to the syphilis, when a syphilitic person has any disorder there is
only one positive way of determining during life how far said disorder
is specific—namely, by studying its amenability to antisyphilitic
treatment. In approaching the question whether a lesion found after
death is specific or not, of course such a therapeutic test as that
just given is inapplicable. We can only study as to the coexistence of
the lesion in consideration with other lesions known to be specific.
Such coexistence of course does not absolutely prove the specific
nature of a nutritive change, but renders such nature exceedingly
probable.
What has just been said foreshadows the method in which the subject in
hand is to be here examined, and the present article naturally divides
itself into two sections—the first considering the coexistence of
anatomical alterations occurring in the cerebral substance with
syphilitic affections of the brain-membranes or blood-vessels, the
second being a clinical study of syphilitic insanity.
In looking over the literature of the subject I have found the
following cases in which a cerebral sclerotic affection coincided with
a gummatous disease of the membrane. Gros and Lancereaux[60] report a
case having a clear syphilitic history in which the dura mater was
adherent to the skull. The pia mater was not adherent. Beneath, upon
the vault of the brain, was a gelatinous exudation. The upper cerebral
substance was indurated, and pronounced by Robin after microscopic
examination to be sclerosed. At the base of the brain there were
atheromatous arteries and spots of marked softening.
[Footnote 60: _Affec. Nerv. Syphilis_, 1861, p. 245.]
Jos. J. Brown[61] reports a case in which the symptoms were
melancholia, excessive irritability, violent outbursts of temper, very
positive delusions, disordered gait, ending in dementia. At the
autopsy, which was very exhaustive, extensive syphilitic disease of
the vessels of the brain and spinal cord was found. The pia mater was
not adherent to the brain. The convolutions, particularly of the
frontal and parietal lobes, were atrophied, with very wide sulci
filled with bloody serum. The neuroglia of these convolutions was much
increased, and “appeared to be more molecular than normal, the cells
were degenerated, and in many places had disappeared, their places
being only occupied by some granules.” These changes were most marked
in the frontal convolutions.
[Footnote 61: _Journ. Ment. Sci._, July, 1875, p. 271.]
H. Schule reports[62] a very carefully and meritoriously studied case.
The symptoms during life exactly simulated those of dementia
paralytica. The affection commenced with an entire change in the
disposition of the patient; from being taciturn, quiet, and very
parsimonious, he became very excited, restless, and desiring
continuously to buy in the shops. Then failure of memory, marked sense
of well-being, carelessness and indifference for the future, developed
consentaneously with failure of the power of walking, trembling of the
hands, inequality of the pupils, and hesitating speech. There was next
a period of melancholy, which was in time followed by continuous
failure of mental and motor powers, and {1019} very pronounced
delirium of grandeur, ending in complete dementia. Death finally
occurred from universal palsy, with progressive increase of the motor
symptoms. At the autopsy characteristic syphilitic lesions were found
in the skull, dura mater, larynx, liver, intestines, and testicles.
The brain presented the macroscopic and microscopic characters of
sclerosis and atrophy; the neuroglia was much increased, full of
numerous nuclei, the ganglion-cells destroyed. The vessels were very
much diseased, some reduced to cords; their walls were greatly
thickened, and full of long spindle-shaped cells, sometimes also
containing fatty granules.
[Footnote 62: _Allgem. Zeitschrift f. Psychiatrie_, xxviii. 171, 172.]
C. E. Stedman and Robt. T. Edes report[63] a case in which the
symptoms were failure of health, ptosis, trigeminal palsy with pain
(anæsthesia dolorosa), finally mental failure with gradual loss of
power of motion and sensation. At the autopsy the following conditions
were noted: apex of the temporal lobe adherent to dura mater and
softened; exuded lymph in neighborhood of optic chiasm; sclerosis of
right Gasserian ganglion, as shown in a marked increase of the
neuroglia; degeneration of the basal arteries of the brain.
[Footnote 63: _American Journ. Med. Sciences_, lxix. 433.]
These cases are sufficient to demonstrate that sclerosis of the
brain-substance not only may coexist with a brain lesion which is
certainly specific in its character, but may also present the
appearance of having developed pari passu with that lesion and from
the same cause.
It has already been stated in this article that cerebral meningeal
syphilis may coexist with various forms of insanity, and cases have
been cited in proof thereof. It is of course very probable that in
some of such cases there has been that double lesion of membrane and
gray brain matter which has just been demonstrated by report of
autopsies; but if we find that there is a syphilitic insanity, which
exists without evidences of meningeal syphilis, and is capable of
being cured by antispecific treatment, such insanity must be
considered as representing the disease of the gray matter of the
brain. Medical literature is so gigantic that it is impossible to
exhaust it, but the following list of cases is amply sufficient to
prove the point at issue—namely, that there is a syphilitic insanity
which exists without obvious meningeal disease, and is capable of
being cured by antisyphilitic treatment:
----+-----------------------+--------------------+------------------
No. | Reporter and Journal. | Symptoms. |Results.—Remarks.
----+-----------------------+--------------------+------------------
1 | Luis Streisand | Epilepsy, delirium | Rapid cure with
| _Die Lues als Ursache | of exaltation, | mercury.
| der Dementia_, Inaug. | alteration of |
| Diss., Berlin, 1878. | speech, headache, |
| | failure of memory. |
----+-----------------------+--------------------+------------------
2 | _Ibid._ | Delusions, | Cure with
| | delirium, general | mercury.
| | mania, great |
| | muscular weakness. |
----+-----------------------+--------------------+------------------
3 | Müller of Leutkirch | Symptoms resembling| Cure by iodide of
| _Journ. of Mental | general paralysis, | potassium.
| Dis._, 1873-74, 561. | and diagnosis of |
| | such made until a |
| | sternal node was |
| | discovered. |
----+-----------------------+--------------------+------------------
4 | Esmarch and W. Jersen | Sleeplessness, | Cure by mercury.
| _Allgem. Zeitschrift | great excitement, |
| f. Psychiatrie_. | restlessness, |
| | great activity, |
| | incoherence, and |
| | violence. |
----+-----------------------+--------------------+------------------
5 | Leidesdorf | Complete mania; | Complete cure by
| _Medizin. | played with his | iodide of
| Jahrbucher_, xx., | excrement, and | potassium.
| 1864, 1. | entirely |
| | irrational. |
----+-----------------------+--------------------+------------------
6 | Beauregard | Symptoms resembling| Cure by iodide of
| _Gaz. hébdom. de Sci. | those of general | potassium.
| méd. de Bordeaux_, | paralysis. |
| 1880, p. 64. | |
----+-----------------------+--------------------+------------------
7 | M. Rendu | Loss of memory, | Mercurial
| _Ibid._ | headache, | treatment, cure.
| | irregularity of |
| | pupils, ambitious |
| | delirium, periods |
| | of excitement, |
| | others of |
| | depression, |
| | embarrassment of |
| | speech, access of |
| | furious delirium, |
| | ending in stupor. | {1020}
----+-----------------------+--------------------+------------------
8 | M. Rendu | Hypochondria, | Mercurial
| _Gaz. hébdom. de Sci. | irregularity of | treatment, cure.
| méd. de Bordeaux_, | pupils, headache, |
| 1880, p. 64. | failure of memory, |
| | melancholy, stupor.|
----+-----------------------+--------------------+------------------
9 | Albrecht Erlenmeyer | Melancholia with | Iodide of
| _Die Luëtischen | hypochondriasis, | potassium, cure.
| Psychosen_, Neuwied, | sleeplessness, |
| 1877. | fear of men, and |
| | belief they were |
| | all leagued |
| | against him. |
----+-----------------------+--------------------+------------------
10 | _Ibid._ | Religious | Iodide of
| | melancholia, with | potassium, cure.
| | two attempts at |
| | suicide, ending in |
| | mania. |
----+-----------------------+--------------------+------------------
11 | _Ibid._ | At times very | Iodide of
| | violent, yelling, | potassium, cure.
| | shrieking, |
| | destroying |
| | everything she |
| | could get hands |
| | on, at times |
| | erotomania; no |
| | distinct history |
| | of infection, but |
| | her habits known |
| | to be bad, and had |
| | bone ozæna and |
| | other physical |
| | syphilitic signs. |
----+-----------------------+--------------------+------------------
12 | _Ibid._ | Epileptic attack | Cured by
| | followed by a long | mercurial
| | soporose condition,| inunction.
| | ending in mental |
| | confusion, he not |
| | knowing his nearest|
| | friends, etc.; |
| | almost dementia. |
----+-----------------------+--------------------+------------------
13 | _Ibid._ | Great fear of | Cured by
| | gensd'armes, etc., | mercurial
| | mania, with | inunctions with
| | hallucinations, | iodide
| | loud crying, | internally;
| | yelling, etc., | subsequently
| | then convulsion, | return of
| | followed by great | convulsions,
| | difficulty of | followed by
| | speech. | hemiplegia and
| | | death.
----+-----------------------+--------------------+------------------
14 | _Ibid._ | Great unnatural | Iodide of
| | vivacity and | potassium, cure.
| | loquacity, wanted | Attended to
| | to buy everything, | business, and
| | bragged of enormous| seems as well as
| | gains at play, | before. Relapsed.
| | etc.; some trouble | (See Symptoms.)
| | of speech. |
| | |
| _Ibid._ | Fifteen months | Failure of
| Relapse of Case 14. | after discharge | various
| | from asylum | anti-specific
| | relapse; symptoms | treatment.
| | developing very |
| | rapidly, delirium |
| | of grandeur of the |
| | most aggravated |
| | type, with marked |
| | progressive |
| | dementia, failure |
| | of power of speech,|
| | and finally of |
| | locomotion. |
----+-----------------------+--------------------+------------------
15 | A. Erlenmeyer | Failure of mental | Iodide of
| _Die Luëtischen_, | powers, inequality | potassium in
| etc. | of pupils, | ascending doses
| | trembling of lip | failed. Recovery
| | when speaking, | under mercurial
| | uncertainty of | inunctions.
| | gait, almost |
| | entire loss of |
| | memory, once |
| | temporary ptosis |
| | and strabismus. |
----+-----------------------+--------------------+------------------
16 | _Ibid._ | Failure of mental | Iodide of
| | powers, pronounced | potassium,
| | delirium of | corrosive-
| | grandeur, | sublimate
| | hallucinations of | injections. Cure.
| | hearing, failure of|
| | memory, strabismus |
| | and ptosis coming |
| | on late. |
----+-----------------------+--------------------+------------------
17 | _Ibid._ | Failure of memory | Cure with use of
| | and mental powers, | iodide and
| | slight ideas of | mercurial
| | grandeur, | inunctions.
| | disturbance of |
| | sensibility and |
| | motility, aphasia |
| | coming on late. |
----+-----------------------+--------------------+------------------
18 | _Ibid._ | Melancholy, great | Iodide of
| | excitability, | potassium failed;
| | ideas of grandeur; | mercurial course
| | after a long time | improved; joint
| | sudden ptosis and | use cured
| | strabismus. | patient.
----+-----------------------+--------------------+------------------
19 | _Ibid._ | Various cerebral |
| | nerve palsies, |
| | great relief by |
| | use of mercurial |
| | inunctions, then |
| | development of |
| | great excitement, |
| | delirium of |
| | grandeur, failure |
| | of memory and |
| | mental powers, and |
| | finally death from |
| | apoplexy; no |
| | autopsy. |
----+-----------------------+--------------------+------------------
20 | J. B. Chapin | Melancholia with | Iodide of
| _Amer. Journ. | attempted suicide, | potassium, cure.
| Insanity_, vol. xv. | epilepsy, headache,|
| p. 249. | somnolent spells. |
----+-----------------------+--------------------+------------------
21 | _Ibid._ | Acute mania, noisy,| Iodide of
| | very destructive; | potassium, cure.
| | syphilitic disease |
| | of tibia. |
----+-----------------------+--------------------+------------------
22 | Snel | Maniacal | Cured by specific
| | excitement. | treatment.
----+-----------------------+--------------------+------------------
23 | Wm. Smith | Apathetic | Rapidly cured by
| _Brit. Med. Journ._, | melancholy, | conjoint use of
| July, 1868, p. 30. | indelicate, | iodide and
| | speaking only in | mercurials. The
| | monosyllables, and | symptoms first
| | much of the time | developed 3
| | not at all, sullen | months after
| | and menacing. | chancre.
----+-----------------------+--------------------+------------------
A study of the brief analyses of the symptoms just given shows that
syphilitic disease of the brain may cause any form of mania, but that
the symptoms, however various they may be at first, end almost always
in dementia unless relieved.
Of all the forms of insanity, general paralysis is most closely and
frequently simulated by specific brain disease. The exact relation of
the diathesis to true, incurable, general paralysis it is very
difficult to {1021} determine. It seems well established that amongst
persons suffering from this disorder the proportion of syphilitics is
not only much larger than normal, but also much larger than in other
forms of insanity. Thus, E. Mendel[64] found that in 146 cases of
general paralysis, 109, or 75 per cent., had a distinct history of
syphilis, whilst in 101 cases of various other forms of primary
insanity only 18 per cent. had specific antecedents. H. Obersteiner
has 1000 cases of mental disease,[65] 175 cases of dementia
paralytica; of these, 21.6 per cent. had syphilis; moreover, of all
the syphilitic patients 51.4 per cent. had dementia paralytica.
[Footnote 64: _Progres. Paral. der Irren_, Berlin, 1880.]
[Footnote 65: _Monatshefte f. prakt. Dermat._, Dec., 1882.]
Various opinions might be cited as to the nature of this relation
between the two disorders, but for want of space the curious reader is
referred to the work just quoted and to the thesis of C. Chauvet[66]
for an epitome of the most important recorded opinions.
[Footnote 66: _Influence de la Syph. sur les Malad. du Syst. nerveux_,
Paris, 1880.]
Those who suffer from syphilis are exposed in much greater proportion
than are other persons to the ill effects of intemperance, sexual
excesses, poverty, mental agony, and other well-established causes of
general paralysis. It may be that in this is sufficient explanation of
the frequency of general paralysis in syphilitics, but I incline to
the belief that syphilis has some direct effect in producing the
disease. However this may be, I think we must recognize as established
the opinion of Voisin,[67] that there is a syphilitic periencephalitis
which presents symptoms closely resembling those of general paralysis.
Such cases are examples of the pseudo-paralysie générale of
Fournier.[68]
[Footnote 67: _Paralysie générale des Alienés_, 1879.]
[Footnote 68: _La Syphilis du Cerveau_, Paris, 1879.]
The question as to the diagnosis of these cases from the true
incurable paresis is of course very important, and has been considered
at great length by Voisin,[69] Fournier,[70] and Mickle.[71]
[Footnote 69: _Loc. cit._]
[Footnote 70: _Loc. cit._]
[Footnote 71: _Brit. and For. Med.-Chir. Review_, 1877.]
The points which have been relied upon as diagnostic of syphilitic
pseudo-general paralysis are—
The occurrence of headache, worse at night and present amongst the
prodromes; an early persistent insomnia or somnolence; early
epileptiform attacks; the exaltation being less marked, less
persistent, and perhaps less associated with general maniacal
restlessness and excitement; the articulation being paralytic rather
than paretic; the absence of tremulousness, especially of the upper
lip (Fournier); the effect of antispecific remedies.
When the conditions in any case correspond with the characters just
paragraphed, or when any of the distinguishing characteristics of
brain syphilis, as previously given, are present, the probability is
that the disorder is specific and remediable. But the absence of these
marks of specific disease is not proof that the patient is not
suffering from syphilis. Headache may be absent in cerebral syphilis,
as also may insomnia and somnolence. Epileptiform attacks are not
always present in the pseudo-paralysis, and may be present in the
genuine affection; a review of the cases previously tabulated shows
that in several of them the megalomania was most pronounced; and a
case with very pronounced delirium of grandeur, in which the autopsy
revealed unquestionably specific brain lesions, may be found in
Chauvet's _Thesis_, p. 31.
{1022} I have myself seen symptoms of general paralysis occurring in
persons with a specific history in which of these so-called diagnostic
differences the therapeutic test was the only one that revealed the
true nature of the disorder. In these cases a primary, immediate
diagnosis was simply impossible.
Case 14 of the table is exceedingly interesting, because it seems to
represent as successively occurring in one individual both pseudo and
true general paralysis. The symptoms of general paralysis in a
syphilitic subject disappeared under the use of mercury, to return
some months afterward with increased violence and with a new obstinacy
that resisted with complete success antisyphilitic treatment. Such a
case is some evidence that syphilis has the power to produce true
general paralysis.
In conclusion, I may state that it must be considered as at present
proven that syphilis may produce a disorder whose symptoms and lesions
do not differ from those of general paralysis; that true general
paralysis is very frequent in the syphilitic; that the only constant
difference between the two diseases is as to curability; that the
curable sclerosis may change into or be followed by the incurable form
of the disease. Whether under these circumstances it is philosophic to
consider the so-called pseudo-general paralysis and general paralysis
as essentially distinct affections, each physician can well judge for
himself.
Spinal Syphilis.
The subject of spinal syphilis is at present a difficult and
unsatisfactory one. The recorded cases with well-observed autopsies
are comparatively few, and when recovery occurs much uncertainty must
rest upon the nature of the lesion. More than this, there is scarcely
any chronic degeneration of the spinal cord which has not been
attributed to syphilis, and my own experience as well as the records
of medical literature lead to the very positive conclusion that all
the various spinal scleroses are much more frequent in infected than
in non-infected persons. Whether this is due to a direct or indirect
influence of the disease is uncertain, but I shall not here discuss
the relation of these chronic inflammations of the cord to syphilis.
It seems necessary to briefly consider at this place acute and
subacute myelitis in their relations to syphilis. That these
affections are not rare in syphilitics is certain. In the _Revue de
Médecine_ (Jan., 1884) Dejerine records the case of a person suffering
from chronic syphilis in whom there were fulgurant pains with
increasing weakness of the legs, and subsequently, after very severe
exposure to the weather, a sudden development of complete paraplegia
followed by trophic troubles, and death in twenty-eight days. At the
autopsy there was found a central myelitis with pronounced lesion of
the ganglionic cells, inflammatory changes of the pia mater,
capillaries, and neuroglia, extreme alteration of the nerve-roots, and
secondary degeneration of the columns of Goll and the lateral columns.
In a second case recorded by Dejerine there appears to have been no
exposure or apparent immediate exciting cause. The symptoms and
lesions were similar to those just spoken of, but death occurred in
eight days.
Whether such attacks as these occurring in syphilitic subjects are
produced directly by the syphilis or not is at present doubtful. The
same is {1023} true of subacute myelitis, of which I have reported two
rather peculiar fatal cases in syphilitic subjects. The general
symptoms of this affection are progressive loss of power with grossly
exaggerated reflexes, severe twitchings and jerkings of the legs,
rigidity, usually more or less marked pain, and other sensory
disturbances in the legs, and finally partial anæsthesia and complete
paraplegia, paralysis of bladder, bed-sores, and death from
exhaustion. At the autopsy the most important change in the cord has
been the presence of great numbers of round neuroglia-cells in both
gray and white matter. One of my cases died of a rapidly developed
central myelitis supervening upon the subacute disease, and affording
lesions similar to those described by Dejerine in addition to the
changes of the subacute affection.
In another class of spinal cases occurring in syphilitics the symptoms
resemble those of the so-called acute ascending paralysis (Landry's
paralysis). The fourth variety of syphilitic diseases of the spinal
cord of Huebner[72] includes these cases. According to Huebner, they
are without anatomical lesions, but in the majority of the recorded
cases no proper microscopic study of the cord has been made. Huebner
states, however, that Kussmaul failed in one case after such study to
detect lesion. As some of these cases may really have been instances
of peripheral neuritis, it is essential that in the future the
peripheral nerves as well as the spinal cord be carefully studied. I
have seen one case which might be placed in this category. The first
symptom was some numbness in the legs, with a small deep sharp-cut
ulcer on the plantar surface of the great toe; directly after this
loss of motion and sensation in the legs and thighs, rapidly becoming
almost complete and spreading quickly to the trunk and arms, so that
in one week the patient was a flaccid, helpless mass, and the
breathing so interfered with that he was believed to be dying. After
almost losing the power of swallowing this patient began to get
better, and finally so regained power of his hands and feet that he
was able to partially dress himself and walk a distance of ten or
twelve feet, when he was suddenly seized with a pleural effusion and
died. During the first week of his disease his temperature was 100° F.
At the autopsy the spinal membranes were found to be normal. But in
the cord there were very distinct lesions found; the neuroglia seemed
everywhere more granular than normal; the ganglionic cells were not
distinctly diseased; the white matter in various places was much
changed, the tissue appearing abnormally dense and opaque where most
affected; the nerve-tubules appeared to gradually lose their myeline,
and in places were reduced to simple axis-cylinders. Finally, the
axis-cylinders became smaller and smaller until in the most altered
portions of the cord they disappeared. As the autopsy was obtained
with great difficulty, it was not possible to get the peripheral
nerves for study.
[Footnote 72: _Ziemssen's Encyclopædia_, vol. xii.]
In regard to these very acute cases, it seems to me uncertain whether
the disease should be attributed to the syphilis. In my own case
twenty years had elapsed since the chancre, alcohol was habitually
used in great excess, and the attack was apparently precipitated by
great exposure. On the other hand, the man bore well enormous doses of
iodide of potassium, and lowly progressed under them.