Initial Stage.—Seeligmüller6signalizes three principal varieties in this stage. The first is characterized by fever; the second by nervous symptoms, principally convulsions, sometimes delirium or coma; in the third no symptoms either precede or accompany the local disease—"la paralysie est toute la maladie.”7
6Gerhardt's Handbuch der Kinderkrankheiten, 1880 (separat Abdruck).
7Rilliet et Barthez,Traité des Maladies des Enfants, ii. p. 551.
Mode of Invasion.—I have elsewhere8described eight different modes of invasion: absolutely sudden, coming on in the daytime; morning paralysis (West), discovered after a quiet night, preceded by fever or by vomiting alone (?), or by another typical disease, especially one of the exanthemata, or, finally, by a traumatism, generally slight. An interval of time almost always elapses between the occurrence of the traumatism and the development of the paralysis—a fact which already indicates that a definitely-evolved morbid process must intervene between the two occurrences. An exception is related by Duchenne fils;9and some apparent exceptions, in which recovery occurred rapidly, seem to belong to the temporary paralysis of Kennedy,10more recently described again by Frey.11
8Am. Journ. Obstet., May, 1874.
9Archives gén., 1864. A father pulled his child from a table by the right arm, and set it rather roughly on the ground. Immediate pain, almost immediate paralysis of arm, which persisted, and was followed by atrophy of its muscles.
10Dublin Quarterly, 1850.
11Berlin. klin. Wochens., 1874. Frey considers these cases to be identical in nature with, though differing in severity from, anterior poliomyelitis.
PRODROMATA.—There is rarely any lengthened period of prodromata. Seeligmüller has noticed in some cases an indisposition on the part of the child to stand or walk during several weeks before the occurrence of the paralysis. He does not say whether such children were rachitical. In marked contrast with cases of cerebral paralysis is the habitual absence of generalized nervous symptoms. Thus in only 1 case of Seeligmüller's (total of 75) did the child suffer, and that during six months preceding the paralysis, from intermittent muscular contractions, and also from attacks of laryngismus stridulus.
The fever is usually of moderate severity (Seeligmüller), but sometimes extremely high (Erb)12—as much as 41° C. (Henoch),13or 104° F. (Barlow).14Duchenne fils observed 7 cases alleged to be entirely without fever, and Laborde counts 10 cases out of 50 as apyretic.15
12Ziemssen's Handbuch, Bd. xi. Abh. 12.
13Vorlesung. über Kinderkrank., 2d Aufl., 1881. Seeligmüller (Jahrb. für Kinderheilk., 1878, p. 345) quotes another case from Henoch's clinic where the fever lasted thirty-six hours, the temperature on the first evening being 39.2°, the following morning 39.0°, the second evening 39.5°; the second morning, at which date the paralysis was discovered, it was normal. The author states this to be the only case known to him in which thermometric measurements were taken.
14Loc. cit.
15De 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,but ceases then with an abruptness which recalls the defervescence of pneumonia when the exudation process is once completed.16
16See p.1144for 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.17But 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.
17Loc. 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;18thus 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).19The convulsive movements are apt to be particularly intense in the limbs destined to become paralyzed (Vogt).
18Die Spinale Kinderlahmung.
19Loc. 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.20The 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.
20Gaz. 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 ofcholera 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. Seguin21speaks 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.
21Loc. 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 theoriginal 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,22the 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.
22Loc. 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 Erb23dyspnœa 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.
23Arch. 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.
Barlow24has seen 6 cases of paralysis of the facial, but the histories render a cerebral paralysis more probable in 4 out of those 6. Henoch25gives 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. Ross26implies that the sides of the neck, face, and tongue are always at first implicated in spinal hemiplegic paralysis, but do not remain permanently affected.
24Loc. cit.
25Loc. cit., p. 205.
26Loc. 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.27This 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 themuscles 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.
27Brit. Med. Journ., 1882.
Duchenne fils28and Seeligmüller29have 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:
This limitation is all the more noteworthy when compared with the frequency of general paralysis at the outset.
28Archives gén., 1864.
29Jahrbuch 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; Rosenthal30relates 1; Lockhart Clarke, 1.31This is the somewhat famous case, described by Clarke as a progressive muscular atrophy, which contributed one of the earlier autopsies.
30Klinik der Nervenkrankheiten, 2 Aufl. p. 413.
31Med.-Chir. Trans., li. p. 219.
Seeligmüller's case32is 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,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.
32Jahrbuch, 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.33It 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
33In 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).
34Seeligmüller 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) Henoch35relates a case of paralysis of the left facial coinciding with paralysis of the left arm. Rapid recovery from facial paralysis: arm atrophied. Barlow36records 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). Ross37implies that the sides of the neck, face, and tongue are always implicated at first in hemiplegic spinal paralysis, but do not remain so.
35Loc. cit., p. 203.
36Loc. cit., p. 76.
37Loc. cit., p. 108.
Crossed paralysis is extremely rare. There are 3 cases in the Duchenne-Seeligmüller table; Leyden38has 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.
38Archiv 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. Eulenburg39relates 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.
39Arch. Virch., Bd. xvii., 1859.
Birdsall40has described one case of unilateral paralysis of the abdominal muscles.
40Journal 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.41Several 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
41Ernst Remak, “Localis. der Atroph. lahmung,”Archiv f. Psych., ix., 1879; Ferrier,Brain, vol. iv. No. 3; also,Proceedings Royal Society, No. 212, p. 12.
42The 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
43Loc. 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 givesresults 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.44The 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
44It 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).
45The 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
46Thus 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 thesartorius 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.47And 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
47Obs. 14, 15, 16, 17, from Remak's essay.
48At 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 OFSPECIALPARALYSIS.—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.49On 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.
49Thus in Cornil's famous case,Soc Biol., 1863.
The following table sums up some special diagnostic marks for the different paralyses50afforded 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.
50See 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,51unless, 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.
51Except 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.52The 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).
52Seeligmüller,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
53Ch. 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.54Hammond relates a case where the local temperature was 75° in anatmosphere 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.
54Loc. 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.55This 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.
55Charcot 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
56Simon 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.57The author remarks that the persistence of galvanic reaction after the complete loss of faradic contractility is completely analogous to anobservation of Brucke's on muscles poisoned by woorara,58where 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.
57Jahrb. 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
59Buzzard 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 intact60and 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.
60Practically, 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.