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.