Case No. | Name of Author. | Date of Autopsy | Year of
| | after Paralysis. | Publication.
---------+-----------------+------------------+-------------
41 | Damaschino. | 6 weeks. | 1883
40 | Turner. | 26 days. | 1879
28 | Roger and | |
| Damaschino. | 2 mos. | 1871
42 | Money. | 4 mos. | 1884
27 | Echeverria. | 6 mos. |
39 | Eisenlohr. | 6 mos. |
29 | Roger. | 6 mos. | 1871
31 | Roth. | 9 mos. | 1873
35 | Leyden. | 11 mos. | 1876
14 | Bouvier and | |
| Laborde. | 12 mos. | 1864
30 | Roger. | 13 mos. | 1871
15 | Cornil and | |
| Laborde. | 16 mos. | 1864
12 | Recklinghausen. | 24 mos. | 1863
38 | Demme. | 24 mos. | 1876
23 | Taylor. | 18 mos. | 1879
25 | Humphrey. | 24 mos. | 1879
---------+-----------------+------------------+-------------
AUTOPSIES OF RELATIVELY RECENT CASES.—In cases relatively recent all
macroscopic changes in the cord may be entirely wanting. There may be
some degree of asymmetry in the surface of section, patches of white
coloration in the anterior gray substance, or of gray or yellow color
in the white columns; the anterior roots may be congested or even
already atrophied.[94] On the other hand, there have several times
been found foci of visible red softening, much more frequently at a
point corresponding to the origin of the paralyzed nerves, but not
absolutely confined to them, and sometimes existing at points where
they have given rise to no symptoms whatever.[95]
[Footnote 94: Roger's first case, No. 28 of Table V.]
[Footnote 95: Case 1st of Roger, Tab. V.]
Microscopic Lesions.—In striking contrast with this paucity of
macroscopic lesions are the interesting structural changes revealed
under the microscope. These lesions are usually comprised within
circumscribed foci whose size may vary from a long diameter of
2 mm.[96] to one of from 10 to 30 mm.[97] Sometimes bilateral foci are
found with monoplegic paralysis; thus one side or the other
preponderates in the morbid process.
[Footnote 96: Case Roth, Tab. V.]
[Footnote 97: Case Schulze, Tab. V.]
In recent cases (Damaschino's, at twenty-six days; Roger's, at two
months; Turner's, at six weeks) patches of red softening existed at
the portions of the cord containing the nuclei of origin of the
paralyzed nerves. In Turner's case the focus contained hemorrhagic
extravasation, and the traces of this were clearly perceptible in
Roger's first case. The blood-vessels are dilated; their lymphatic
sheaths infiltrated with leucocytes and with granular corpuscles;
their walls are thickened, pigmented, or fatty.
{1138} Leucocytes are often disseminated through the diseased area,
and in one case (Demme) were accumulated into a focus of pus. Besides
the leucocytes, the foci are often infiltrated with large round
granular cells that seem to be transformed neuroglia-cells (Leyden).
In one case neuroglia nuclei were accumulated in a ring around the
focus, seeming to indicate the beginning of encapsulation.
The most striking lesion, however, and the one which is common to the
most recent as well as to old cases, is the deformation, atrophy, and
final disappearance of the large ganglionic cells of the anterior
cornua. The first change consists in granular pigmentation;[98] then
the prolongations disappear, leaving the body of the cell shrunken and
deformed; at last the whole cell disappears. Sometimes all the cells
of an anterior horn have disappeared throughout the entire depth of
the focus; quite as often, in certain sections at least, the atrophy
is limited to certain groups, as the external,[99] or the external in
one focus, the antero-lateral in another, situated on the opposite
side of the cord.[100]
[Footnote 98: Case of Echeverria, Tab. V.]
[Footnote 99: Case by Schultze (this is a case of ancient lesion),
Tab. V. (_Virch. Arch._, Bd. lviii.).]
[Footnote 100: Case by Taylor, Tab. IV. (_Path. Trans._, London,
1879.)]
In a case rendered celebrated by Charcot it is stated that in many
sections of the cord atrophy of ganglionic cells constituted the
unique alteration, the tissue immediately surrounding the place whence
they had disappeared being perfectly healthy. It is on this appearance
that has been built up the theory of a primary idiopathic atrophy of
the ganglionic cells as the characteristic lesion of infantile
paralysis. But in other portions of the same cord Charcot himself
describes destruction of the gray reticulum imbedding the cells; and
this destruction is insisted upon in many other observations. In other
words, there is a general disintegration of the gray nervous tissue of
the anterior cornua which contain the focal lesion. The normal tissue
is then replaced by a reticulum of conjunctive fibres, more or less
dense according to the age of the case.
These focal lesions of the cord explain admirably, as will be seen,
the permanent symptoms of the disease. But of great importance for
understanding its initial period is the fact that structural
changes—similar to, but less severe than, those just described—have
beer found diffused throughout the cord. In several cases hyperæmia,
partial atrophy of ganglionic cells and nerve-fibres, infiltration
with exudation corpuscles, in the anterior cornua and even central
gray canal from the lumbar to the cervical region.[101] In
Damaschino's case, besides the focal lesions which corresponded to the
paralyses of the right arm and left leg, were others corresponding to
the left arm and right leg where no paralysis existed.
[Footnote 101: See cases by Leyden, Roth, Schultze, Clarke,
Damaschino.]
This case (1883) is also interesting in bringing out another lesion
not usually noticed. This is the breaking up into balls of the myeline
in the medullated tubes, both of the anterior intraspinal nerve-roots
and of such fibres as traversed the anterior nerves. In balsam
preparations these myeline drops are dissolved; but in osmic acid and
glycerin preparations they appear as black balls all over the field.
The lesion is identical with that already described by Dejerine (1875)
in both nerves, roots, and white columns. The columns of Clarke have
always been found intact.
{1139} In the recent cases no lesions of the white columns have been
observed—a fact upon which reposes the doctrine that such lesions,
when existing, are secondary to those of the cornua.
SECOND GROUP OF CASES.—The next group of cases contains 16, where the
autopsy was made more than two years after the début of the paralysis.
TABLE VII.—AUTOPSIES SHOWING LESIONS OLDER THAN TWO YEARS.
---------+-----------------+------------------+-------------
Case No. | Name of Author. | Date of Lesion. | Year of
| | | Publication.
---------+-----------------+------------------+-------------
11 | Longet. | 7 yrs. | 1842
27 | Echeverria. | 7 yrs. | 1866
43 | Money. | 7 yrs. | 1884
16 | Lancereaux. | 16 yrs. | 1873
38 | Leyden. | 17 yrs. | 1876
33 | Schultze. | 19 yrs. | 1876
17 | Charcot and | |
| Joffroy. | 25 yrs. | 1870
20 | Müller. | 30 yrs. | 1871
22 | Clarke. | 31 yrs. | 1868
10 | Hutin. | 42 yrs. | 1825
13 | Cornil. | 47 yrs. | 1863
36 | Leyden. | 54 yrs. |
34 | Leyden. | 58 yrs. | 1876
19 | Vulpian. | 64 yrs. | 1870
22 | Raymond. | 70 yrs. | 1875
16 | Prévost. | 76 yrs. | 1866
---------+-----------------+------------------+-------------
The date of these lesions varies from seven to seventy-six years. In
two or three cases, where the autopsy was made on very old people, the
early history of the disease was unknown, but the probable date of the
paralysis was calculated.
In this group of cases patches of atrophy, semi-transparent and
grayish in color, focal or diffused, are clearly perceptible to the
naked eye. As a rule, the atrophy is unilateral, and sensibly affects
the entire half of the cord. In some cases of paraplegia, however,
there is a bilateral, symmetrical shrinkage of the entire lumbar cord,
which has been reduced to the size of a quill.
The atrophy involves, first and most markedly, one or both anterior
cornua; second, the anterior nerve-roots arising from them; third, the
antero-lateral columns.
In both the latter localities the microscope will often find
individual nerve-tubes wasted and deprived of their myeline. The
atrophied patches are generally sclerosed as the seat of a
proliferated neuroglia, coloring deeply with carmine. In Laborde's
cases, published at the very beginning of what may be called the
anatomical period, the atrophy and sclerosis were said to be limited
to the antero-lateral columns and the nerve-roots, while the cornua
remained intact. In all more recent observations, however, the lesion
of the white columns and roots has been found strictly proportioned to
that of the gray horns. The sclerosis extended into the latter,
constituted by a reticulum of connective-tissue fibres, sometimes
fine, sometimes so matted together as to form a dense felt-like
substance, sometimes offering the ordinary aspect of sclerosis.
Amyloid corpuscles have been found infiltrated in great numbers {1140}
through both the gray and white substance in these old cases (case by
Cornil),[102] seeming to replace the infiltration with exudation
corpuscles observed in the more recent ones.[103] When the lesion is
distinctly circumscribed the focus is often surrounded by a zone of
embryonic cells, seeming to indicate a reactive proliferation on the
periphery.[104]
[Footnote 102: _Loc. cit._, _Soc. Biol._,1863.]
[Footnote 103: Seguin (_loc. cit._) observes that the opinion is
gaining ground which ascribes these to a transformation of the
neuroglia corpuscles.]
[Footnote 104: Case by Schultze, _loc. cit._]
As in the relatively fresh cases, the circumstance which has attracted
the most attention is the atrophy of the ganglionic cells from the
sclerosed patches of the anterior cornua. The completeness with which
these have disappeared in any focus seems to be proportioned to the
completeness of the paralysis in the corresponding limb. Partial
atrophy or disappearance of spinal groups of cells from the cornua may
sometimes be correlated with paralysis of special muscles.[105]
[Footnote 105: Thus in Schultze's case, already quoted, the external
group of cells had disappeared from the focus in one gray horn, and
the extensors of the foot were alone paralyzed. This seems to confirm
the opinion advanced by Spitzka, that this external group of
motor-cells corresponds to the extensor, the internal groups to the
flexor muscles.]
Study of the pathology of infantile paralysis is not completed when
the above series of spinal lesions has been enumerated. Most various
interpretations have been made of these lesions as they have been
successively discovered. Thus, after the theory of congestion came the
theory of primary sclerosis, built upon Laborde's two autopsies;[106]
then the theory of primary atrophy of ganglionic cells;[107] then the
theory of myelitis;[108] finally, a theory of complex and variable
lesion.[109]
[Footnote 106: Laborde, _loc. cit._; Cornil, _loc. cit._]
[Footnote 107: Charcot, _Leçons sur les Maladies du Syst. nerveux_;
Prévost, _Soc. Biol._, 1864; Joffroy, _Arch. de Physiol._, 1870;
Petitfils, “De l'Atrophie aigue des Cellules matrices,” _Thèse de
Paris_, 1873.]
[Footnote 108: Schultze, _Virch. Arch._, Bd. lxviii.; Roth, _Ibid._,
Bd. lviii.; Henoch, _loc. cit._, p. 208; Ross, _loc. cit._, p. 125;
Seguin, _loc. cit._, 1877; Erb, _Ziemssen's Handbuch_; Seeligmüller,
_Gerhardt's Handbuch_; Roger and Damaschino, _Gaz. méd._, 1871;
Turner, _Path. Trans. Lond._, 1879; Hammond, _loc. cit._]
[Footnote 109: Leyden, _Archiv für Psych._, Bd. vi., 1876.]
It was Prévost who first ascribed a predominant importance to the
atrophy of the ganglionic cells of the anterior cornua; but it was in
the hands of Vulpian, Joffroy, and more especially Charcot and his
pupils, that the theory was fully developed. Infantile paralysis was
ranked in a newly-formed group of diseases, all characterized by
atrophy of these same cells, and differing from each other principally
in the acuteness of the process and in its complications.[110] Seguin,
in his original lecture in 1874, supported the same views, but in 1877
fully adopted that of myelitis. The objections to this theory are:
1st, that by it two diseases so different in their course,
localization, electrical reactions, and form of paralysis as atrophic
paralysis and progressive muscular atrophy are essentially identified
on account of the identity of one lesion, the atrophy of the anterior
ganglionic cells;[111] 2d, the presence of other lesions or of traces
of them peremptorily proves the pre-existence of a complex {1141}
morbid process which involves the ganglionic cells, but is neither
limited to them, nor, necessarily, originates in them.
[Footnote 110: Thus, acute anterior poliomyelitis, subacute anterior
poliomyelitis, progressive muscular atrophy, amyotrophic lateral
sclerosis, bulbar paralysis.]
[Footnote 111: When this objection is accepted, Barlow's remark falls
to the ground, that “the similarity of lesion found in two such
different diseases as infantile paralysis and progressive muscular
atrophy proves the failure of anatomical characters, taken alone, to
serve as a basis of nosology” (_Brain_, April, 1879, p. 74).]
This inference was drawn by Roger from the hemorrhagic softening,
dilatation, and degeneration of blood-vessels, infiltrations with
exudation-corpuscles, and hyperplasia of conjunctive nuclei present in
his case. Similarily, Schultze, in a case examined nineteen years
after the occurrence of the paralysis, found traces of an extensive
myelitis in the diffusion of the lesions,[112] in the exquisite
cellular infiltration, the proliferation of the neuroglia, and the
atrophy of axis-cylinders of nerve-fibres together with the cells; and
inferred an anterior myelitis, diffused in the long axis of the cord,
but limited to the antero-posterior region. Schultze defines Charcot's
theory to be an hypothesis of such an acute atrophy of ganglionic
cells as leads to a rapid melting down of these bodies, whereby
reactionary inflammation is excited in the surrounding tissue. This
implies that the dying cells are able to act like a virulent substance
on the imbedding tissue, and of this, declares Schultze, “Charcot has
offered no proof.”[113]
[Footnote 112: In this case of paraplegia without lesion of the upper
extremity, to which we have several times alluded, there was bilateral
atrophy of the lumbar cord, atrophy of the right anterior nerve in the
dorsal and lower cervical region, also in the cervical enlargement.]
[Footnote 113: It might be said that the fall of the fever as soon as
the paralysis is declared and the motor cells presumably melted down
should contradict the idea that their dying substance acts as an
irritant upon surrounding tissues.]
A third objection has been brought forward by Leyden, and is really an
enlargement on the second. It is, that various lesions or morbid
processes may underlie the same clinical history. In four autopsies of
cases presenting all the clinical history of acute anterior
poliomyelitis this author has found three different lesions. In one an
extensive lepto-meningitis, together with irregular focal sclerosis of
the white columns, evidently depended upon the latter, and in turn
caused sclerosis of the anterior cornua with consequent destruction of
their cells.[114] In two other cases an anterior poliomyelitis was
accompanied by diffused lesions of the central canal. Finally, in a
fourth case the lesions were limited to the anterior cornua, as is
most usual.
[Footnote 114: This case of Leyden's throws light on the two autopsies
by Laborde with sclerosis of the white columns and intact cornua. It
seems probable that a process originating in the cornua had then been
arrested or had receded, while continuing its evolution in the white
columns.]
The theory of acute atrophy of ganglionic cells is not sensibly
different from that of a parenchymatous myelitis.[115] But all the
objections which can be urged against the former theory apply to the
latter also, with the exception that the hypothesis of inflammation
suggests a cause for the otherwise inexplicable atrophy. Observation
of the pathological appearances alone could not decide whether the
irritation started in the parenchymatous or interstitial tissues.
Reference to the etiology of the disease shows that of the two most
frequent apparent causes, {1142} blood-poisoning and traumatisms, the
first would indicate that the inflammation started in the connective
tissue supporting the blood-vessels; the second suggests that the
irritation began in the spinal elements constituting the origin of the
nerves.
[Footnote 115: Hammond assumes such a form of myelitis in his
classification of inflammations limited to the anterior part of the
gray matter of the spinal cord:
1. Inflammation of motor and trophic nerve-cells: (_a_) Infantile
spinal paralysis; (_b_) Spinal paralysis of adults; (_c_)
Pseudo-hypertrophic spinal paralysis.
2. Inflammation of motor cells: (_a_) Glosso-labia-laryngeal
paralysis.
3. Inflammation of trophic cells: (_a_) Progressive muscular atrophy;
(_b_) Progressive facial atrophy (_Dis. Nerv. Syst._, 6th ed., p.
464).
We think this classification open to several fundamental criticisms.]
Whatever be the starting-point, however, it is very evident that the
morbid process soon involves all the tissues contained in the gray
matter of the anterior horns, and constitutes, therefore, a real
anterior poliomyelitis.
A question of much interest is the relation to this of the lesions of
the anterior roots and of the white columns. Is the atrophy of
nerve-tubes a passive consequence of their separation from the
ganglionic cells, the sclerosis a secondary consequence of this? or is
the sclerosis the cause of the atrophy, itself the result of an
irritation propagated downward from the myelitic focus, according to
the usual law for secondary degenerations in motor tracts? or,
finally, is it a residuum of a leucomyelitis (or of the white
substance), complicating by simple extension the inflammation of the
gray substance?
Review of the autopsies recorded would indicate that the lesions in
question are brought about sometimes in one, sometimes in another, of
these ways—sometimes even, as in Leyden's case, by extension from a
meningitis. That sclerosis of the white columns is most frequently a
secondary degeneration is indicated by the frequency with which it
appears below the lesion of the cornua, by the rarity with which it is
found above, and also by the general proportion between its intensity
and that of the disease of the gray matter.
We have devoted so much space to consideration of spinal-cord lesions,
because they are by far the most constant and the most important;
after these rank the structural alterations of the muscles, which
received for a while such a preponderance of attention.
Hammond has studied the progress of these changes on the living
subject by fragments of fibre successively removed with Duchenne's
harpoon. In an incipient stage of degeneration the fibrillæ are found
to be irregular and torn,[116] the transverse striæ dim; oil-globules
are seen arranged according to the long axis of the fibre. In a more
advanced stage the transverse striæ nearly disappear, the oil-globules
are in large numbers, and fat-corpuscles are also abundant. Finally,
the whole specimen is seen as a mass of air-globules. Six weeks later,
however, these had in turn disappeared, and there remained a mass of
connective tissue.
[Footnote 116: Though, from the method of removal, this appearance
cannot be considered as certainly pathological.]
This series of changes, however, does not always take place, as
Hammond himself recognizes. Laborde[117] first described a granular
form of muscle atrophy, where the muscular substance gradually wastes
away without ever becoming fatty, and leaving a transparent and
hyaline sheath. The two forms of fatty and of simple atrophy can be
distinguished by the naked eye. In the latter the muscle begins by
being thinner or lighter and softer than usual, ultimately turning
light brown. The fatty muscle becomes a homogeneous yellowish-white,
diversified by occasional remnants of reddish fibres.
[Footnote 117: _Loc. cit._, p. 131.]
{1143} Proliferation of the interstitial connective tissue may be
combined with either simple or fatty atrophy. A combination of
abundant sclerosis and abundant fatty infiltration may lead to a
pseudo-hypertrophy of the muscles.
“There cannot be the slightest doubt,” observes Erb, “that the lesions
described constitute a degenerative atrophy similar to what may be
caused by section or sense traumatism of a peripheric nerve.”
The peripheric nerves have been much less thoroughly studied than the
spinal cord. Leyden first directed special attention to the nerves. He
found the sciatic altered in two cases,[118] in the first by an
interstitial neuritis; in the second by partial atrophy. In 1880 the
same writer, in an extensive article on poliomyelitis and
neuritis,[119] greatly extends his views as earlier expressed. Not
only does he claim the coexistence of neuritis with spinal-cord
disease in atrophic paralysis, but thinks that many cases of this, and
also of other forms of paralysis, “lately supposed to originate in the
spinal cord, may really begin in any part of the motor apparatus,”
thence sometimes generalize throughout the whole apparatus, sometimes
remain limited to the original portion affected. Thus, progressive
muscular atrophy may sometimes begin in the nerves, sometimes in the
muscles, and sometimes in the ganglionic cells of the cord; and this
variety of origin explains the discrepancies of opinion which have
been held upon the nature of this disease. Similarly, all forms of
acute or chronic atrophic paralysis in either children or adults may
begin in either the nerves or cord, thence become generalized to both,
or remain limited to one part of the spinal motor system. Cases of
atrophic paralysis which recover are probably not cases of
poliomyelitis at all, but of multiple neuritis, rheumatic, traumatic,
or infectious in nature. The regeneration of peripheric nerves is a
well-demonstrated possibility, but not that of the cells of the cord.
Lead-paralysis is usually confined to the nerves, but sometimes
extends to the cord. In diphtheritic paralysis Buhl has found
injection, thickening, and granular infiltration of nerves at the
union of their anterior and posterior roots;[120] and as long ago as
1876, Dejerine, in a case of atrophic paralysis in a syphilitic woman,
found varicose swelling of the medullary sheath in the nerves of the
paralyzed lower extremities, together with heaping up of the myeline
into large drops, colored black in glycerin and osmic-acid
preparations. Coincidently, in the cord, at the origin of the same
nerves, the number of motor-cells was diminished, and of those that
remained the prolongations, and even the body, of the cell were
atrophied.[121]
[Footnote 118: Cases 34 and 35 of Table V., quoted from _Arch. de
Psychiatrie_, Bd. vi., 1876.]
[Footnote 119: _Zeitschrift für Klin. Med._, 1880.]
[Footnote 120: _Zeitschrift für Biol._, 1867.]
[Footnote 121: _Arch. de Phys._, 1876.]
These views of Leyden's are extremely interesting, and should
stimulate future research into the condition of nerves in all cases of
atrophic paralysis. It is quite incorrect to say, as Archambault and
Damaschino have recently done,[122] that Leyden denies the existence
of anterior poliomyelitis in such cases, especially in such as prove
permanent. He only insists on the frequent coincidence of neuritis, on
a varying point of departure for the morbid process, and on the
probability that in cases of recovery this process has always remained
peripheric.
[Footnote 122: _Le Union méd._, 1883, 7, 35, case quoted in Table V.
It is much to be regretted that Damaschino, who strongly controverts
Leyden's views, did not examine the nerves in his own most interesting
case.]