Remarks: H. Ewen, _Tr. Path. Soc. Lond._, 1848-50, i. 179.
37. Sex and Age: F. 28.
Clinical History: Weakness in legs. Aching and shooting pains in legs.
Numbness and formication. Slight spasm in legs. “Felt as if ground was
some distance below feet.” Œdema of ankles. Later, numbness extended
to abdomen. Paralysis of bladder. Hyperæsthesia in right leg.
Obstinate constipation. Bed-sores. Some paralysis of respiratory
muscles. Duration, fourteen months.
Path. Anat. and Location: Tubercle the size of cherry, which had
almost obliterated cord. Tubercles in lungs, bowels, and uterus.
Bed-sore had opened spinal canal.
Lower part of dorsal cord.
Remarks: S. O. Habershon, M.D., _Guy's Hosp. Rep._, London, 1872, 3d
S., xvii. 428-436.
38. Sex and Age: M. 63.
Clinical History: Progressive paresis of left leg for five years.
Right leg then paretic. Paralysis then in left leg. Rigidity on
extension of right leg. Paroxysms of clonic spasms in right leg. Joint
pains, sciatic pains. In left leg, hyperæsthesia, in right leg,
anæsthesia. Plantar reflex retained; other reflexes exaggerated.
Diplegic contractions in right leg from irritation in left. Late
symptoms: purulent urine, with retention; chest and lumbar pains like
bone pain; extension changed to flexion; swelling of legs and
ecchymosis; sacral and other eschars.
Path. Anat. and Location: Psammoma adherent to dura mater. Cord
softened. Ascending degeneration in posterior columns, and descending
degeneration of lateral columns.
In dorsal region just above lumbar enlargement, anterior left side.
Remarks: Charcot, _Arch. de Physiol._, Paris, 1869, ii. 291-296.
39. Sex and Age: M. 20.
Clinical History: Paralysis of lower extremities; tremor; exaggerated
reflexes, hyperæsthesia of trunk; bed-sores. Œdema of feet. Fever. Pus
in urine.
Path. Anat. and Location: Organized blood-clot exterior to dura mater.
Cord compressed and softened.
Opposite lower dorsal and upper lumbar.
Remarks: C. B. Nancrede, _Am. Journ. Med. Sci._, O. S., lxi. 156.
40. Sex and Age: F. 38.
Clinical History: Pain around abdomen, in back, and legs. Paraplegia.
Anæsthesia and tingling of feet and legs. Paralysis of bladder.
Path. Anat. and Location: Hydatid cysts of vertebræ (?) and spin.
canal.
Lower part of spinal canal (probably lumbar region).
Remarks: S. Wilks, _Dis. Nerv. Syst._, p. 265.
41. Sex and Age: F. 23.
Clinical History: Bronzing of skin for two years; then headache,
giddiness, fever. Choreic movements in left arm, then in leg, then
general. Bronzing increased. Vomiting after meals. Duration, two years
and two months.
Path. Anat. and Location: Tumor, consisting of granular matter, with a
few nerve-fibres and cells, springing from centre of cord backward to
posterior fissure. Cord slightly widened. Suprarenal capsules large
and nodulated.
Lumbar enlargement.
Remarks: W. H. Broadbent, _Trans. Path. Soc. Lond._, 1861-62, viii.
246.
42. Sex and Age: 10 ms.
Clinical History: Twitching and convulsive movements of right leg.
After removal of exterior tumor the movements ceased. Child died of
peritonitis.
Path. Anat. and Location: Tumor outside of sacrum, and also protruding
through sacral opening. Reported to have been behind and pressing upon
cord (?). Fatty growth within membranes.
Remarks: Arthur Johnson, _ibid._, 1856-57, viii. 28, 29.
{1112} 43. Sex and Age: F. 54.
Clinical History: Paresis, first of left arm and leg; then paralysis
of these and of right arm and leg. Pain in back and hips early; then,
suddenly, darting pains and incontinence of urine. Paræsthesia of left
arm and leg; no anæsthesia. Coma.
Path. Anat. and Location: Hydatid cyst. Cyst also in liver. Fluid
beneath membranes of cord and brain.
At first and second left sacral foramen, opposite last lumbar and
upper three sacral vertebræ.
Remarks: H. S. Wood, _Australian Med. Journ._, 1879, N. S. i. 222.
44. Sex and Age: M. 46.
Clinical History: Fibrillary twitching. Increased patellar reflexes.
Paræsthesia and hyperæsthesia in legs, disappearing. Constriction of
chest (?). Headache. Dysuria for two years. Straining at stool.
Indigestion. Bloody vomiting. Cardiac palpitation; intracardial
murmurs; slow pulse. Swollen inguinal glands. Variations in
temperature. Bed-sores.
Path. Anat. and Location: Glioma.
At filum terminale, upper part.
Remarks: Lachman, _Arch. f. Psychiat._, Berl., 1882, xiii. 50-62, 1
pl.
45. Sex and Age: M. 38.
Clinical History: Pain in legs. Œdema. After two years could not lie
down: rested on hands and knees. Paralyzed in legs; pain in seat.
Anæsthesia in legs, not complete in right. Paræsthesia in left.
Dysuria and constipation. Before death had incontinence with
hæmaturia, and was able to lie down.
Path. Anat. and Location: A lobulated tumor from pia mater at lower
end of spinal canal, surrounded by nerves of cauda equina. Structure
not made out.
At cauda equina.
Remarks: W. W. Fisher, _Tr. Prov. M. and S. Ass._, 1882, x. 203-208.
46. Sex and Age: ——
Clinical History: This case had symptoms of posterior spinal
sclerosis, which possibly had no relation to growth, according to
reporter.
Path. Anat. and Location: Myo-lipoma attached to conus medullaris.
Crescentic, clasping cord from anterior to posterior fissure.
Nerve-roots of cauda equina imbedded in it. Contained striated
muscular fibres.
Remarks: W. R. Gowers, _Tr. Path. Soc. Lond._, 1875-76, xxvii. 19-22.
47. Sex and Age: Und. 1 yr.
Clinical History: Spina bifida (?); hydrocephalus; convulsions, bloody
stools; partial paraplegia. (Above symptoms came on after closing of
sacral opening by surgical operation.)
Path. Anat. and Location: Congenital sacral neuroma amyilinicum.
Remarks: W. F. Jenks, M.D., _Trans. Path. Soc. Philada._ (1871-73),
1874, iv. 190-192.
48. Sex and Age: M. 30.
Clinical History: Pain in back; abdominal girdle sensation. Pain in
legs; paraplegia; nearly complete anæsthesia; paralysis of bladder;
bed-sores.
Path. Anat. and Location: Aneurism, eroding vert. and compressing
cord. Location not given.
Remarks: Wilks, _Dis. Nerv. Syst._
49. Sex and Age: M. 54.
Clinical History: Paralysis of both legs, of sphincter ani, and of
bladder; urine alkaline, with pus and blood. Partial anæsthesia.
Pyonephritis.
Path. Anat. and Location: Gumma from inner layer of dura mater and
involving pia mater. Location not given.
Remarks: Delafield, _N. Y. Med. Rec._, 1875, x. 131.
50. Sex and Age: Stillborn.
Clinical History: ——
Path. Anat. and Location: Tumor, size of head of child two years old,
projected between legs from spinal column. Nerves of cauda equina over
anterior part. Some bone in tumor (dermoid cyst?).
Remarks: Virchow, _Monatschr. f. Geburtsk._, Berl., 1857, ix. 259-262.
{1113}
INFANTILE SPINAL PARALYSIS.
BY MARY P. JACOBI, M.D.
SYNONYMS.—Essential paralysis of childhood (Rilliet and Barthez);
Myogenic paralysis (Bouchut); Acute fatty atrophic paralysis
(Duchenne); Atrophic paralysis (Ferrier); Acute anterior poliomyelitis
(Kussmaul, Erb, Seguin); Regressive paralysis (Barlow); Tephromyelitis
(Charcot).
DEFINITION.—Of all the titles which have been given to the disease it
is our purpose to describe, two alone may be considered
irreproachable. In the present state of our knowledge it is
unnecessary to argue that this disease is not essential—_i.e._
destitute of characteristic anatomical lesions. Neither can the theory
of its myogenic origin be maintained; nor even is fatty degeneration
invariably present in the paralyzed muscles. Finally, the disease
cannot longer be regarded as peculiar to childhood,[1] since cases in
adults have been in these last years quite numerously reported[2]—four
with autopsies demonstrating the identity of the lesion. But there are
two definitions in our list of synonyms which embrace between them the
most striking characteristics of the disease, yet contain no error of
fact. Atrophic paralysis describes at once the two most salient
symptoms; acute anterior poliomyelitis defines at once the seat and
nature {1114} of the lesion, classes it with the systematic
diseases[3] of the spinal cord, and notes the peculiarity in the mode
of invasion by which it is so remarkably distinguished from nearly all
the organic diseases of this centre.
[Footnote 1: W. H. Barlow, _On Regressive Paralysis_, 1828. See
_Brain_, April, 1879.]
[Footnote 2: In Dec., 1873, I quoted 14 cases of adult spinal
paralysis, as follows: Duchenne, 4 cases; Charcot and Petitfils, 3;
Moritz Meyer, 2; Bernhardt (_Archiv Psych._, 1873), 1; Cumming
(_Dublin Quart. Journ._, 1869), 1; Lucas Championnière (by Hallopeau,
_Archives gén._, 1861), autopsy, 1; Gombault (_Archives de Psych._,
1873), 1; personal, 1.
In 1874, Seguin published a summary of all the foregoing cases except
the last, and added 6 personal observations, also 3 from Duchenne and
1 from Hammond. In the enlarged edition of his essay in 1877, Séguin
increased the list to 45—by new personal cases, 3; cases related by
Frey (_Berlin. Wochens._, 1874), 4; cases by Erb (_Arch. f. Psych. u.
Nervenkrank._, v.), 4; case by Cornil and Lépine (_Gaz. méd._, 1875),
autopsy, 1; case by Soulier (_Lyon méd._, 1875), 1; case by D. H.
Lincoln (_Boston Med. and Surg. Journ._, 1875), 1; case by Lemoine
(_Lyon méd._, 1875), 1; case by George M. Beard, 1; case by Leyden
(_Klinik Ruckenmarks Krankheiten_) Bd. iv. 1; case by Hammond (6th ed.
_Treatise_), 4; case by Courty (_Gaz. méd._, 1876), 1; case by
Dejerine (_Arch. de Phys._, 1876), 1.
To these may be added—case by Goltdammer (_Berl. klin. Wochen._,
1876), 1; case by Webber (_Trans. Amer. Neurol. Ass._ for 1875, vol.
i.), autopsy, 1; case by Klose (_Diss. Breslau_, 1876), 1; case by
Schultze (_Virchow's Archiv_, Bd. lxviii.; also Bd. lxxiii.), autopsy,
1; case by Bernhardt (_Archiv für Psych._, Bd. ix., 1879); case by
Sinkler (_Amer. Journ. Med. Sci._, Oct., 1878), 5; case by Althaus
(_ibid._, April, 1878), 2; case by Ross (_Dis. Nerv. Syst._, vol. ii.
p. 139), 1—total, 57 cases.
Morton (_St. Bartholomew's Hospital Reports_).
Others have doubtless been published since this date, but, as they do
not immediately concern our subject, need no further citation.]
[Footnote 3: Vulpian, _Leçons sur les Myelitis_, 1880.]
SUMMARY OF CLINICAL HISTORY.—The clinical features of an acute attack
of infantile paralysis are well known. The children affected are
usually between eighteen months and four years of age (Henoch). The
attack is more likely to occur in summer than in winter, as Sinkler[4]
found that 47 out of 57 cases began between May and September, and
Barlow noted 27 out of 53 in July and August.[5]
[Footnote 4: _Amer. Journ. Med. Sci._, April, 1875.]
[Footnote 5: _Loc. cit._, p. 75. Among Sinkler's 57 cases, only 6
furnish autopsies, thus:
Case by Cornil and Lepine and case by Webber (quoted and accepted by
Erb in _Ziemssen's Handbuch_, Bd. xi.); case by Gombault (rejected by
Erb and Westphal); case by Schultze; cases by Dejerine and Lucas
Championnière (quoted by Hallopeau).
The influence of heat is perhaps shown in the case related by
Dyce-Duckworth in the _Lancet_ of 1877: a child two and a half years,
after exposure to great heat on a steamboat-landing, became paralyzed
in all four limbs, but the paralysis was subsequently confined to the
lower extremities. Coincidently, the patient became delirious;
suffered from anæsthesia and temporary paralysis of the sphincters.
The paralyzed muscles wasted rapidly and lost faradic contractility.
Treatment by faradization was begun in a month from the date of the
attack, and recovery was complete three months later.]
The onset of the paralysis is either really sudden, occurring in the
daytime, while the child is under competent observation, or apparently
sudden, being discovered in the morning after a quiet night, the child
having gone to bed in health (West); or is preceded by some hours or
days of fever or of nervous symptoms, especially convulsions, or both.
The paralysis is almost always at its maximum of extent and intensity
when first discovered, and from this maximum begins, within a few
hours or days, to retrocede. The improvement may, however, be delayed
much longer. A variable number of muscles remain permanently
paralyzed, and in these, within a week (thirty-six hours, according to
some observers), faradic contractility is first diminished, then
abolished; galvanic reaction is exaggerated, ultimately is
characterized by the degeneration signs (entartungs reaction). The
temperature of the paralyzed limbs falls; the muscles waste; the
atrophy may rapidly become extreme. The paralysis and loss of faradic
contractility are complete, however, while the atrophy is only
incipient and progressing. The absence of lesions of sensibility, of
visceral disturbance, of trophic lesions of the skin, or of sphincter
paralysis is as characteristic of the disease as are the positive
symptoms above enumerated.
In the third or chronic stage the paralyzed limbs often become
contracted and deformed. At other times, and with more complete
paralysis, the growth of the bones is arrested, the muscles remain
flaccid, the entire limb shrivels, and dangles so loosely from its
articulations that it may be dislocated by slight effort (membre de
Polichinelle). The general health of the patient remains remarkably
good, the intelligence clear, the disposition lively. The duration of
life seems to be in nowise shortened by the paralysis. Thus,
suddenness of development, intimate association of trophic, motor, and
electrical disturbance, absence of cerebral or sensory lesion,
peculiar localization and grouping of the permanent paralyses,—such
are the salient characteristics of this remarkable disease.
SYMPTOMS IN DETAIL.—Three well-defined stages exist—the initial, the
paralytic, and the chronic.
{1115} Initial Stage.—Seeligmüller[6] signalizes 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]
[Footnote 6: _Gerhardt's Handbuch der Kinderkrankheiten_, 1880
(separat Abdruck).]
[Footnote 7: Rilliet et Barthez, _Traité des Maladies des Enfants_,
ii. p. 551.]
Mode of Invasion.—I have elsewhere[8] described 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;[9] and some apparent exceptions, in which recovery occurred
rapidly, seem to belong to the temporary paralysis of Kennedy,[10]
more recently described again by Frey.[11]
[Footnote 8: _Am. Journ. Obstet._, May, 1874.]
[Footnote 9: _Archives 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.]
[Footnote 10: _Dublin Quarterly_, 1850.]
[Footnote 11: _Berlin. 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),[13] or
104° F. (Barlow).[14] Duchenne fils observed 7 cases alleged to be
entirely without fever, and Laborde counts 10 cases out of 50 as
apyretic.[15]
[Footnote 12: _Ziemssen's Handbuch_, Bd. xi. Abh. 12.]
[Footnote 13: _Vorlesung. 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.]
[Footnote 14: _Loc. cit._]
[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.