tissues of parts which are the seat of the appearances above described
have received the special study of many excellent observers[9] in
recent times, and are now well understood. A section through the
thickened skin or a tubercle shows the corium and underlying
connective tissue infiltrated with round cells, as in lupus and
syphilis; in other words, converted into "granulation tissue." This
change first takes place along the course of the cutaneous vessels and
glands, penetrating more deeply and forming a firmer cell new-growth
in proportion to duration, the cells being enclosed in a coarse
meshwork of fibrous tissue, and encroaching upon the various
structures of the skin, so as to produce atrophy and finally
destruction of all its characteristic tissues. This cell-infiltration
may of itself undergo later changes, as fatty degeneration and
softening (ulceration). The lymph-glands and corpuscles assume a
special fatty metamorphosis. An examination of the tubercles upon the
mucous membrane reveals the same small-celled new-growth. In the
nerve-tissues also marked structural changes are found, both in the
central and peripheral systems, in the anæsthetic form of the disease.
In many cases the posterior segments of the gray cornua and the fibres
of the commissure, as well as the nerves of the extremities, have been
found altered by inflammation, which will account for the disordered
sensibility and the subsequent disturbances of nutrition, muscular
atrophy, etc. The nerve-trunks are often to be felt beneath the skin,
thickened and sensitive on pressure. The chronic cell-infiltration
affects the fibrous structure of the outer sheath, the neurilemma, and
the septa between the nerve-bundles, producing fatty metamorphosis and
atrophy of the nerve-bundles. Similar cell-infiltrations are found
also in the connective tissue of all the internal organs of the body,
which lead to destructive processes in their respective structures.
[Footnote 9: Boeck and Danielssen, _Traité de la Spedalskhed_, Paris,
1848; Virchow, _Die Krankhaften Geschwülste_; Kaposi in _Hebra's
Lehrbuch der Hautkrankheiten_; Monasterski, _Vierteljahressch. für
Derm. u. Syph._, 1879, p. 203; Hansen, _Virchow's Archiv_, Band 79,
1880; Neisser, _Virchow's Archiv_, Band 84, 1881; Cornil et Souchard,
_Annales de Derm. et de Syph._, 1881, No. 4.]
Within the last two years repeated observations have been made which
confirm the statement published by Hansen in 1873, that a peculiar
bacterium occurs in leprous tissues, which, it is claimed, establishes
the parasitic nature of the affection. These examinations have been
carried on with leprous material derived from many parts of the world,
and the results have been uniform. Within the round cells which
characterize the cutaneous neoplasms, both in the distinct tubercles
and the diffused {792} infiltrations, small agglomerations of minute
rod- or staff-like bodies (bacilli) are found, arranged in parallel
rows or placed end to end. Their length is one-half or three-fourths
the diameter of a red blood-globule, and their breadth is one-fourth
their length. With them minute granular particles are seen in the
cells. They occur in greatest numbers in the cells of the upper layers
of the true skin, which are considerably swollen by their presence.
They never penetrate the epithelial layer, nor are they found in
epithelial cells in any position. When the protoplasm of the cell is
interfered with by the later tissue-changes of the disease, the
bacillus perishes. They are found not only in the leprous cells, but
also in those of the connective tissue running between the
agglomerated masses of the former. Between the leprous cells and the
filaments of connective tissue but few free bacilli are seen. The
neoplasms of the mucous membrane and of many organs of the body have
been found to contain them also. In the blood they have been detected
by some observers. Their presence in the nerve-tissues is of
importance as throwing light upon the question of the specific or
inflammatory nature of the morbid processes above described as
affecting them. If we regard the bacteria as pathognomonic of leprous
tissue-changes, their occurrence, recognized in the cells penetrating
between the fibres of the peripheral nerves, would seem to make all
primary structural changes identical, and the anæsthetic as much as
the tubercular form the direct result of their presence. Neisser draws
the following conclusions from his investigations: "Leprosy is a real
bacterial disease, caused by a special kind of bacterium. The bacilli
appear in the tissues as such, or more probably as spores, and remain
for a longer or shorter time in a state of incubation, according to
circumstances, in dépôts, perhaps in the lymph-glands. This period,
much longer than in other infective diseases, is in proportion to the
physiological resistance of the human organism compared with the
feeble developing power of the bacilli. It, as well as the course of
the disease, is more rapid in tropical countries than in Europe. From
these dépôts the disease extends throughout the body in those portions
of the skin most exposed, the face, hands, elbows, knees, and into the
peripheral nerves. The other organs are less freely invaded. The
bacilli excite inflammation, and by a specific action transform the
migrating cell into the leprous cell. Leprosy is probably an
infectious disease, and its specific products are contagious--viz. the
leprous cells of the tubercles, the tissue-fluids, and the pus
containing bacilli or viable spores. On the other hand, the pus may
not always be infectious, as the fluid contained in the bullæ is not."
It must be said that the bacterial nature of leprosy, if established
in accordance with the above observations, furnishes a satisfactory
basis of explanation of all facts, historical, clinical, and
pathological, which have so long been awaiting solution. The inability
of the parasite to penetrate the epithelial layer of the skin and
mucous membrane explains why contagion is so difficult, and why the
ulcerative tubercular form would be more favorable to such
transference than the anæsthetic variety.
DIAGNOSIS.--Leprosy in some of its early appearances may be readily
confounded with vitiligo, morphoea, pemphigus, lupus, and syphilis. In
some cases its prodromal manifestations cannot be positively
diagnosticated until other symptoms have developed, which by
concurrence establish their true significance. Such are the
pemphigus-like bullæ, the {793} pigment-changes, and the smaller
tubercular efflorescences. In regions where the disease occurs only by
importation, and in the so-called sporadic cases, it is not at all
strange that it should fail of recognition, even in well-advanced
forms, unless the observer is acquainted with its whole
symptomatology. On the other hand, there is no disease which presents
more strikingly characteristic features in its advanced stages.
PROGNOSIS.--Leprosy is almost uniformly a fatal affection, and its
course toward this termination varies but slightly under the most
diverse conditions of life. Its development and progress are naturally
more rapid under circumstances of least individual resistance, where
food is poor and scanty, where extremes of climate are most felt,
where the constitution of the individual or nation is debilitated by
previous disease, as that of the Hawaiians by syphilis, or where no
proper professional care is employed. It has been believed that a
change of residence from infected to non-leprous regions would retard
its advance or avert its appearance in those supposed to be
hereditarily disposed; but the former effect follows probably only so
far as the general condition of the patient is affected by the change,
as in other constitutional disorders, and the latter is necessarily a
matter wholly of conjecture. No case of leprosy in the Norwegian
colony in our North-western States has ceased to progress after
arrival toward its fatal ending, even if this has been somewhat
delayed in individual cases under more generous ways of living. If it
could be known that a child born in Norway had escaped leprosy by
removal to America, we should not, if we accept the bacterial origin
of the disease, consider that climate or other mysterious influences
had overcome its inherited tendencies, but that it had been taken away
from the chance of direct inoculation. It is stated that very rarely
cases cease to progress beyond certain stages even in countries where
the disease is endemic. The course, as has been stated, varies
according to the clinical form, the duration of the tubercular variety
being on an average but one-half that of the purely anæsthetic type.
Leprosy may be called the slow disease, its period of incubation, so
far as this can be determined, extending from one to several years,
its prodromal stage lasting often several more years, and its
well-developed forms requiring at times more than twenty years to
destroy the patient. Cases sometimes prove fatal, however, in a single
year.
TREATMENT.--In a disease which affects so many of the races and such
great numbers of mankind, which has been for centuries the object of
special attention on the part of physicians, and of late years of
government commissions and of eminent pathologists, it is evident that
every remedy which the materia medica includes, as well as those of
merely popular reputation in the widely-diverse geographical regions
in which it prevails, must have been employed in its treatment. None
of them exert any specific action upon it; it remains incurable. Every
year some new article is employed with the usual claims of success
which accompany the introduction of new remedies, but they merely
swell the long list of failures in the therapeutics of the affection.
Still, leprosy is influenced somewhat by medical care; life may be
prolonged and made more comfortable. To this end we may employ
remedies which are capable of improving and maintaining the
constitutional powers of resistance to the disease, such as are found
of service in other chronic wasting affections. {794} The patient is
to be put in as healthy ways of living as possible, removed from
debilitating localities, and given generous diet and tonics, as iron
and quinia. Several new drugs which seem to stimulate the nutrition
and produce temporary improvement in the local and general symptoms
have lately been widely employed, as Gurjun balsam and chaulmoogra
oil, but they have wrought no cure. Digestion is to be aided,
diarrhoea to be checked, and disturbances of respiration to be
alleviated. Local treatment is also of service. The tubercles may
sometimes be made to disappear--partly, at least--by stimulating
applications, and ulcers made to heal by cauterization and other
well-known methods of dressing. These ulcers and their secretions
should be regarded as possible sources of infection by attendants and
members of the patient's household. For the anæsthetic alterations in
the tissues but little can be done locally. If the bacterial origin
and causation of the disease be eventually established, its future
extinction must be based upon studies directed to the nature and mode
of protection against this organism. Collectively, the disease should
be treated by every nation by thorough segregation, and importation
should be prevented by the most rigid quarantine laws.
{795}
EPIDEMIC CEREBRO-SPINAL MENINGITIS.
BY ALFRED STILLÉ, M.D., LL.D.
DEFINITION.--A febrile, and often malignant, but non-contagious
disease of unknown origin; usually occurring as a local epidemic;
confined hitherto to the North American and European continents, and
to the vicinity of the latter; characterized by its rapid and
irregular course, and usually by a tetanic rigidity or retraction of
the neck, a tendency to disorganization of the blood, and the
formation of inflammatory exudates beneath the membranes of the brain
and spinal cord.
SYNONYMS.--Spotted fever; petechial fever; malignant purpuric fever;
malignant purpura; pestilential purpura; black death; typhus
petechialis; typhus syncopalis; febris nigra; febbre
soporoso-convulsivo; tifo apoplettico tetanico; fièvre
cérébro-spinale; typhus cérébro-spinale; phrenitis typhodes; epidemic
meningitis; epidemic cerebro-spinal meningitis; malignant meningitis;
typhoid meningitis; méningite cérébro-spinale épidémique; méningite
cérébro-rachidienne; Genickkrampf; Genickstarre.
The names which have been given to this disease convey more or less
distinctly one or the other of two ideas: 1st, that the disease is
essentially a blood-disorder; and 2d, that it is an inflammation of
the cerebro-spinal meninges. Under the first head belong the following
names: Malignant purpuric fever; malignant purpura; pestilential
purpura; petechial fever; spotted fever; febris nigra; black death,
etc. Under the second head belong epidemic cerebro-spinal meningitis;
epidemic meningitis; malignant meningitis; typhoid meningitis, etc. As
partaking of the qualities of both categories may be cited the names
cerebro-spinal fever and fever with cerebro-spinal meningitis. In
regard to all those of the first class it is sufficient to repeat the
criticism made by the early American writers who described this
disease after having largely studied it. One only of them need be
cited, because he expresses the opinion of all. Miner, writing in
1822, said: "It is quite unfortunate that a single symptom (petechiæ),
and one, too, that is wanting in a great majority of cases, should
have been seized upon to give it the odious and deceptive name of
spotted fever, as that name has been applied by European writers to a
very different kind of fever." Among the names given to the disease,
cerebro-spinal fever is perhaps the least suitable and the least in
harmony with the principles of scientific nomenclature. It is one of
those terms which may be pardoned when used by the laity, but which
educated physicians ought not tolerate. Parallel examples may be found
in such compounds as brain-fever, lung-fever, gastric-fever, and, most
unfortunate of all, enteric fever. The first three of these are {796}
inflammations, pure and simple, of the brain, lung, and stomach; and,
after their example, cerebro-spinal meningitis would be, what it is
not, merely an inflammation of the membranes of the brain and spinal
marrow. The name of the remaining disease has only to be turned into
English and called intestinal fever to demonstrate its defects. It is
evident that other diseases--and dysentery in particular--are equally
entitled to be called enteric fever. Moreover, there are cases of
enteric fever in which death takes place so early that the intestinal
lesion is undeveloped, and the fatal issue must be attributed to the
fever-poison in the blood or else to the changes it has wrought in
that fluid. Analogous illustrations abound in the history of the
eruptive fevers. The disease we are studying presents another
affection in which the septic element sometimes so far overrides the
inflammatory as to destroy life before the latter has developed
characteristic tissue-changes. There may be no valid objection against
classing it among the fevers, but there can be no excuse for
denominating it cerebro-spinal fever. The very reasons that militate
against its being regarded as a meningitis forbid its being considered
as a meningeal fever. But if it is a meningitis, inchoate or complete,
then the prefix epidemic denotes its constitutional nature and its
probable blood origin, and a term is employed which is descriptive and
accurate, and not misleading. Moreover, the term epidemic indicates,
or at least implies, the characteristic type of the disease, which is
asthenic and sometimes more or less typhoidal, just as other
inflammatory diseases become so in their epidemic form--_e.g._
pneumonia, bronchitis (influenza), dysentery, etc.
There ought to be no doubt whether epidemic meningitis should be
classed with general diseases or with inflammations. It is excluded
from the latter class by the total absence of any tangible external
cause from its causation, as well as by its frequent fatal termination
before the characteristic signs of inflammation have had time to form,
or because the peculiar type of the disease prevents their
development. It belongs to the former class because it is epidemic in
the largest sense, its outbreaks occurring simultaneously in remote
parts of the earth and independently of all cognizable celestial or
terrestrial influences. In this as in other elements of its pathology
the disease stands absolutely alone. While the acute affections of the
pulmonary and digestive organs, which were just now alluded to, affect
large districts, and even sweep over a whole continent, epidemic
meningitis breaks out in limited localities, and may for years prevail
in a populous city within a hundred miles of another still more
populous which during that time may altogether escape its ravages. Of
this curious fact the cities of Philadelphia and New York present a
striking illustration. Since, then, we are ignorant of the
circumstances under which the disease arises, and since, as will more
distinctly appear later on, its several forms really include quite
various morbid conditions, we are compelled to consider it as
occupying a peculiar and exceptional nosological position.
HISTORY.--Previous to the present century the existence of this
disease can hardly be demonstrated. And yet Dr. B. W. Richardson
believed that some faint traces of it could be discovered, as in the
following statement:[1] "The great plague which visited Constantinople
in 543, and which Procopius and Enagrius described, the plague of
{797} hallucination, drowsiness, slumbering, distraction, and ardent
fever, with eruption on the skin of black pimples the size of a
lentil,--this plague, which usually killed in five days, and left many
who recovered with withered limbs, wasted tongues, stammering speech
or such utterance of sound that their words could not be
distinguished,--this plague, which had passed into mythical learning
under the name of cerebro-spinal meningitis, has also in our time
reappeared." The concluding statement in regard to the name of the
plague is quite erroneous, and there is nothing in the description
which distinctively applies to the disease we are examining. On the
other hand, we know that Procopius wrote a history of the Oriental
plague, which invaded Europe for the first time at the very date above
given. It had as a distinctive symptom the well-known inguinal bubo,
and there is no mention whatever, in the descriptions of it that have
survived, of the tetanoid symptoms belonging to epidemic meningitis.
In 1802 an epidemic occurred at Roetlingen in Franconia which had a
certain resemblance to the subject of this article, for it was
characterized by lacerating pains in the back of the neck. According
to Hecker, this was the sweating sickness which had ravaged various
parts of Europe during the Middle Ages, and of which limited outbreaks
still recur. In 1880 such a one took place at l'Ile d'Oléron in
France, and many of the patients were affected with tonic or clonic
spasms, both general and local, but not, apparently, opisthotonic.[2]
[Footnote 1: _Diseases of Modern Life_, p. 16.]
[Footnote 2: Pineau, _Archives gén. de méd._, tom. i., 1882, pp. 25,
169.]
If epidemic meningitis occurred before the nineteenth century, it must
have been confounded with other affections, but when we consider its
characteristic symptoms such an error seems improbable. The
comparatively rare resort at that time to post-mortem examinations,
particularly of the cranial and spinal cavities, may in part account
for such a confusion of ideas; and even when dissections were made,
the skill to interpret the discovered lesions was possessed by few. It
has been thought that in the latter part of the last century some
cases of this disease were seen and described, although their
nosological value was unrecognized. Thus, Stoll[3] speaks of a young
soldier who was seized with a pain in the back of the head and neck,
and who was affected with opisthotonos before he died. On examination
pus was found between the arachnoid and the pia mater. The first clear
and unquestionable description of epidemic meningitis was published in
1805, first by Vieusseux and directly afterward by Mathey.[4] The
disease appeared at Geneva in the spring of the year, in a family
composed of a woman and three children, of whom two of the latter died
within twenty-four hours. A fortnight later four children in a
neighboring family died of it after fourteen or fifteen hours'
illness, and a young man in an adjoining house, being attacked, died
the same night, with his whole body of a violet color. The disease
ceased during the spring, after having destroyed thirty-three lives.
Its distinctive features were an abrupt attack during the night,
bilious vomiting, excruciating headache, rigidity of the spine,
difficult deglutition, convulsions, nocturnal paroxysms, petechiæ, and
death in from twelve hours to five days. Vieusseux calls it "a
malignant non-contagious fever," and Mathey gives as the lesions
revealed by dissection a gelatinous {798} exudation covering the
convex surface of the brain, and a yellow puriform matter upon its
posterior aspect, upon the optic commissure, the inferior surface of
the cerebellum, and the medulla oblongata.
[Footnote 3: Quoted by Boudin, _Hist. du typhus cérébro-spinal_, p.
5.]
[Footnote 4: _Journ. de Méd., Chirurg. et Pharm., etc._, an. xiv.,
tom. xi, pp. 163, 243.]
After its first appearance at Geneva the disease does not seem to have
extended in any direction from that place as a centre, but we next
hear of it at two points remote from it and from one another--Germany
and the United States. From the former it extended to the conterminous
countries, Bavaria, Holland, and the east of France, where, however,
it prevailed neither extensively nor fatally, and soon died out; while
in America it first appeared at Medfield, Mass., in 1806. The European
epidemic was faintly felt in England the following year, and between
that time and 1816 it prevailed at several places in the east of
France, and slightly at Paris, while during the corresponding period
it had extended through New England into Canada, New York,
Pennsylvania, and several Western and South-western States. It is a
noteworthy fact that on both sides of the Atlantic it ceased in the
same year (1816). During the six following years we can discover no
trace of its existence, but in 1822-23 it reappeared at Vesoul in
France, and at Middletown, Connecticut, and does not seem to have
extended beyond those places. Again, after an interval of five years,
in 1828 it was heard of in Trumbull co., Ohio, two years later at
Sunderland in England, and three years afterward (in 1833) at Naples.
After four years of quiescence the disease entered upon a wider and
more destructive career than ever before, which was almost
uninterrupted from 1837 to 1850. During the first two years of its
recurrence in Europe it was confined almost wholly to France. It began
in the southern departments, with Bayonne as a centre, and extended
gradually westward and northward, in some places attacking only
military garrisons and in others only civilians. Elsewhere the
predilection was reversed, or, again, civilians and soldiers were
equally affected. As Boudin has pointed out, "it located itself in
certain districts; in garrison-towns it seemed to affect certain
barracks only, and in them only certain rooms. In one place it broke
out in a prison and spared the soldiers; in another its victims were
among the soldiers and the citizens, while the prisoners were
untouched." Thus the disease spread over the whole of France, and was
more fatal almost everywhere else than in Paris itself. Almost at the
gates of the capital, at Versailles, and among the garrison, it was
very destructive in 1839, causing a mortality among those attacked of
from 50 to 75 per cent. About the same time it occasioned a great
mortality at other military posts, especially at Rochefort and Metz,
and in 1840-41 at Strasbourg. In 1843 the disease had almost ceased to
prevail in France, but in 1846 it reappeared at Lyons, and in the
following years, and until 1849, affected the garrisons of Orléans,
Cambrai, Saint-Étienne, Metz again, Lunéville, Dijon, Bourges, and
Toulon. In some of these places the military experienced five, and
even seven, successive epidemics. Meanwhile, the disease spread to
Algeria (1839-47), and to Italy in the former year--not, however, on
the confines of France, but at Naples and in the Romagna, whence it
extended to Sicily and Gibraltar, and did not cease there until 1845.
In 1839 it first showed itself in Denmark, and remained for about
three years, while in 1846 it "appeared in the {799} majority of the
workhouses of Ireland," and in the spring of the same year it occurred
in England, at Liverpool and Rochester.