionic cells.

In a certain number of cases the atrophy seemed to be the unique lesion. But it never was confined to the ganglionic cells, but included the reticulum of gray fibres in which these were imbedded, and which was replaced by a reticulum of connective tissue. These cases were all examined many years after the occurrence of the paralysis.

TABLE III.—AUTOPSIES SHOWING ATROPHY OR SCLEROSIS OF WHITE COLUMNS OR ROOTS.

No. Year. Name of Author and Patient. Age at time of Paralysis. Age at time of Autopsy. Nature of Symptoms. Limbs Paralyzed. Electric Reaction. Appearance at Autopsy. Reference in Literature.

10 1825 Hutin. 7 49 Convulsions. b. u. ? Atrophy of cord from below 8th dorsal nerve to thickness pencil, and of nerves. Quoted by Heine, p. 151.

11 1842 Lunget. ? 8 ? r. u. ? Atrophy and brown discol. of ant. roots of lumbar and sacral nerves going to sciatic. Anat. et Phys. du Syst. nerveux, i. p. 358.

12 1863 Reckling- hausen. ? ? ? b. u. ? Atrophy of ant.-lateral columns. Deutsche Klinik, 1863, Jan. 3. Seel. says also atrophy of cells; Seguin and J., tubercles.

13 1863 Cornil. 2 49 ? b. u. ? Atrophy antero-lateral columns. Seeligmüller says also atrophy of ganglion-cells, as if quoting Charcot. Soc. de Biol., Comptes Rendus, 1863. Infiltration of gray and white corpuscles, with abundant amyloid corpuscles, especially in anterior gray cornua. These through entire extent of cord—cells—intact. Paraplegia, but walks by muscles of thigh and pelvis; leg and foot atrophied.

14 1864 Laborde and Bouvier. 1 2 ? all four. noted. Atrophy antero-lateral columns; nerve-fibres atrophied and varicose. Laborde, De la Paralysie de l'Enfance, pp. 109-119.

15 1864 Laborde and Cornil. ⅔ 2 ? b. u. ? Sclerosis of ant.-lateral columns. Ganglion-cells normal; sciatic neuritis. Laborde, loc. cit., p. 104.

The first group is contained in the following table:

TABLE IV.—AUTOPSIES SHOWING ATROPHY OF THE ANTERIOR CORNUA AND GANGLION-CELLS.

No. Year. Name of Author and Patient. Age at time of Paralysis. Age at time of Autopsy. Nature of Symptoms. Limbs Paralyzed. Result of Autopsy. Reference in Literature.

16 1866 Prévost. ? 78 ? L. u. Atrophy of left anterior cornu, especially of ganglion-cells. Comptes Rendus, Soc. Biol., Gaz. méd., 1866.

17 1870 Charcot and Joffroy. 7 32 Sudden. All esp. l. u. Atrophy of cells ant. horns and ant. lateral columns. Archives de Phys., 1870.

18 1870 Parrot and Joffroy. — 3 Unknown. B. l. Atrophy and partial sclerosis of anterior cornua and ant. lat. columns.

19 1870 Vulpian. ? 66 ? L. l. Atrophy of ganglion-cells, sclerosis of antero-lateral columns. Archives Physiol., 1870.

20 1871 Müller. 4 34 Fall from bed. L. lower. Atrophy of ganglion-cells ant. cornua; sclerosis of anterior roots. Quoted by Seeligmüller, Gerhardt's Handbuch, Tabeln.

21 1873 Lancereaux and Pierret. 2 18 ? L. upper. Atrophy of external group cells in ant. cornua; general atrophy of left half of lumbar cord. Petitfils, Thesis on Atrophie aigue des Cellules motrices, 1873, p. 33.

22 1875 Raymond. ? 75 ? R. arm. Atrophy of ganglion-cells in right ant. horn cerv. region. Gaz. méd de Paris, No. 19, 1875.

23 1879 Taylor. 1½ 3 3 days' fever. L. leg. Atrophy of left ant. cornua; disappearance of ganglion-cells; no lesion in white column. Trans. Path. Soc. London, 1879.

24 1875 Dejerine. — — — R. foot (congenital equinus). Atrophy of ant. horn right side; atrophy external group of motor-cells. Arch. Phys., 1874.

25 1879 Humphreys. 1½ 3½ ? L. leg. Atrophy of left half lumbar cord; disappearance of medial group cells both sides; antero-lateral on left side. Trans. Path. Soc. London, 1879.

In the remaining cases the ganglion-cells of the anterior cornua had also disappeared; but in addition to this atrophy excited distinct evidence of more or less extensive inflammation. This table includes one case of autopsy at two months (Roger's); one at six weeks after paralysis (Turner's).

TABLE V.—AUTOPSIES SHOWING EVIDENCE OF MYELITIS, TO WHICH THE ATROPHY OF THE GANGLION-CELLS WAS CONSECUTIVE.

No. Year. Name of Author and Patient. Age at time of Paralysis. Age at time of Autopsy. Nature of Symptoms. Limbs Paralyzed. Appearance at Autopsy. Reference in Literature.

26 1866 Echeverria. 3 10 Fever. R. arm, R. leg. Granular pigmentation anterior cornua, diffuse myelitis. Quoted by Seguin, loc. cit.

27 1866 Echeverria. 2 2½ Febrile diarrhœa. Both legs. Ibid. Ibid.

28 1871 Roger and Damaschino. 1⁵/6 2 Scarlatina with second attack. L. arm, then both legs. Foci of softening throughout cord. Atrophy ganglion-cells and nerve-fibres. Dilatation, fatty degen. vessels. Sclerosis ant. columns. Atrophy ant. roots. Gaz. méd. de Paris, 1871.

29 1871 Roger and Damaschino. 2 2½ Variola. Both legs. Foci of softening in ant. cornua. Fatty degen. blood-vessels, circumscribed myelitis. Ibid.

30 1871 Roger and Damaschino. 2 3 Fever. Ibid. Foci of softening, as above. Ibid.

31 1873 Roth. 1 2 Ibid. Ibid. Focus myelitis on both sides. Disappearance ganglion-cells, abundant exudation corpuscles. Virch. Arch., 1873, Bd. lviii.

32 1868 L. Clarke. 1 32 After inoculation. Both arms. Extensive myelitis ant. and central part gray matter. Disappearance ganglion-cells. Med.-Chir. Trans., li. p. 219.

33 1876 Schultze. 3 22 Acute disease. Both legs. Atrophy lumbar cord ant. lat. columns. Atrophy ganglion-cells. Abundant exud. corpuscles. Proliferation neuroglia. Virch. Archiv, Bd. lxviii., 1876.

34 1876 Leyden. 2 60 Fall from table. L. leg. Circumscribed cicatrix with complete destruction gray substance. Amyloid degeneration. Atrophy ant. roots. Archiv f. Psych., 1876, Bd. vi.

35 1876 Leyden. ¾ 1¾ Suddenly. L. leg. Atrophy ganglion-cells. Cellular diffused infilt. (myelitis) gray subst'ce. Ibid.

36 1876 Leyden. 4 58 Sudden over night. L. leg. Amyloid infiltration ant. horns, atrophy ganglion-cells left lumbar horn. Diffuse atrophy gray substance. Ibid.

37 1876 Leyden. 3 20 Ibid. R. leg, L. arm. Sclerotic focus in right lumbar and in left cerv. region. Diffuse meningo-myelitis. Atrophy ant. lat. columns. Encapsulated myelitis. Ibid.

38 1876 Demme. ? 3½ ? — Purulent focus in ant. cornua lumbar region. Atrophy ganglion-cells. Quoted by Seeligmüller, loc. cit., p. 18.

39 1876 Eisenlohr. ? ¾ ? B. legs. Diffuse inflammation anterior horns and ant. lat. columns. Ibid.

40 1879 Turner. 2½ 2¾ ? Myelitic disorganization gray substance ant. cornua lumbar cord. Trans. Path. Soc. Lond., 1879.

41 1883 Archambault et Damaschino. 30 m's 31 m's, or 26 d'ys fr. début. Malaise and prostration one day. Left leg and right arm. Focus red softening ant. horns left lumbar; right cervical region. Enormous enlargement vascular network, and distension blood-vessels; granular corpuscles in lymphatic sheaths; marked atrophy cells and of myeline sheaths of fibres in ant. roots; myeline balls in sheath; axis-cylinders disappeared. Lesions more or less marked throughout cord. Nerves not examined. Le Union médicale, 1883.

42 1884 Money. 2 yrs. 28 m's. Fever and vomiting for a week. Paraplegia. Red area both anterior cornua lumbar region. Here distension and thrombosis vessels; infiltration leucocytes; absence multipolar cells. Trans. Path. Soc. Lond., 1884.

1884 Money. Unknown 7 yrs. Unknown. Right leg. Wasting anterior horn almost throughout lumbar region. Atrophy cells chiefly in ant., ant.-lat., post.-lateral, and central groups, replaced by dense nucleated tissue.

It will be useful to add another table, which will group together the cases in which the autopsies were made within two years after the occurrence of the paralysis. Of these, all but the two made by Laborde, in which the cornua are declared to be healthy and the lesion limited to the white columns, show traces of destructive morbid processes in the gray substance of the cord, greatly predominating in the anterior cornua, but not absolutely limited to them, nor even to the part of the cord which corresponds to the paralyzed limb:

TABLE VI.

Case No. Name of Author. Date of Autopsy after Paralysis. Year of Publication.

41 Damaschino. 6 weeks. 1883

40 Turner. 26 days. 1879

28 Roger and Damaschino. 2 mos. 1871

42 Money. 4 mos. 1884

27 Echeverria. 6 mos.

39 Eisenlohr. 6 mos.

29 Roger. 6 mos. 1871

31 Roth. 9 mos. 1873

35 Leyden. 11 mos. 1876

14 Bouvier and Laborde. 12 mos. 1864

30 Roger. 13 mos. 1871

15 Cornil and Laborde. 16 mos. 1864

12 Recklinghausen. 24 mos. 1863

38 Demme. 24 mos. 1876

23 Taylor. 18 mos. 1879

25 Humphrey. 24 mos. 1879

AUTOPSIES OF RELATIVELY RECENT CASES.—In cases relatively recent all macroscopic changes in the cord may be entirely wanting. There may be some degree of asymmetry in the surface of section, patches of white coloration in the anterior gray substance, or of gray or yellow color in the white columns; the anterior roots may be congested or even already atrophied.⁹⁴ On the other hand, there have several times been found foci of visible red softening, much more frequently at a point corresponding to the origin of the paralyzed nerves, but not absolutely confined to them, and sometimes existing at points where they have given rise to no symptoms whatever.⁹⁵

⁹⁴ Roger's first case, No. 28 of Table V.

⁹⁵ Case 1st of Roger, Tab. V.

Microscopic Lesions.—In striking contrast with this paucity of macroscopic lesions are the interesting structural changes revealed under the microscope. These lesions are usually comprised within circumscribed foci whose size may vary from a long diameter of 2 mm.⁹⁶ to one of from 10 to 30 mm.⁹⁷ Sometimes bilateral foci are found with monoplegic paralysis; thus one side or the other preponderates in the morbid process.

⁹⁶ Case Roth, Tab. V.

⁹⁷ Case Schulze, Tab. V.

In recent cases (Damaschino's, at twenty-six days; Roger's, at two months; Turner's, at six weeks) patches of red softening existed at the portions of the cord containing the nuclei of origin of the paralyzed nerves. In Turner's case the focus contained hemorrhagic extravasation, and the traces of this were clearly perceptible in Roger's first case. The blood-vessels are dilated; their lymphatic sheaths infiltrated with leucocytes and with granular corpuscles; their walls are thickened, pigmented, or fatty.

Leucocytes are often disseminated through the diseased area, and in one case (Demme) were accumulated into a focus of pus. Besides the leucocytes, the foci are often infiltrated with large round granular cells that seem to be transformed neuroglia-cells (Leyden). In one case neuroglia nuclei were accumulated in a ring around the focus, seeming to indicate the beginning of encapsulation.

The most striking lesion, however, and the one which is common to the most recent as well as to old cases, is the deformation, atrophy, and final disappearance of the large ganglionic cells of the anterior cornua. The first change consists in granular pigmentation;⁹⁸ then the prolongations disappear, leaving the body of the cell shrunken and deformed; at last the whole cell disappears. Sometimes all the cells of an anterior horn have disappeared throughout the entire depth of the focus; quite as often, in certain sections at least, the atrophy is limited to certain groups, as the external,⁹⁹ or the external in one focus, the antero-lateral in another, situated on the opposite side of the cord.¹⁰⁰

⁹⁸ Case of Echeverria, Tab. V.

⁹⁹ Case by Schultze (this is a case of ancient lesion), Tab. V. (Virch. Arch., Bd. lviii.).

¹⁰⁰ Case by Taylor, Tab. IV. (Path. Trans., London, 1879.)

In a case rendered celebrated by Charcot it is stated that in many sections of the cord atrophy of ganglionic cells constituted the unique alteration, the tissue immediately surrounding the place whence they had disappeared being perfectly healthy. It is on this appearance that has been built up the theory of a primary idiopathic atrophy of the ganglionic cells as the characteristic lesion of infantile paralysis. But in other portions of the same cord Charcot himself describes destruction of the gray reticulum imbedding the cells; and this destruction is insisted upon in many other observations. In other words, there is a general disintegration of the gray nervous tissue of the anterior cornua which contain the focal lesion. The normal tissue is then replaced by a reticulum of conjunctive fibres, more or less dense according to the age of the case.

These focal lesions of the cord explain admirably, as will be seen, the permanent symptoms of the disease. But of great importance for understanding its initial period is the fact that structural changes—similar to, but less severe than, those just described—have beer found diffused throughout the cord. In several cases hyperæmia, partial atrophy of ganglionic cells and nerve-fibres, infiltration with exudation corpuscles, in the anterior cornua and even central gray canal from the lumbar to the cervical region.¹⁰¹ In Damaschino's case, besides the focal lesions which corresponded to the paralyses of the right arm and left leg, were others corresponding to the left arm and right leg where no paralysis existed.

¹⁰¹ See cases by Leyden, Roth, Schultze, Clarke, Damaschino.

This case (1883) is also interesting in bringing out another lesion not usually noticed. This is the breaking up into balls of the myeline in the medullated tubes, both of the anterior intraspinal nerve-roots and of such fibres as traversed the anterior nerves. In balsam preparations these myeline drops are dissolved; but in osmic acid and glycerin preparations they appear as black balls all over the field. The lesion is identical with that already described by Dejerine (1875) in both nerves, roots, and white columns. The columns of Clarke have always been found intact.

In the recent cases no lesions of the white columns have been observed—a fact upon which reposes the doctrine that such lesions, when existing, are secondary to those of the cornua.

SECOND GROUP OF CASES.—The next group of cases contains 16, where the autopsy was made more than two years after the début of the paralysis.

TABLE VII.—AUTOPSIES SHOWING LESIONS OLDER THAN TWO YEARS.

Case No. Name of Author. Date of Lesion. Year of Publication.

11 Longet. 7 yrs. 1842

27 Echeverria. 7 yrs. 1866

43 Money. 7 yrs. 1884

16 Lancereaux. 16 yrs. 1873

38 Leyden. 17 yrs. 1876

33 Schultze. 19 yrs. 1876

17 Charcot and Joffroy. 25 yrs. 1870

20 Müller. 30 yrs. 1871

22 Clarke. 31 yrs. 1868

10 Hutin. 42 yrs. 1825

13 Cornil. 47 yrs. 1863

36 Leyden. 54 yrs.

34 Leyden. 58 yrs. 1876

19 Vulpian. 64 yrs. 1870

22 Raymond. 70 yrs. 1875

16 Prévost. 76 yrs. 1866

The date of these lesions varies from seven to seventy-six years. In two or three cases, where the autopsy was made on very old people, the early history of the disease was unknown, but the probable date of the paralysis was calculated.

In this group of cases patches of atrophy, semi-transparent and grayish in color, focal or diffused, are clearly perceptible to the naked eye. As a rule, the atrophy is unilateral, and sensibly affects the entire half of the cord. In some cases of paraplegia, however, there is a bilateral, symmetrical shrinkage of the entire lumbar cord, which has been reduced to the size of a quill.

The atrophy involves, first and most markedly, one or both anterior cornua; second, the anterior nerve-roots arising from them; third, the antero-lateral columns.

In both the latter localities the microscope will often find individual nerve-tubes wasted and deprived of their myeline. The atrophied patches are generally sclerosed as the seat of a proliferated neuroglia, coloring deeply with carmine. In Laborde's cases, published at the very beginning of what may be called the anatomical period, the atrophy and sclerosis were said to be limited to the antero-lateral columns and the nerve-roots, while the cornua remained intact. In all more recent observations, however, the lesion of the white columns and roots has been found strictly proportioned to that of the gray horns. The sclerosis extended into the latter, constituted by a reticulum of connective-tissue fibres, sometimes fine, sometimes so matted together as to form a dense felt-like substance, sometimes offering the ordinary aspect of sclerosis.

Amyloid corpuscles have been found infiltrated in great numbers through both the gray and white substance in these old cases (case by Cornil),¹⁰² seeming to replace the infiltration with exudation corpuscles observed in the more recent ones.¹⁰³ When the lesion is distinctly circumscribed the focus is often surrounded by a zone of embryonic cells, seeming to indicate a reactive proliferation on the periphery.¹⁰⁴

¹⁰² Loc. cit., Soc. Biol.,1863.

¹⁰³ Seguin (loc. cit.) observes that the opinion is gaining ground which ascribes these to a transformation of the neuroglia corpuscles.

¹⁰⁴ Case by Schultze, loc. cit.

As in the relatively fresh cases, the circumstance which has attracted the most attention is the atrophy of the ganglionic cells from the sclerosed patches of the anterior cornua. The completeness with which these have disappeared in any focus seems to be proportioned to the completeness of the paralysis in the corresponding limb. Partial atrophy or disappearance of spinal groups of cells from the cornua may sometimes be correlated with paralysis of special muscles.¹⁰⁵

¹⁰⁵ Thus in Schultze's case, already quoted, the external group of cells had disappeared from the focus in one gray horn, and the extensors of the foot were alone paralyzed. This seems to confirm the opinion advanced by Spitzka, that this external group of motor-cells corresponds to the extensor, the internal groups to the flexor muscles.

Study of the pathology of infantile paralysis is not completed when the above series of spinal lesions has been enumerated. Most various interpretations have been made of these lesions as they have been successively discovered. Thus, after the theory of congestion came the theory of primary sclerosis, built upon Laborde's two autopsies;¹⁰⁶ then the theory of primary atrophy of ganglionic cells;¹⁰⁷ then the theory of myelitis;¹⁰⁸ finally, a theory of complex and variable lesion.¹⁰⁹

¹⁰⁶ Laborde, loc. cit.; Cornil, loc. cit.

¹⁰⁷ Charcot, Leçons sur les Maladies du Syst. nerveux; Prévost, Soc. Biol., 1864; Joffroy, Arch. de Physiol., 1870; Petitfils, “De l'Atrophie aigue des Cellules matrices,” Thèse de Paris, 1873.

¹⁰⁸ Schultze, Virch. Arch., Bd. lxviii.; Roth, Ibid., Bd. lviii.; Henoch, loc. cit., p. 208; Ross, loc. cit., p. 125; Seguin, loc. cit., 1877; Erb, Ziemssen's Handbuch; Seeligmüller, Gerhardt's Handbuch; Roger and Damaschino, Gaz. méd., 1871; Turner, Path. Trans. Lond., 1879; Hammond, loc. cit.

¹⁰⁹ Leyden, Archiv für Psych., Bd. vi., 1876.

It was Prévost who first ascribed a predominant importance to the atrophy of the ganglionic cells of the anterior cornua; but it was in the hands of Vulpian, Joffroy, and more especially Charcot and his pupils, that the theory was fully developed. Infantile paralysis was ranked in a newly-formed group of diseases, all characterized by atrophy of these same cells, and differing from each other principally in the acuteness of the process and in its complications.¹¹⁰ Seguin, in his original lecture in 1874, supported the same views, but in 1877 fully adopted that of myelitis. The objections to this theory are: 1st, that by it two diseases so different in their course, localization, electrical reactions, and form of paralysis as atrophic paralysis and progressive muscular atrophy are essentially identified on account of the identity of one lesion, the atrophy of the anterior ganglionic cells;¹¹¹ 2d, the presence of other lesions or of traces of them peremptorily proves the pre-existence of a complex morbid process which involves the ganglionic cells, but is neither limited to them, nor, necessarily, originates in them.

¹¹⁰ Thus, acute anterior poliomyelitis, subacute anterior poliomyelitis, progressive muscular atrophy, amyotrophic lateral sclerosis, bulbar paralysis.

¹¹¹ When this objection is accepted, Barlow's remark falls to the ground, that “the similarity of lesion found in two such different diseases as infantile paralysis and progressive muscular atrophy proves the failure of anatomical characters, taken alone, to serve as a basis of nosology” (Brain, April, 1879, p. 74).

This inference was drawn by Roger from the hemorrhagic softening, dilatation, and degeneration of blood-vessels, infiltrations with exudation-corpuscles, and hyperplasia of conjunctive nuclei present in his case. Similarily, Schultze, in a case examined nineteen years after the occurrence of the paralysis, found traces of an extensive myelitis in the diffusion of the lesions,¹¹² in the exquisite cellular infiltration, the proliferation of the neuroglia, and the atrophy of axis-cylinders of nerve-fibres together with the cells; and inferred an anterior myelitis, diffused in the long axis of the cord, but limited to the antero-posterior region. Schultze defines Charcot's theory to be an hypothesis of such an acute atrophy of ganglionic cells as leads to a rapid melting down of these bodies, whereby reactionary inflammation is excited in the surrounding tissue. This implies that the dying cells are able to act like a virulent substance on the imbedding tissue, and of this, declares Schultze, “Charcot has offered no proof.”¹¹³

¹¹² In this case of paraplegia without lesion of the upper extremity, to which we have several times alluded, there was bilateral atrophy of the lumbar cord, atrophy of the right anterior nerve in the dorsal and lower cervical region, also in the cervical enlargement.

¹¹³ It might be said that the fall of the fever as soon as the paralysis is declared and the motor cells presumably melted down should contradict the idea that their dying substance acts as an irritant upon surrounding tissues.

A third objection has been brought forward by Leyden, and is really an enlargement on the second. It is, that various lesions or morbid processes may underlie the same clinical history. In four autopsies of cases presenting all the clinical history of acute anterior poliomyelitis this author has found three different lesions. In one an extensive lepto-meningitis, together with irregular focal sclerosis of the white columns, evidently depended upon the latter, and in turn caused sclerosis of the anterior cornua with consequent destruction of their cells.¹¹⁴ In two other cases an anterior poliomyelitis was accompanied by diffused lesions of the central canal. Finally, in a fourth case the lesions were limited to the anterior cornua, as is most usual.

¹¹⁴ This case of Leyden's throws light on the two autopsies by Laborde with sclerosis of the white columns and intact cornua. It seems probable that a process originating in the cornua had then been arrested or had receded, while continuing its evolution in the white columns.

The theory of acute atrophy of ganglionic cells is not sensibly different from that of a parenchymatous myelitis.¹¹⁵ But all the objections which can be urged against the former theory apply to the latter also, with the exception that the hypothesis of inflammation suggests a cause for the otherwise inexplicable atrophy. Observation of the pathological appearances alone could not decide whether the irritation started in the parenchymatous or interstitial tissues. Reference to the etiology of the disease shows that of the two most frequent apparent causes, blood-poisoning and traumatisms, the first would indicate that the inflammation started in the connective tissue supporting the blood-vessels; the second suggests that the irritation began in the spinal elements constituting the origin of the nerves.

¹¹⁵ Hammond assumes such a form of myelitis in his classification of inflammations limited to the anterior part of the gray matter of the spinal cord:

1. Inflammation of motor and trophic nerve-cells: (a) Infantile spinal paralysis; (b) Spinal paralysis of adults; (c) Pseudo-hypertrophic spinal paralysis.

2. Inflammation of motor cells: (a) Glosso-labia-laryngeal paralysis.

3. Inflammation of trophic cells: (a) Progressive muscular atrophy; (b) Progressive facial atrophy (Dis. Nerv. Syst., 6th ed., p. 464).

We think this classification open to several fundamental criticisms.

Whatever be the starting-point, however, it is very evident that the morbid process soon involves all the tissues contained in the gray matter of the anterior horns, and constitutes, therefore, a real anterior poliomyelitis.

A question of much interest is the relation to this of the lesions of the anterior roots and of the white columns. Is the atrophy of nerve-tubes a passive consequence of their separation from the ganglionic cells, the sclerosis a secondary consequence of this? or is the sclerosis the cause of the atrophy, itself the result of an irritation propagated downward from the myelitic focus, according to the usual law for secondary degenerations in motor tracts? or, finally, is it a residuum of a leucomyelitis (or of the white substance), complicating by simple extension the inflammation of the gray substance?

Review of the autopsies recorded would indicate that the lesions in question are brought about sometimes in one, sometimes in another, of these ways—sometimes even, as in Leyden's case, by extension from a meningitis. That sclerosis of the white columns is most frequently a secondary degeneration is indicated by the frequency with which it appears below the lesion of the cornua, by the rarity with which it is found above, and also by the general proportion between its intensity and that of the disease of the gray matter.

We have devoted so much space to consideration of spinal-cord lesions, because they are by far the most constant and the most important; after these rank the structural alterations of the muscles, which received for a while such a preponderance of attention.

Hammond has studied the progress of these changes on the living subject by fragments of fibre successively removed with Duchenne's harpoon. In an incipient stage of degeneration the fibrillæ are found to be irregular and torn,¹¹⁶ the transverse striæ dim; oil-globules are seen arranged according to the long axis of the fibre. In a more advanced stage the transverse striæ nearly disappear, the oil-globules are in large numbers, and fat-corpuscles are also abundant. Finally, the whole specimen is seen as a mass of air-globules. Six weeks later, however, these had in turn disappeared, and there remained a mass of connective tissue.

¹¹⁶ Though, from the method of removal, this appearance cannot be considered as certainly pathological.

This series of changes, however, does not always take place, as Hammond himself recognizes. Laborde¹¹⁷ first described a granular form of muscle atrophy, where the muscular substance gradually wastes away without ever becoming fatty, and leaving a transparent and hyaline sheath. The two forms of fatty and of simple atrophy can be distinguished by the naked eye. In the latter the muscle begins by being thinner or lighter and softer than usual, ultimately turning light brown. The fatty muscle becomes a homogeneous yellowish-white, diversified by occasional remnants of reddish fibres.

¹¹⁷ Loc. cit., p. 131.

Proliferation of the interstitial connective tissue may be combined with either simple or fatty atrophy. A combination of abundant sclerosis and abundant fatty infiltration may lead to a pseudo-hypertrophy of the muscles.

“There cannot be the slightest doubt,” observes Erb, “that the lesions described constitute a degenerative atrophy similar to what may be caused by section or sense traumatism of a peripheric nerve.”

The peripheric nerves have been much less thoroughly studied than the spinal cord. Leyden first directed special attention to the nerves. He found the sciatic altered in two cases,¹¹⁸ in the first by an interstitial neuritis; in the second by partial atrophy. In 1880 the same writer, in an extensive article on poliomyelitis and neuritis,¹¹⁹ greatly extends his views as earlier expressed. Not only does he claim the coexistence of neuritis with spinal-cord disease in atrophic paralysis, but thinks that many cases of this, and also of other forms of paralysis, “lately supposed to originate in the spinal cord, may really begin in any part of the motor apparatus,” thence sometimes generalize throughout the whole apparatus, sometimes remain limited to the original portion affected. Thus, progressive muscular atrophy may sometimes begin in the nerves, sometimes in the muscles, and sometimes in the ganglionic cells of the cord; and this variety of origin explains the discrepancies of opinion which have been held upon the nature of this disease. Similarly, all forms of acute or chronic atrophic paralysis in either children or adults may begin in either the nerves or cord, thence become generalized to both, or remain limited to one part of the spinal motor system. Cases of atrophic paralysis which recover are probably not cases of poliomyelitis at all, but of multiple neuritis, rheumatic, traumatic, or infectious in nature. The regeneration of peripheric nerves is a well-demonstrated possibility, but not that of the cells of the cord. Lead-paralysis is usually confined to the nerves, but sometimes extends to the cord. In diphtheritic paralysis Buhl has found injection, thickening, and granular infiltration of nerves at the union of their anterior and posterior roots;¹²⁰ and as long ago as 1876, Dejerine, in a case of atrophic paralysis in a syphilitic woman, found varicose swelling of the medullary sheath in the nerves of the paralyzed lower extremities, together with heaping up of the myeline into large drops, colored black in glycerin and osmic-acid preparations. Coincidently, in the cord, at the origin of the same nerves, the number of motor-cells was diminished, and of those that remained the prolongations, and even the body, of the cell were atrophied.¹²¹

¹¹⁸ Cases 34 and 35 of Table V., quoted from Arch. de Psychiatrie, Bd. vi., 1876.

¹¹⁹ Zeitschrift für Klin. Med., 1880.

¹²⁰ Zeitschrift für Biol., 1867.

¹²¹ Arch. de Phys., 1876.

These views of Leyden's are extremely interesting, and should stimulate future research into the condition of nerves in all cases of atrophic paralysis. It is quite incorrect to say, as Archambault and Damaschino have recently done,¹²² that Leyden denies the existence of anterior poliomyelitis in such cases, especially in such as prove permanent. He only insists on the frequent coincidence of neuritis, on a varying point of departure for the morbid process, and on the probability that in cases of recovery this process has always remained peripheric.

¹²² Le Union méd., 1883, 7, 35, case quoted in Table V. It is much to be regretted that Damaschino, who strongly controverts Leyden's views, did not examine the nerves in his own most interesting case.

The strongest objection to Leyden's theory is the absence in most recorded cases, either infantile or adult, of the usual signs of nerve inflammation, local pain, or tenderness. Autopsies of old cases are not able to differentiate an inflammation from an atrophic process in the nerves, followed by a secondary thickening of the endoneurium. This thickening was found in three cases examined by Edmonds in 1882, whose subjects had suffered from infantile paralysis in early life, and had had the paralyzed limb amputated at the age of fifteen or sixteen. Transverse sections were made from the internal popliteal nerves. The specimens showed some healthy nerve-fibres, presumed to be sensory; others much smaller, with the axis-cylinders wasted or degenerated; while strands of connective tissue traversed the nerve-bundles, resulting from hypertrophy of the endoneurium. The vessels showed inflammation of their coats, with proliferation of the endothelium.¹²³

¹²³ Trans. Path. Soc. London, 1883.

The brain is usually normal, unless indeed the paralysis has affected children previously rendered idiotic by congenital atrophia cerebri. Sandie, however, examined one brain with an interesting positive result.¹²⁴ The brain was taken from a boy of fifteen paralyzed since the age of three in almost all his muscles, with even paresis of the muscles of the trunk and neck. The paralysis was more marked upon the right than on the left side. At the autopsy, in addition to atrophy of the muscles and of the motor nerves, with exquisite atrophy of the anterior columns and anterior cornua, was found a decided atrophy of the left central convolution, and, less marked, of the paracentral lobule. This was shown by comparative measurements with the opposite side of the same brain, and also with the corresponding convolution and lobule in two other brains. The child's intelligence had not been affected.

¹²⁴ Centralblatt f. d. Med. Wissensch., No. 15, 1875.

The arrest of development of the bones has been already mentioned, as well as that of their epiphyses and apophyses. The compact osseous tissue is atrophied: the medullary, on the contrary, abundantly developed and rich in fat.

PATHOGENY OF INFANTILE PARALYSIS.—In the pathological anatomy of infantile paralysis there are two principal facts to be correlated with its clinical phenomena—namely, the limitation of the myelitis to the anterior gray horns of the spinal cord; the destruction of the ganglionic nerve-cells in these gray horns. That the other lesions observed are subordinate to these is shown by their variableness as compared with the constancy of the anterior poliomyelitis. These lesions are, in the cord, the atrophy and sclerosis of the anterior nerve-roots and white columns; in the muscle, the fatty degeneration or simple atrophy of the fibre; in the nerve, breaking down, and finally atrophy of the myeline sheath, sometimes of the axis-cylinder; proliferation of the endoneurium.

Consequence of Limitation of Myelitis.—Limitation of the morbid process to a portion of the motor tract, the anterior cornua, and exclusion of the posterior horns and roots, readily explain the predominant positive symptom of motor paralysis, together with the absence of sensory disturbance. The absence of muscular rigidity, spasm, active contraction, and of exaggerated reflexes is similarly explained by the immunity from the morbid process of the posterior white columns and the portion of the lateral columns immediately adjacent to them. The motor paralysis resulting from destruction of the anterior ganglionic cells of the cord is much more complete than that which depends on simple interruption of the motor tracts passing from the brain. The manner in which the motor tracts are connected by a succession of arching fibres with these cells already indicates that the latter are dépôts for the reinforcement of the motor impulses. We must believe, indeed, that the centrifugal impulses reaching the anterior cornua are not yet motor in character, but to become so must sustain a new elaboration in the ganglionic cells of this region. Evidently, the network of gray fibres connecting the arcuate strands of the antero-lateral columns with the cells become, in virtue of that fact alone, essential to the process. But it is also probable that the multiplied transmission of impressions, which lies perhaps at the basis of the process of their higher elaboration in ganglionic centres, is carried on in the larger network of gray fibres as well as in the smaller network contained in the ganglionic cells. Destruction of a portion of this network would therefore interfere with the elaboration of the motor impulse, in the same manner, though to a relatively less extent, as destruction of the ganglionic cells themselves.

Trophic Lesions.—The rapid wasting of the paralyzed muscles, with their degenerative electrical reactions, seems, however, to be an effect altogether peculiar to lesions of the ganglionic bodies.¹²⁵ According to Charcot, who has so especially formulated the laws of amyotrophic paralysis, all the ganglionic cells essential to the elaboration of motor impulses exercise a trophic influence upon muscles. The spinal cell, nerve-fibre, and muscle-fibre combine into a complex indissoluble unity or element. One part of this lesion of complex elements is necessarily followed by proportionate lesion of all its other parts.

¹²⁵ The amyotrophic lateral sclerosis of Charcot exhibits in an exquisite manner the difference between paralysis without atrophy, caused by sclerosis of the antero-lateral columns, and paralysis with atrophy when the morbid process has extended to the anterior cornua.

According to Erb, however, who extends Samuel's doctrine of special trophic nerves, it is not the motor cells which influence the nutrition of the muscle-fibres with which they are connected, but special trophic cells lying among the others in the anterior cornua. This theory is principally based on the existence of muscular atrophies of central origin (progressive muscular atrophy, bulbar paralysis), unaccompanied for a long time by paralysis.¹²⁶ Hammond cites as a converse example the anterior poliomyelitis “where the peripheric disturbance is, in the first place, solely one of motility; this is paralysis without atrophy. After a time, which may be as much as six months or even more, the trophic changes begin.”¹²⁷

¹²⁶ Ziemssen's Handbuch.

¹²⁷ Loc. cit., p. 429.

But surely this is an exaggerated emphasis on the exception, rather than the true inference from the rule of rapid wasting in anterior poliomyelitis—a rule so general as to have originated the title atrophic paralysis. Erb gives an ingenious scheme (Fig. 55) of the mental relations of motor and trophic cells with cerebral and spinal nerve-fibres. It will be seen that isolated lesions of one or the other trophic apparatus might occur without paralysis of motor tracts, while simultaneous lesion of the trophic apparatus and of the ganglion-cells, or of the latter, involving the tracts coming from the trophic cells, would cause, as in anterior poliomyelitis, motor paralysis, muscular atrophy, loss of the reflexes, degenerative reaction in nerves and muscles.

FIG. 55.

c, trophic cell for nerve; a, cerebral fibre; b, trophic cell for muscle; d, ganglionic cell; s, sensory fibre; f, trophic path to muscle; m, muscle. (From Ziemssen's Handbuch der Speciellen Pathol., Bd. xi. Zweite H., Zweite Abtheil, p. 313.)

Duchenne and Joffroy¹²⁸ also argue the existence of special trophic nerve-cells. The absence¹²⁹ of the nutritive lesions of the skin and cellular tissue which are so conspicuous when the gray matter around the central canal or posterior to it is involved,¹³⁰ the dependence of the nutrition of the motor apparatus, nerves, muscles, bones on the integrity of the anterior horns, are facts which, taken together, seem to indicate that the maintenance of nutrition depends on the unbroken continuity of the motor or sensory apparatus from the periphery to the ultimate central element, rather than on any special central cells endowed with trophic functions.¹³¹ Erb's hypothesis, as his own scheme moreover denotes, demands not only trophic cells distinct from motor cells, but separate trophic cells for the muscles, for the motor, and for the sensory nerves.

¹²⁸ “De l'Atrophie aigue et chronique des Cellules nerveuses,” Arch. de Phys., No. 4, 1870.

¹²⁹ Money, and also Gowers, have signalized a condition of the skin resembling myxœdema (Tr. Path. Soc. London, 1884, and Brit. Med. Journ., 1879).

¹³⁰ Mayer (Herman's Handbuch Physiol.) sums up the great mass of evidence now accumulated, which demonstrates the trophic influence of the central gray mass of the cord upon the tissue.

¹³¹ Nepveu (La France médicale, 1879) mentions some cases of infantile paralysis complicated with trophic lesions of the skin. The facts, if accepted, could only indicate an extension of the myelitis to the central and posterior regions of the gray columns. The relations between non-atrophic paralysis caused by interruptions of the motor tracts and muscular atrophy dependent on lesion of the anterior cornua are exquisitely shown in a case reported by Sander. An adult suffered from chronic motor paralysis, gradually increasing, in the right arm, with paresis of the lower extremities. In the hand, arm, and shoulder the paralysis was followed by gradual atrophy and diminution of the faradic contractility; in the lower extremities no atrophy occurred. At the autopsy was found a gliomatous tumor seated in the anterior cornua predominating on the right side, extending from the level of the sixth dorsal to that of the eighth cervical vertebra. The ganglion-cells were pigmented and compressed, not altogether destroyed. The lumbar cord was intact, and the non-atrophic paresis of the lower extremities evidently resulted from the interruption of the motor tract above.

The peculiar grouping of nerve-centres within the cord that seems to be indicated by some of the groupings of infantile paralysis shows, as has been said, a probable divergence within the cord of nerve-fibres which run together in the same nerve-stem. The associations to be expected from the data of functional association and of clinical history are by no means fully decided. It is even a matter of dispute whether the tibialis anticus is functionally more associated with the flexors or with the extensors of the thigh, and whether its experimental irritation or clinical paralysis really coincides with that of the first or of the second group. This entire field of observation is new and promises fertile results.¹³²

¹³² It is from this field that has come a new argument for the spinal nature of lead-paralysis, from its peculiar grouping, and from analogy with that of anterior poliomyelitis of the upper extremities (Remak, “Ueber die Local. Atropa. Spinal Lahm.,” Archiv für Psych., Bd. ix.; also, Ferrier, loc. cit.).

Relation between Limitation of Myelitis and Age.—From the relative frequency of anterior poliomyelitis in childhood, as compared with its much greater rarity in adult life, we must infer the existence of some special conditions in childhood which tend to limit the morbid process to such a portion of the cord. The theory of a primitive spontaneous atrophy of the motor cells would serve, indeed, to explain this limitation. The reasons already alleged for regarding the morbid process as a systematic myelitis decisively hinder the acceptance of such an explanation as it stands. On the assumption, however, that the myelitis is usually of functional origin, and starts, therefore, in the elements of the anterior cornua essentially involved in the motor functions, the morbid susceptibility of these elements may be ranked with the liability to disease of the entire locomotor system which is known to be so predominant in children. From pathological evidence, even without anatomical proof, we may reasonably infer an incompleteness of development in the anterior cornua of the cord correlative with that well demonstrated in the bones and functionally inexperienced muscles. If the antero-posterior fibres which connect the anterior cornua with the central and posterior gray masses be also incomplete, the radiation of irritations, and consequent vascular irritation, would also be arrested within the boundaries of the original lesion. Thus a peculiarly circumscribed, instead of the common diffused, myelitis of adults.

Money¹³³ points out that for the gray matter of the cord, as of the brain, the centre or maximum force of the circulation is on the periphery, and the nutritive supply of the centre is thus easily cut off. Moreover, while the blood-vessels of the cervical and dorsal regions of the cord pass to it transversely, the vessels of the lumbar region are compelled to describe a somewhat prolonged vertical course before reaching their point of distribution. From these circumstances, even transitory congestions in the circulation of the cord are easily followed by irreparable injury of its delicate elements.

¹³³ Loc. cit., Path. Trans., 1884.

Finally, in all discussions on pathogeny must not be forgotten the doctrine of Leyden¹³⁴ that infantile paralysis, also progressive muscular atrophy, is a disease which may begin at the periphery and extend to the centres, as well as the reverse. It must also be remembered that, as yet, only very scanty evidence exists to support this, in itself, plausible theory.

¹³⁴ See loc. cit., ut supra.

COURSE OF INFANTILE PARALYSIS.—The most ordinary course of infantile paralysis is that already described as typical—namely, extremely rapid development to a maximum degree of intensity, then apparent convalescence, retrocession of paralysis, atrophy, and ultimate deformities in limbs in which paralysis persists.

Several variations from this typical course are observed. Complete recovery may take place, as in the so-called temporary paralysis of Kennedy¹³⁵ and of Frey.¹³⁶ These cases are very rare. But their possibility seriously complicates the estimate we may make of the efficacy of therapeutic measures.¹³⁷

¹³⁵ Dublin Quarterly Journal, 1840.

¹³⁶ Berlin. Klin. Wochensch., 1874. I have described one such temporary case in the article already quoted. These cases seem about as frequent in adults. (See Frey, loc. cit.; also case of Miles, etc. etc.)

¹³⁷ As of the case of complete recovery, the only one the author had seen, related by Dally, Journal de Thérap., 1880, 1, vii.

On the other hand, there may be a complete absence of regression; and this is observed sometimes in cases where the paralysis is originally limited; sometimes where it is extremely extensive, involving nearly all the muscles of the trunk or limbs;¹³⁸ or muscles or limbs originally spared may become involved in a fresh attack. Laborde relates cases of this kind. In Roger's first case paraplegia occurred under the influence of scarlatina two months after paralysis of one arm.

¹³⁸ Thus in Eulenburg's case, quoted ut supra.

The form of anterior poliomyelitis most frequent in adults is the subacute, and after that the chronic. Both are extremely rare in children, the latter excessively so. Seeligmüller and Seguin¹³⁹ both admit the possibility of a chronic form in children, and the latter has kindly communicated to me one case from his private practice:

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.

¹³⁹ Loc. cit. (ed. 1877).

Erb¹⁴⁰ relates 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

¹⁴⁰ Brain, 1883.

¹⁴¹ In the same number of Brain, 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 WITH PROGRESSIVE MUSCULAR ATROPHY.—Raymond¹⁴² and 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.¹⁴³ Similar 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.)

¹⁴² Gaz. méd., 1875. No. 17.

¹⁴³ It 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.”¹⁴⁴ This encouraging statement must be read as applying rather to individual muscles than to cases as a whole. Few complete 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.

¹⁴⁴ Loc. 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

¹⁴⁵ Loc. 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

¹⁴⁶ Loc. cit.

Volkmann¹⁴⁷ considers the paralysis entirely hopeless, and advises the concentration of all effort upon the prevention or palliation of deformities.

¹⁴⁷ Loc. 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.

SPECIAL PARALYSES.—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.

Ballet¹⁴⁸ has 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 one related by Dejerine in 1882.¹⁴⁹ The 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.

¹⁴⁸ Revue de Médecine, 1884.

¹⁴⁹ Revue de Médecine, 1882.

The observations of progressive muscular atrophy in persons bearing the stigmata of an infantile paralysis are quite numerous.¹⁵⁰

¹⁵⁰ Charcot, Soc. Biol., 1875, and Gaz. méd.; Seeligmüller (4 cases), in Gerhardt'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.