Chapter L: J. Lautenbach, in a recent communication to the Philadelphia (5)

[Footnote 15: _De la Paralysie de l'Enfance_.]

The duration of the fever usually varies from a single night to forty-eight hours; much more rarely does it last six, eight, twelve, or fourteen days, or even, but quite exceptionally, three or four weeks. According to Duchenne, its intensity and duration increase with the age of the child, perhaps indicating greater resistance on the part of the nerve-tissues which are the seat of the morbid process of which it is symptomatic. Rarely does it last after the paralysis has once occurred, {1116} but ceases then with an abruptness which recalls the defervescence of pneumonia when the exudation process is once completed.[16]

[Footnote 16: See p. 1144 for pathogenic inferences to be drawn from this fact. Seguin (_New York Med. Record_, Jan. 15, 1874) seems to throw some doubt on the existence of apyretic cases; but, as Seeligmüller remarks, there is too much testimony to this possibility to render it really doubtful.]

There is no proportion between the intensity of the fever and the extent of the subsequent paralysis; nor is there any marked contrast between the fever in children and that in adults in those rare cases in which the disease, instead of being subacute, is sudden as in children.

Erb considers the fever to be purely symptomatic of an inflammatory process in the spinal cord.[17] But Vogt regards it rather as an essential factor in the development of a spinal lesion, and thus explains the occurrence of this in the course of febrile diseases which at the outset have no special relation to the cord.

[Footnote 17: _Loc. cit._, p. 279.]

Convulsions, usually accompanied by fever, were observed in 11 of Seeligmüller's cases out of 67; Duchenne had 13 out of 70; Heine, 9 out of 86;[18] thus a total of 33 cases of convulsions in 223 cases of infantile paralysis—nearly 15 per cent. The paralysis may set in after a single brief convulsion, or this may be repeated several times at variable intervals before the paralysis is definitely declared (Ross).[19] The convulsive movements are apt to be particularly intense in the limbs destined to become paralyzed (Vogt).

[Footnote 18: _Die Spinale Kinderlahmung_.]

[Footnote 19: _Loc. cit._, p. 107. The author is quoting Laborde.]

The convulsion may be very slight—an isolated spasm of a limb or even a single group of muscles. Whether, on the other hand, it can ever be so intense that the child succumbs to it before the development of paralysis, is a question which could only be decided by repeatedly examining the cord in the cases of convulsion which have terminated fatally. In a case of Seeligmüller's the child was affected for eight days preceding the paralysis by tremblings generalized through all his muscles.

The convulsion is usually followed by a soporous or even comatose condition, or this may replace the convulsion. Delirium may take the place of either.

Special interest attaches to those cases where the paralysis develops in the course of an acute specific disease; for then becomes most plausible the suggestion of Vogt, that a fever excited by some cause remote from the spinal cord may itself become a cause of lesion in this centre. In Roger's first and most celebrated case, paraplegia developed suddenly during the course of a fatal scarlatina in a child already suffering from paralysis of the left deltoid of two months' standing.[20] The scarlatina was hemorrhagic, and, as will be shown farther on, the autopsy showed traces of a hemorrhagic extravasation in the cord. Thus a double influence was presumably exerted by the scarlatina, while, moreover, the previous and recent occurrence of a deltoid paralysis indicated a morbid predisposition in the spinal cord. Of Seeligmüller's 75 cases, 1 occurred during scarlet fever, 1 with measles, 1 in the course of an erysipelas, and 1 of pneumonia.

[Footnote 20: _Gaz. méd._, 1871.]

Apyretic diseases, especially of the gastro-intestinal tract (Brown-Séquard), also seem to have an influence on the development of infantile paralysis. Two of my own cases occurred during an attack of {1117} cholera infantum; another in a child who had been for several weeks in bed with a purulent conjunctivitis. Study of these varied antecedents is of interest in connection with the obscure question of the etiology of infantile paralysis. In this latter connection we will refer to them again.

Vomiting, or even the entire symptom-complex of gastric fever, not infrequently ushers in the paralysis. Fever is then usually present, but I have recorded one case of vomiting where, according to the mother's assurance, no fever at all existed.

At the moment that the symptoms of the invasion subside, and the child seems to enter upon convalescence, the terrifying discovery is made that an arm or a leg or all four limbs, or even they and the muscles of the trunk, are paralyzed.

In the severest form the child lies motionless, unable to stir hand or foot, or even a finger or toe. Yet, singularly enough, this extensive paralysis is sometimes overlooked, especially in very young children, as the immobility of the patient is attributed merely to weakness caused by previous illness. General paralysis, during at least the first few hours of the paralytic stage, is probably more common than appears from our present statistics. Not only, as has just been noted, may this condition be overlooked, but it may exist during the hours of sleep which precede the cases of morning paralysis. Seguin[21] speaks as if the paralysis were at first always generalized, but this statement seems to me somewhat exaggerated. Referring merely to the statements of the parents, a considerable number of paralyses would be found limited from the beginning. Heine's third table of partial paralysis is entirely composed of cases so limited. In 16 out of the 19 cases of hemiplegia (monoplegia) the original limitation of the paralysis is also specified; similarly with 7 out of the 20 cases of paraplegia contained in the first table.

[Footnote 21: _Loc. cit._]

Paralysis of one or both lower extremities is often first detected when the child gets out of bed and attempts to walk; or in children too young to walk the flaccid immobility of the limb attracts attention as soon as they are again carried on the mother's arm. Paralysis of the upper extremities is discovered early in proportion to the liveliness recovered by the child, leading him to occupy himself with his toys as usual. In unilateral paralysis of the trunk the child will fall over to one side when placed in the sitting position; in bilateral paralysis it cannot be made to sit up at all.

From lack of competent observation during the initial stage it is really not quite certain whether any degree of paresis precedes the paralysis; but from the testimony at present accumulated the paralysis is nearly always complete when first observed. This is in striking contrast with adult spinal paralysis. In some few cases the paralysis has been observed to creep on slowly, and not reach its maximum for several days (Ross). Laborde relates a case where recovery from a first attack of paralysis was followed by two relapses in the same limbs at intervals, each ushered in by fever. After the second relapse the paralysis remained permanent.

Significance of Original Extent of Paralysis.—The question of the {1118} original distribution of the paralysis is of special interest in connection with that of the original distribution of the morbid process in the spinal cord. The real effect of the latter cannot be adequately measured by the permanent paralyses; for, as will be seen, it is not unusual to find traces of an extensively diffused process in the cord in cases of quite partial paralysis.

It is interesting to notice that certain muscles are always exempt from paralysis. With the exception of a single case of paralysis of one temporal muscle, cited by Seguin,[22] the muscles of the head, eyeballs, ears, larynx, and pharynx are always exempt, as are also the diaphragm and intercostals. The arrest of the spinal lesion below the medulla explains the immunity of muscles supplied by the vagus and spinal accessory nerves. But since the cervical plexus is often involved, the constant escape of the diaphragm, innervated by the phrenic nerve which comes from this plexus, is remarkable. Still more so the immunity of the intercostal muscles, whose nerves arise in the dorsal region—a position of the cord frequently affected. This fact tends to confirm Ross's hypothesis, that the nuclei of the intercostal nerves lie in the vesicular columns of Clarke—columns confined to the dorsal region of the cord, and which are invariably found intact at autopsies of atrophic paralysis.

[Footnote 22: _Loc. cit._]

The immunity of these respiratory nerves explains the absence of the dyspnœa which is so marked in Landry's ascending paralysis. In the adult case described by Schultze and Erb[23] dyspnœa was present for a short time. The disease terminated fatally twenty months from the time of invasion. In this case traces of myelitis were found extending through the dorsal region of the cord, and including not only the anterior nerves, but, to a less degree, the columns of Clarke.

[Footnote 23: _Arch. Virch._, Bd. lxviii.]

The facial nerve (itself a respiratory nerve) shares the immunity of the phrenic and intercostals. In the cases in which facial paralysis has been noted the limb paralysis has been hemiplegic, as in Seeligmüller's twentieth case. A cerebral origin is then always to be at least suspected.

Barlow[24] has seen 6 cases of paralysis of the facial, but the histories render a cerebral paralysis more probable in 4 out of those 6. Henoch[25] gives a case of paralysis of left arm, accompanied by paralysis of corresponding facial nerve. The latter rapidly recovered, but the paralysis of the arm persisted and was followed by atrophy. Ross[26] implies that the sides of the neck, face, and tongue are always at first implicated in spinal hemiplegic paralysis, but do not remain permanently affected.

[Footnote 24: _Loc. cit._]

[Footnote 25: _Loc. cit._, p. 205.]

[Footnote 26: _Loc. cit._, p. 108.]

That the facial should be affected while the other medullary nerves escape probably depends on the more anterior position of its nucleus.

The regression of the original paralysis is characteristic, indeed almost pathognomonic, of the disease. It is on this account that Barlow has proposed the name regressive paralysis.[27] This author quotes the case of a boy who at five months was affected with a universal paralysis, even affecting the neck, but entirely recovered except in the extensor longus digitorum of the foot. This improvement constitutes a second apparent convalescence, as deceptive as that which immediately succeeds the pyrexia. Only in rare cases do all the {1119} muscles at first paralyzed remain so permanently (Seeligmüller); nor, on the other hand, do all entirely recover (temporary paralysis of Kennedy and Frey). Even when an entire limb appears to be paralyzed, careful examination will usually detect certain muscles that retain their faradic contractility. Thus the order of frequency of paralysis in the different limbs must be distinguished from that observed for different muscles.

[Footnote 27: _Brit. Med. Journ._, 1882.]

Duchenne fils[28] and Seeligmüller[29] have tabulated, for lists of 62 and 75 cases respectively, the general locality of the permanent paralyses in their order of frequency. The cases of monoplegia are by far the most numerous. Thus in the table quoted below there are 97; in Heine's tables (86 cases), 47; in Sinkler's tables (86 cases), 29—total, 173 from a total of 309, or nearly one-half:

Duchenne. Seeligmüller. Total.
Left lower extremity 7 27 34
Right " " 25 15 40
Right upper " | 10 13 |9 23
Left " " | |4
All four extremities 5 2 7
Both upper " 2 1 3
Both lower " 9 14 23
Left over and under extremity 1 1 2
Right " " " 0 1 1
Right over and left under " 2 1 3
Muscles of trunk and abdomen 1 0 1
-- -- ---
62 75 137

This limitation is all the more noteworthy when compared with the frequency of general paralysis at the outset.

[Footnote 28: _Archives gén._, 1864.]

[Footnote 29: _Jahrbuch der Kinderheilkunde_, N. H. xii. pp. 338-343.]

The next peculiarity is the great preponderance of paralysis of the lower over that of the upper extremities. This is noticeable even in the monoplegias. In Sinkler's cases only two of these affected an arm. But in bilateral paralysis the predilection is still more remarkable, paraplegia of the lower extremities being among the most frequent, paraplegia cervicalis the rarest, form of paralysis. This is not because a lesion situated in the cervical spinal cord must interfere with the motor tract going toward the lumbar, and hence nearly always paralyze all four extremities, if any. Because when this does happen the upper extremities alone exhibit the atrophic changes characteristic of anterior poliomyelitis: the lower, though paralyzed, do not atrophy and retain their faradic contractility. Heine denied the existence of paraplegia cervicalis. But in the table of Duchenne-Seeligmüller 3 cases are recorded; Rosenthal[30] relates 1; Lockhart Clarke, 1.[31] This is the somewhat famous case, described by Clarke as a progressive muscular atrophy, which contributed one of the earlier autopsies.

[Footnote 30: _Klinik der Nervenkrankheiten_, 2 Aufl. p. 413.]

[Footnote 31: _Med.-Chir. Trans._, li. p. 219.]

Seeligmüller's case[32] is remarkable in several respects. The paralysis, occurring after a brief fever in a child seventeen months old, exclusively attacked the two arms at the moment of invasion, and never retreated from them, thus offering a double exception to the usual rule. At four rears of age the arms were much atrophied, and faradic contractility was lost in their muscles, the legs being sturdily developed. Nevertheless, {1120} the child constantly fell in walking, because, observes Seeligmüller, he was unable to balance himself with his arms, as is habitual with little children learning to walk.

[Footnote 32: _Jahrbuch, loc. cit._, p. 349.]

The hemiplegic variety of paralysis is again very rare. The Duchenne-Seeligmüller table contains (out of 137) 3 cases; Sinkler records (out of 86) 4; West, 5; Heine (out of 86), 1; Leyden, 1; Duchenne, 1. West's 5 cases all present certain peculiarities, at least unusual in spinal paralysis. In 2 the paralysis came out gradually; in 1 succeeded to remittent fever (pigmentary embolism?); in 1 was preceded by heaviness of the head for several days; and in 1 the leg was paralyzed fourteen days after the arm.[33] It is probable that in almost all, if not in all cases, hemiplegic spinal paralysis is the residue of a paralysis originally generalized to all four limbs, if only for a few hours.[34]

[Footnote 33: In three cases of hemiplegia observed by myself, and previously diagnosed as spinal paralysis by other physicians, I doubted the diagnosis from the coincidence of unusual cerebral symptoms. In the first case the hemiplegia appeared after coma, during cerebro-spinal meningitis; in the second, after a violent convulsion the face was drawn to the opposite side, and the patient, a child of seven, remained for a month in a state of intense maniacal excitement. In a third case, developed during convalescence from scarlet fever, the hemiplegia was preceded during two days by hemiparesis, and accompanied for a year by complete aphasia. Finally, in these cases faradic contractility persisted in the paralyzed limbs (_Am. Journ. Obstet._, May, 1874).]

[Footnote 34: Seeligmüller relates one case where hemiplegia, including the facial nerve, was observed in two days from the beginning of the fever.]

The question of hemiplegia is closely connected with that of paralysis of the facial nerve, inasmuch as the existence of the latter often serves to suggest a cerebral paralysis—a suggestion confirmed later by the absence of atrophy and of characteristic electrical reactions. However, in some cases of undoubted spinal paralysis the facial does really seem to have become involved. Thus in the case just quoted from Seeligmüller (Case 20 of his table) Henoch[35] relates a case of paralysis of the left facial coinciding with paralysis of the left arm. Rapid recovery from facial paralysis: arm atrophied. Barlow[36] records temporary facial paralysis in six cases, but only two of these seem to be really spinal. Such temporary paralysis is not altogether infrequent in the poliomyelitis anterior of adults (Sinkler, Seguin). Ross[37] implies that the sides of the neck, face, and tongue are always implicated at first in hemiplegic spinal paralysis, but do not remain so.

[Footnote 35: _Loc. cit._, p. 203.]

[Footnote 36: _Loc. cit._, p. 76.]

[Footnote 37: _Loc. cit._, p. 108.]

Crossed paralysis is extremely rare. There are 3 cases in the Duchenne-Seeligmüller table; Leyden[38] has one. But paraplegia of the lower extremities, coinciding with paralysis of one upper extremity, is by no means so rare, especially as a residual paralysis.

[Footnote 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. Eulenburg[39] 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.

[Footnote 39: _Arch. Virch._, Bd. xvii., 1859.]

{1121} Birdsall[40] has described one case of unilateral paralysis of the abdominal muscles.

[Footnote 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]

[Footnote 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.]

[Footnote 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]

[Footnote 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 {1122} 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]

[Footnote 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).]

[Footnote 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]

[Footnote 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 {1123} 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]

[Footnote 47: Obs. 14, 15, 16, 17, from Remak's essay.]

[Footnote 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.

[Footnote 49: Thus in Cornil's famous case, _Soc Biol._, 1863.]

The following table sums up some special diagnostic marks for the different paralyses[50] 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. {1124}

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.

[Footnote 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.

[Footnote 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).

[Footnote 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]

[Footnote 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 {1125} 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.

[Footnote 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.

[Footnote 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]

[Footnote 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 {1126} 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.

[Footnote 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.)]

[Footnote 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]

[Footnote 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 intact[60] 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.

[Footnote 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.]

{1127} 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 development[61] by being easily reducible. This remark especially applies to paralytic club-foot:

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

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

| Luxation.
Hip. | Permanent flexion.
| Permanent adduction.

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

Elbow. | No deformity (Seeligmüller).

Shoulder. | Subluxation humeri.

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

[Footnote 61: _Volkmann's Handbuch_, Billroth und Pitha.]

{1128} 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.

[Footnote 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. Werner[63] first protested against this theory, and the protest has been further developed and a different theory built up through the efforts of Hueter[64] 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.

[Footnote 63: _Reform der Orthopædie_, 1845.]

[Footnote 64: _Gelenkkrankheiten_.]

[Footnote 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 {1129} 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.

[Footnote 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.]

{1130} 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. Reclus[67] 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]

[Footnote 67: _Revue mensuelle de Méd. et de Chir._, Mars, 1878.]

[Footnote 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 {1131} 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]

[Footnote 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.