most recorded cases, either infantile or adult, of the usual signs of
nerve inflammation, local pain, or tenderness. Autopsies of old cases
are not able to differentiate an inflammation from an atrophic process
in the nerves, followed by a secondary thickening of the endoneurium.
This thickening was found in three cases examined by Edmonds in 1882,
whose subjects had suffered from infantile paralysis in early life,
and had had the paralyzed limb amputated at the age of fifteen or
sixteen. Transverse sections were made from the internal popliteal
nerves. The specimens showed some healthy nerve-fibres, presumed to be
sensory; others much smaller, with the axis-cylinders wasted or
degenerated; while strands of connective tissue traversed the
nerve-bundles, resulting from hypertrophy of the endoneurium. The
vessels showed inflammation of their coats, with proliferation of the
endothelium.[123]
[Footnote 123: _Trans. Path. Soc. London_, 1883.]
The brain is usually normal, unless indeed the paralysis has affected
children previously rendered idiotic by congenital atrophia cerebri.
Sandie, however, examined one brain with an interesting positive
result.[124] The brain was taken from a boy of fifteen paralyzed since
the age of three in almost all his muscles, with even paresis of the
muscles of the trunk and neck. The paralysis was more marked upon the
right than on the left side. At the autopsy, in addition to atrophy of
the muscles and of the motor nerves, with exquisite atrophy of the
anterior columns and anterior cornua, was found a decided atrophy of
the left central convolution, and, less marked, of the paracentral
lobule. This was shown by comparative measurements with the opposite
side of the same brain, and also with the corresponding convolution
and lobule in two other brains. The child's intelligence had not been
affected.
[Footnote 124: _Centralblatt f. d. Med. Wissensch._, No. 15, 1875.]
The arrest of development of the bones has been already mentioned, as
well as that of their epiphyses and apophyses. The compact osseous
tissue is atrophied: the medullary, on the contrary, abundantly
developed and rich in fat.
PATHOGENY OF INFANTILE PARALYSIS.—In the pathological anatomy of
infantile paralysis there are two principal facts to be correlated
with its clinical phenomena—namely, the limitation of the myelitis to
the anterior gray horns of the spinal cord; the destruction of the
ganglionic nerve-cells in these gray horns. That the other lesions
observed are subordinate to these is shown by their variableness as
compared with the constancy of the anterior poliomyelitis. These
lesions are, in the cord, the atrophy and sclerosis of the anterior
nerve-roots and white columns; in the muscle, the fatty degeneration
or simple atrophy of the fibre; in the nerve, breaking down, and
finally atrophy of the myeline sheath, sometimes of the axis-cylinder;
proliferation of the endoneurium.
Consequence of Limitation of Myelitis.—Limitation of the morbid
process to a portion of the motor tract, the anterior cornua, and
exclusion of the posterior horns and roots, readily explain the
predominant positive symptom of motor paralysis, together with the
absence of sensory disturbance. The absence of muscular rigidity,
spasm, active contraction, and of exaggerated reflexes is similarly
explained by the immunity from the morbid process of the posterior
white columns and the portion of the {1145} lateral columns
immediately adjacent to them. The motor paralysis resulting from
destruction of the anterior ganglionic cells of the cord is much more
complete than that which depends on simple interruption of the motor
tracts passing from the brain. The manner in which the motor tracts
are connected by a succession of arching fibres with these cells
already indicates that the latter are dépôts for the reinforcement of
the motor impulses. We must believe, indeed, that the centrifugal
impulses reaching the anterior cornua are not yet motor in character,
but to become so must sustain a new elaboration in the ganglionic
cells of this region. Evidently, the network of gray fibres connecting
the arcuate strands of the antero-lateral columns with the cells
become, in virtue of that fact alone, essential to the process. But it
is also probable that the multiplied transmission of impressions,
which lies perhaps at the basis of the process of their higher
elaboration in ganglionic centres, is carried on in the larger network
of gray fibres as well as in the smaller network contained in the
ganglionic cells. Destruction of a portion of this network would
therefore interfere with the elaboration of the motor impulse, in the
same manner, though to a relatively less extent, as destruction of the
ganglionic cells themselves.
Trophic Lesions.—The rapid wasting of the paralyzed muscles, with
their degenerative electrical reactions, seems, however, to be an
effect altogether peculiar to lesions of the ganglionic bodies.[125]
According to Charcot, who has so especially formulated the laws of
amyotrophic paralysis, all the ganglionic cells essential to the
elaboration of motor impulses exercise a trophic influence upon
muscles. The spinal cell, nerve-fibre, and muscle-fibre combine into a
complex indissoluble unity or element. One part of this lesion of
complex elements is necessarily followed by proportionate lesion of
all its other parts.
[Footnote 125: The amyotrophic lateral sclerosis of Charcot exhibits
in an exquisite manner the difference between paralysis without
atrophy, caused by sclerosis of the antero-lateral columns, and
paralysis with atrophy when the morbid process has extended to the
anterior cornua.]
According to Erb, however, who extends Samuel's doctrine of special
trophic nerves, it is not the motor cells which influence the
nutrition of the muscle-fibres with which they are connected, but
special trophic cells lying among the others in the anterior cornua.
This theory is principally based on the existence of muscular
atrophies of central origin (progressive muscular atrophy, bulbar
paralysis), unaccompanied for a long time by paralysis.[126] Hammond
cites as a converse example the anterior poliomyelitis “where the
peripheric disturbance is, in the first place, solely one of motility;
this is paralysis without atrophy. After a time, which may be as much
as six months or even more, the trophic changes begin.”[127]
[Footnote 126: _Ziemssen's Handbuch_.]
[Footnote 127: _Loc. cit._, p. 429.]
But surely this is an exaggerated emphasis on the exception, rather
than the true inference from the rule of rapid wasting in anterior
poliomyelitis—a rule so general as to have originated the title
atrophic paralysis. Erb gives an ingenious scheme (Fig. 55) of the
mental relations of motor and trophic cells with cerebral and spinal
nerve-fibres. It will be seen that isolated lesions of one or the
other trophic apparatus might occur without paralysis of motor tracts,
while simultaneous lesion of the trophic {1146} apparatus and of the
ganglion-cells, or of the latter, involving the tracts coming from the
trophic cells, would cause, as in anterior poliomyelitis, motor
paralysis, muscular atrophy, loss of the reflexes, degenerative
reaction in nerves and muscles.
[Illustration: FIG. 55. _c_, trophic cell for nerve; _a_, cerebral
fibre; _b_, trophic cell for muscle; _d_, ganglionic cell; _s_,
sensory fibre; _f_, trophic path to muscle; _m_, muscle. (From
_Ziemssen's Handbuch der Speciellen Pathol._, Bd. xi. Zweite H.,
Zweite Abtheil, p. 313.)]
Duchenne and Joffroy[128] also argue the existence of special trophic
nerve-cells. The absence[129] of the nutritive lesions of the skin and
cellular tissue which are so conspicuous when the gray matter around
the {1147} central canal or posterior to it is involved,[130] the
dependence of the nutrition of the motor apparatus, nerves, muscles,
bones on the integrity of the anterior horns, are facts which, taken
together, seem to indicate that the maintenance of nutrition depends
on the unbroken continuity of the motor or sensory apparatus from the
periphery to the ultimate central element, rather than on any special
central cells endowed with trophic functions.[131] Erb's hypothesis,
as his own scheme moreover denotes, demands not only trophic cells
distinct from motor cells, but separate trophic cells for the muscles,
for the motor, and for the sensory nerves.
[Footnote 128: “De l'Atrophie aigue et chronique des Cellules
nerveuses,” _Arch. de Phys._, No. 4, 1870.]
[Footnote 129: Money, and also Gowers, have signalized a condition of
the skin resembling myxœdema (_Tr. Path. Soc. London_, 1884, and
_Brit. Med. Journ._, 1879).]
[Footnote 130: Mayer (Herman's _Handbuch Physiol._) sums up the great
mass of evidence now accumulated, which demonstrates the trophic
influence of the central gray mass of the cord upon the tissue.]
[Footnote 131: Nepveu (_La France médicale_, 1879) mentions some cases
of infantile paralysis complicated with trophic lesions of the skin.
The facts, if accepted, could only indicate an extension of the
myelitis to the central and posterior regions of the gray columns. The
relations between non-atrophic paralysis caused by interruptions of
the motor tracts and muscular atrophy dependent on lesion of the
anterior cornua are exquisitely shown in a case reported by Sander. An
adult suffered from chronic motor paralysis, gradually increasing, in
the right arm, with paresis of the lower extremities. In the hand,
arm, and shoulder the paralysis was followed by gradual atrophy and
diminution of the faradic contractility; in the lower extremities no
atrophy occurred. At the autopsy was found a gliomatous tumor seated
in the anterior cornua predominating on the right side, extending from
the level of the sixth dorsal to that of the eighth cervical vertebra.
The ganglion-cells were pigmented and compressed, not altogether
destroyed. The lumbar cord was intact, and the non-atrophic paresis of
the lower extremities evidently resulted from the interruption of the
motor tract above.]
The peculiar grouping of nerve-centres within the cord that seems to
be indicated by some of the groupings of infantile paralysis shows, as
has been said, a probable divergence within the cord of nerve-fibres
which run together in the same nerve-stem. The associations to be
expected from the data of functional association and of clinical
history are by no means fully decided. It is even a matter of dispute
whether the tibialis anticus is functionally more associated with the
flexors or with the extensors of the thigh, and whether its
experimental irritation or clinical paralysis really coincides with
that of the first or of the second group. This entire field of
observation is new and promises fertile results.[132]
[Footnote 132: It is from this field that has come a new argument for
the spinal nature of lead-paralysis, from its peculiar grouping, and
from analogy with that of anterior poliomyelitis of the upper
extremities (Remak, “Ueber die Local. Atropa. Spinal Lahm.,” _Archiv
für Psych._, Bd. ix.; also, Ferrier, _loc. cit._).]
Relation between Limitation of Myelitis and Age.—From the relative
frequency of anterior poliomyelitis in childhood, as compared with its
much greater rarity in adult life, we must infer the existence of some
special conditions in childhood which tend to limit the morbid process
to such a portion of the cord. The theory of a primitive spontaneous
atrophy of the motor cells would serve, indeed, to explain this
limitation. The reasons already alleged for regarding the morbid
process as a systematic myelitis decisively hinder the acceptance of
such an explanation as it stands. On the assumption, however, that the
myelitis is usually of functional origin, and starts, therefore, in
the elements of the anterior cornua essentially involved in the motor
functions, the morbid susceptibility of these elements may be ranked
with the liability to disease of the entire locomotor system which is
known to be so predominant in children. From pathological evidence,
even without anatomical proof, we may reasonably infer an
incompleteness of development in the anterior {1148} cornua of the
cord correlative with that well demonstrated in the bones and
functionally inexperienced muscles. If the antero-posterior fibres
which connect the anterior cornua with the central and posterior gray
masses be also incomplete, the radiation of irritations, and
consequent vascular irritation, would also be arrested within the
boundaries of the original lesion. Thus a peculiarly circumscribed,
instead of the common diffused, myelitis of adults.
Money[133] points out that for the gray matter of the cord, as of the
brain, the centre or maximum force of the circulation is on the
periphery, and the nutritive supply of the centre is thus easily cut
off. Moreover, while the blood-vessels of the cervical and dorsal
regions of the cord pass to it transversely, the vessels of the lumbar
region are compelled to describe a somewhat prolonged vertical course
before reaching their point of distribution. From these circumstances,
even transitory congestions in the circulation of the cord are easily
followed by irreparable injury of its delicate elements.
[Footnote 133: _Loc. cit._, _Path. Trans._, 1884.]
Finally, in all discussions on pathogeny must not be forgotten the
doctrine of Leyden[134] that infantile paralysis, also progressive
muscular atrophy, is a disease which may begin at the periphery and
extend to the centres, as well as the reverse. It must also be
remembered that, as yet, only very scanty evidence exists to support
this, in itself, plausible theory.
[Footnote 134: See _loc. cit._, ut supra.]
COURSE OF INFANTILE PARALYSIS.—The most ordinary course of infantile
paralysis is that already described as typical—namely, extremely rapid
development to a maximum degree of intensity, then apparent
convalescence, retrocession of paralysis, atrophy, and ultimate
deformities in limbs in which paralysis persists.
Several variations from this typical course are observed. Complete
recovery may take place, as in the so-called temporary paralysis of
Kennedy[135] and of Frey.[136] These cases are very rare. But their
possibility seriously complicates the estimate we may make of the
efficacy of therapeutic measures.[137]
[Footnote 135: _Dublin Quarterly Journal_, 1840.]
[Footnote 136: _Berlin. Klin. Wochensch._, 1874. I have described one
such temporary case in the article already quoted. These cases seem
about as frequent in adults. (See Frey, _loc. cit._; also case of
Miles, etc. etc.)]
[Footnote 137: As of the case of complete recovery, the only one the
author had seen, related by Dally, _Journal de Thérap._, 1880, 1,
vii.]
On the other hand, there may be a complete absence of regression; and
this is observed sometimes in cases where the paralysis is originally
limited; sometimes where it is extremely extensive, involving nearly
all the muscles of the trunk or limbs;[138] or muscles or limbs
originally spared may become involved in a fresh attack. Laborde
relates cases of this kind. In Roger's first case paraplegia occurred
under the influence of scarlatina two months after paralysis of one
arm.
[Footnote 138: Thus in Eulenburg's case, quoted ut supra.]
The form of anterior poliomyelitis most frequent in adults is the
subacute, and after that the chronic. Both are extremely rare in
children, the latter excessively so. Seeligmüller and Seguin[139] both
admit the possibility of a chronic form in children, and the latter
has kindly communicated to me one case from his private practice:
{1149} Miss N. D——, æt. 15, paresis in both legs, first at age of
nine, increased at age of twelve, when weakness of vision first noted.
At fourteen both feet in rigid pes equinus, and both tendons achilleis
cut, without benefit. Hands became tremulous, without paresis. On
examination at age of fifteen found moderate atrophy of muscles of
both legs. Tendo Achillis united on both sides, and equinus persists.
Voluntary movement exists, both in anterior tibial and in
gastrocnemius muscles, but diminished in anterior tibial. Faradic
contractility diminished in both sets of muscles; examination
difficult from extreme sensibility of patient. In both hands
interossei, muscles of thumb, and little finger show tremors and
fibrillary contractions. Thenar eminences small, abductor pollicis
nearly absent, not reacting to faradic current. Optic nerves slightly
atrophied. Mind enfeebled, memory poor; articulation not affected.
Five years later the motor paralysis and mental enfeeblement had still
further progressed, but no exact notes exist of this period.
[Footnote 139: _Loc. cit._ (ed. 1877).]
Erb[140] relates a case that he considers unique at the time in a girl
of six. The paralysis began insidiously in the right foot in July; a
fortnight later had extended to the left foot; complete motor
paralysis existed in August, without any lesion of sensibility: after
electrical treatment, then instituted, first return to motility to
peroneal muscles in November; by January child able to walk again and
electrical reactions nearly normal.[141]
[Footnote 140: _Brain_, 1883.]
[Footnote 141: In the same number of _Brain_, A. Hughes Bennett quotes
cases of so-called chronic paralysis in very young children which are
evidently cases of general paresis from congenital cerebral atrophy.
The children were defective in intelligence, could not sit up nor hold
up the head; the electrical reactions were preserved. I have seen a
great many such cases: they are indeed not at all uncommon. Much more
so is Bennett's diagnosis.]
COMPLICATION WITH PROGRESSIVE MUSCULAR ATROPHY.—Raymond[142] and
Seeligmüller describe some rare cases where progressive muscular
atrophy declared itself in persons previously affected with infantile
paralysis in other limbs. Both observers infer a gradual and chronic
extension along the cord of the originally acute anterior
poliomyelitis.[143] Similar cases have much more recently (1884) been
quoted by Ballet as tending to modify the prognosis which has usually
been pronounced favorable quoad life and further spinal accidents.
(See infra.)
[Footnote 142: _Gaz. méd._, 1875. No. 17.]
[Footnote 143: It seems to me that Seguin's case, above quoted, might
be an example of such complication(?). But I have not seen the patient
myself, and describe the case according to the views of the author.]
PROGNOSIS.—The prognosis of atrophic paralysis, quoad vitam, is, as is
well known, extremely good. The prospect of recovery from the
paralysis is variable. It cannot be estimated either by the extent of
the initial paralysis or by the severity of the fever or attendant
nervous symptoms. The electrical reactions alone are of value in the
prognosis, and their value is very great. Duchenne first formulated
their law: “All the cases of infantile paralysis which I have seen
where the faradic contractility was diminished but not lost, and which
could be treated by faradic electricity within two years after the
onset of the paralysis, have completely recovered.”[144] This
encouraging statement must be read as applying rather to individual
muscles than to cases as a whole. Few {1150} complete recoveries of
patients are claimed even by so enthusiastic an electrician as
Duchenne; who nevertheless affirms his not unfrequent success in
re-creating entire muscles out of a few fibres saved from
degeneration.
[Footnote 144: _Loc. cit._]
The persistence of galvanic irritability in muscles which fail to
contract to the faradic current has been shown by Erb to belong to the
degenerative reactions. Hammond, however, without alluding to the
qualitative changes in the galvanic contractions, sees in them the
elements of a relatively favorable prognosis, even when faradic
contractility is lost. Thus, out of 87 cases, in 39 of which the
paralyzed muscles contracted to the galvanic but not the faradic
current, 14 were entirely cured, 28 greatly improved, 30 slightly
improved, 15 discontinued treatment very early.[145]
[Footnote 145: _Loc. cit._, p. 482.]
Examination of fragments of living muscle obtained by Duchenne's
harpoon, though useful, should not be allowed to exaggerate an
unfavorable prognosis. Much fat may be found in such fragments when
the muscle is as yet by no means completely degenerated and can be
made to contract to one or the other current. Erb, however, admits
that the results of treatment have not, in his hands, been brilliant;
but adds that he has had no opportunity to treat any cases which were
not of long standing.[146]
[Footnote 146: _Loc. cit._]
Volkmann[147] considers the paralysis entirely hopeless, and advises
the concentration of all effort upon the prevention or palliation of
deformities.
[Footnote 147: _Loc. cit._]
It seems probable that at the present moment sufficient data do not
exist for formulating a fair prognosis; nor will they until a much
larger number of cases than hitherto have been submitted to all the
resources of a complex and persevering system of therapeutics from the
earliest period of the disease.
SPECIAL PARALYSES.—Among the paralyses, some exercise a more
unfavorable influence on locomotion than others. Thus, paralysis of
the muscles of the trunk is more difficult to palliate, either by
apparatus or by the efforts of the patient, than any paralysis of the
limbs. Similarly, paralysis of the upper segments of a limb is more
crippling than when confined to the lower. Partial paralysis of the
muscles surrounding a joint is often (but not always) more liable to
lead to deformity than total paralysis.
Influence of Neglect.—Apart from the influence of treatment in curing
the paralysis, must be estimated in the prognosis the effect of care
and watchfulness in limiting the disease and in averting many
consequences, even of those which are incurable. The rescue of muscles
only partially degenerated may often serve to compensate the inaction
of those which are irretrievably ruined.
Ballet[148] has recently called attention to the fact that in certain
cases persons who had been attacked with an anterior poliomyelitis in
childhood became predisposed to different forms of spinal disease.
Four have been observed: (1) transitory congestion of the cord,
causing paralysis of a day or two's duration; (2) an acute spinal
paralysis of the form usually seen in adults; (3) subacute spinal
paralysis; (4) progressive muscular atrophy. The author relates cases
under each of these heads, and further quotes one {1151} related by
Dejerine in 1882.[149] The patient, a carpenter aged fifty-five and
with an atrophic deformity of the foot, became suddenly paralyzed in
the four limbs, trunk, and abdomen. The paralysis was complete in a
month, was stationary for three months, then began to improve, and at
the end of six months from the onset of the disease recovery was
complete.
[Footnote 148: _Revue de Médecine_, 1884.]
[Footnote 149: _Revue de Médecine_, 1882.]
The observations of progressive muscular atrophy in persons bearing
the stigmata of an infantile paralysis are quite numerous.[150]
[Footnote 150: Charcot, _Soc. Biol._, 1875, and _Gaz. méd._;
Seeligmüller (4 cases), in _Gerhardt's Handbuch_, 1880; Hayem, _Bull.
Soc. de Biol._, 1879; Vulpian, _Clinique méd. de la Charité_, 1879;
Pitres, new observation, quoted by Ballet in 1884.]