Part 36

Special attention should be drawn to a certain NEUROSYPHILITIC FAMILY[122] in which both parents and five children showed a variety of syphilitic diseases, including syphilis without apparent neural complications, paretic neurosyphilis, juvenile paresis, aortic aneurysm, achondroplasia and caries of the spine, and an as yet indefinite neurosis. There was a sixth child that died shortly after birth, as well as three stillborn.

One =cannot conclude= from the normal[123] look of a neurosyphilitic’s family =that the normal-looking members are not syphilitic=, as illustrated by the family of our draughtsman.

The most =intricate social complications= may arise. We present a case of a syphilitic man (a well-to-do merchant) who was apparently being goaded into a second marriage[124] because he was continually being charged with having caused his first wife’s death. This he had actually done in a certain sense because his wife had died of general paresis, having contracted syphilis from him.

In the fifth section on THERAPY, we have attempted to outline some of the principles and problems that arise in the treatment of neurosyphilis. Enough has probably been said concerning the attitude of optimism or pessimistic nihilism that may be adopted toward the whole subject. It must be borne in mind, however, that a great deal of the work on treatment of neurosyphilis is still in the experimental stage. As a rule, each case must be considered separately and individually and the prognosis can be made satisfactorily only after treatment has been given. This section contains a group of cases that have been treated rather intensively and the results of this treatment are indicated. The section is introduced by _five untreated cases_, the brains and cords of which have been studied post mortem. These illustrate the pathological conditions which we have to meet, and from these examples we can draw the theoretical conclusion that some cases are beyond the aid of therapy on account of the brain destruction. Others, in which the symptomatology bespeaks just as grave a situation, turn out on autopsy to have very little actual damage to the brain tissues and therefore should theoretically at any rate be amenable to antisyphilitic therapy.

In order to get any adequate conception of the possibilities of therapeutic results in cases of neurosyphilis, one must consider the pathological changes that occur and how far these changes are reparable. In cases in which the destruction of tissue is marked, it is, of course, out of the question to expect to get any marked clinical improvement. A case of spastic hemiplegia[125] in paretic neurosyphilis is given with the autopsy findings as an illustration of irreparable damage that may occur to the parenchymatous structure, thus precluding any chance of functional recovery.

On the other hand, there is a group of cases in which the symptoms may be exceedingly severe and yet the actual destruction of tissue be almost nil. This point is illustrated by a case[126] in which _total duration of symptoms_ terminating in death was _only 22 days_. At autopsy there was very little in the way of macroscopical lesions, and microscopically there was no marked evidence of destruction in the parenchymatous tissue. The lesions were represented chiefly by perivascular infiltration. According to all our modern ideas, this type of reaction is resolvable under antisyphilitic treatment. Though this case was one of very short duration, similar pathological pictures may be obtained in cases of considerably longer standing. It is also of great importance to remember that symptomatically such a case may be in no way distinguished from a case with marked atrophy.

Another autopsied case is given which shows an exceedingly =marked meningitis=.[127] The meningitic processes according to the literature and experience react very readily to antisyphilitic treatment in the form either of mercury and iodid or in combination with salvarsan. The lesion here present would probably have improved had intensive treatment been given. Clinically the diagnosis of general paresis was made and, as has been the rule in the past, treatment was not given on the ground that it had no value in paresis. While this is an extreme case of meningitis, it is to be remembered that the vast majority of cases of paretic neurosyphilis show some degree of meningitis. Just as in the marked meningitis of the diffuse neurosyphilis, so with the meningitis of the paretic form, improvement is expected under treatment. As a part or even the whole of the symptomatology in a given case may be due to this meningitic process, we have reason occasionally to expect marked improvement as the result of antisyphilitic treatment.

As a contrast to this case with marked meningitis, another case of =marked atrophy=[128] is given. Here the atrophy was very perceptible on macroscopical examination and the mere view of the brain at once indicated that in such a case important results from treatment were not to be expected.

The =topographical variation= of the lesions in neurosyphilis must be remembered when treatment is to be instituted. Thus very marked lesions may exist in portions of the brain which do not give any very definite localizing symptoms. As a result, one may be led to believe from clinical evidence that the case is a very mild one though the lesions may really be very extensive. The topographical distribution must, therefore, be taken into consideration in trying to estimate the damage done. This point of topographical distribution of the lesions is illustrated by a case.[129]

It has been generally recognized that =clinical improvement=, if not cure, may be =readily obtained in the group of diffuse neurosyphilis=, i.e., so-called cerebral and cerebrospinal forms of syphilis. These are cases in which the parenchyma is very slightly, if at all, affected and in which the lesion is chiefly in the meninges and blood vessels, irritative rather than degenerative. A case[130] is given to illustrate this point. In our experience systematic intravenous salvarsan therapy associated with mercury and iodid gives remarkably good results in the vast majority of this group of cases.

It is generally conceded that antisyphilitic treatment, particularly salvarsan, has a very satisfactory result applied to diffuse neurosyphilis. But the same good results may be obtained in cases which are not so typically of the diffuse type. An illustration is given in the case of a machinist in which the diagnosis was in doubt between paretic, tabetic or diffuse neurosyphilis.[131] The result of treatment was as satisfactory as could be expected in any type of neurosyphilis and this in a case of several years’ duration with Argyll-Robertson pupils.

As a rule, the Argyll-Robertson pupil is taken as a grave omen for treatment, an idea based upon a conception that the Argyll-Robertson pupil so frequently represents the old so-called “parasyphilitic” cases, which, in the past were taught as being incapable of improvement by the ordinary antisyphilitic methods.

A second case[132] with Argyll-Robertson pupil shows again that the =prognosis may be very good despite the Argyll-Robertson sign=.

But even in the diffuse neurosyphilis, the symptomatic results of treatment may not be entirely happy. Under treatment it may be possible to reduce the spinal fluid tests to negative without, however, as in the case of our hemiplegic lady,[133] making the physical or mental symptoms disappear. In other words, it may be possible to stop the active progress of the disease without removing the symptoms.

One is always warned of the danger of intravenous salvarsan therapy in hemiplegic cases due to arteriosclerotic conditions. While this warning is well justified, it does not mean that the most intensive treatment is contraindicated, as shown in the case of our hemiplegic machinist.[134] Such may be given over long periods of time with the most satisfactory results.

A case[135] is given which illustrates the value of antisyphilitic treatment in cases showing symptoms of intracranial pressure due to syphilitic disease. In the case of the woman which we cite, we believe that the symptoms of intracranial pressure were probably due to a gummatous new growth, although it is possible that they were due to a marked meningitic process. However, the results of a limited amount of antisyphilitic treatment in this case were very brilliant. Similar results may often be obtained in gumma of the brain. This is not always true, however, and it may become necessary to use surgical procedure in order rapidly to overcome the effects of intracranial pressure.

While it has always been conceded that treatment would greatly help cases of diffuse and vascular neurosyphilis, the utmost pessimism has existed concerning the results to be obtained by treatment in cases of tabetic and paretic neurosyphilis. Only in the last five or six years, due to the stimulus of Ehrlich’s discovery of salvarsan and the introduction of the intraspinous methods of therapy, have intensive work and study been given to the treatment of these cases. And though it has been by no means settled in the minds of the various workers in this field, as to what the ultimate results of such treatment will be and though some do not believe that there is any good to be expected from our present methods, still the majority of men who are treating these cases systematically feel very much encouraged.

=At times very brilliant results= are to be obtained by intraspinous treatment =in tabetic neurosyphilis= (“tabes dorsalis”). A very striking illustration is given of a case of this sort in which the symptoms dated only a few months but which had all the classical symptoms, signs and laboratory tests. Five intraspinous injections of mercurialized serum were sufficient to cause the disappearance of the subjective symptoms and to reduce the spinal fluid test to negative.[136]

It must be emphasized that the best results in cases of tabetic neurosyphilis are usually to be expected in cases in which the symptoms are of short standing. Where the process is of long duration and much destruction of spinal cord tissue has occurred, the best one can expect is that the activity and progress may be halted. This is illustrated by our case of a baker, 43 years of age, who had been suffering from the symptoms of tabes for some years. Under treatment it was possible to get an entirely negative serology of the blood and spinal fluid.[137] Despite this evidence that the activity of syphilis had ceased, the symptoms continued unabated. We are ready to believe, however, that much good was accomplished. For the patient should not have any further untoward developments or the appearance of any new symptoms. These, without such treatment, might well be expected. At times excellent clinical results are obtained in long-standing cases.

The results of treatment in paretic neurosyphilis (“general paresis”) have been considered even less hopeful than in tabetic neurosyphilis (“tabes dorsalis”); indeed, it has often been stated that the patients are made worse by treatment. Recent work, however, supports a much more optimistic viewpoint. We feel that =intensive treatment has been of the greatest value in a number of cases of paretic neurosyphilis=. Two cases are given which show the most satisfactory and brilliant results of intensive intravenous salvarsan therapy in cases diagnosed as general paresis. The first case, an excellent salesman, 46 years of age, with most aggravated mental symptoms, recovered symptomatically and all his tests were rendered negative.[138] He has now remained entirely well and economically efficient for about two years without further treatment. The other case,[139] a housewife, also with very marked symptoms suggestive in all ways of general paresis, also recovered rapidly under treatment and her tests became negative. Her remission has now lasted for nearly three years without further treatment.

At times it is not possible to get the spinal fluid tests to become negative in cases of paretic neurosyphilis under the most intensive salvarsan therapy. In spite of this, the clinical condition of the patient may improve so greatly that the patient can be considered =clinically recovered=. An illustration is given of an undertaker[140] who was brought from a condition of the greatest cachexia and mental confusion to a condition of robust appearance and mental efficiency under intravenous salvarsan therapy, in spite of the fact that his tests were very slightly if at all reduced in intensity. He has been able to resume his former occupation and his former life with great satisfaction to himself and his family.

Improvement in paretic neurosyphilis under treatment is not to be expected very early. =Two or three months of active treatment= may elapse before one sees signs of improvement. Indeed, as illustrated by our case of the shipping clerk, this improvement may begin to make its appearance only after more than four months of intensive treatment consisting of two injections of salvarsan per week.[141] In spite of the long delay in this case, complete clinical recovery occurred and the tests became almost negative at the end of a year of treatment.

It is not only in the central nervous system that the syphilitic process may resist the most intensive treatment. In the case of the speculator, a victim of paretic neurosyphilis, which we cite, a perennially recurrent iritis appeared after several months of the most intensive salvarsan treatment which was apparently sufficient to reduce the symptoms of the paretic neurosyphilis,[142] but not of non-neural syphilis.

We give the case of a charwoman having the diagnosis of paretic neurosyphilis, who, under intensive treatment, made a symptomatic recovery. The interesting point in her findings is that all the tests in the spinal fluid became negative except the gold sol reaction which remained of the “paretic” type.[143] There is no general rule as to the reaction of the spinal fluid tests under treatment. At times one test is the first to disappear under treatment; again it is another. We have seen many cases in which the gold sol was the first test to become negative and others, as the case given, in which it is the last to show any change. As in our undertaker, symptomatic clinical improvement may be practically complete without any change in the spinal fluid tests.

One must remember that it is the condition of the patient that is of first importance; not so much the laboratory tests. Having shown the clinical recoveries with the tests remaining positive, we now have to report two cases in which there was =improvement= as shown =by the tests but no clinical improvement=. The first patient, a bank teller[144] of 39 years, with a diagnosis of paretic neurosyphilis, received intensive intravenous salvarsan for several months. Under this treatment all the tests became negative except the gold sol which remained of the paretic type. In spite of this, there was not the slightest improvement in his mental condition.

The second case, a young man of 29 years in whom the symptoms of neurosyphilis had recently appeared, under treatment showed a marked diminution in the intensity of the spinal fluid tests, notwithstanding which the patient became more and more demented and died after a series of convulsions.[145]

Of course, good results indicated above in some of our cases of paretic neurosyphilis are not to be expected in every case no matter how intensive the treatment. We give a case of paretic neurosyphilis in which the most intensive intravenous salvarsan therapy gave no satisfactory results. This was followed by several intraventricular injections of salvarsanized serum. The results of this combined treatment, however, were still not satisfactory, and the patient died.[146]

In order to emphasize as strongly as possible what we believe is a great =advantage of systematic intensive treatment= for neurosyphilis, we offer two cases in different time periods of neurosyphilis. The first is a printer with the symptoms of diffuse neurosyphilis six months after the appearance of his chancre.[147] These symptoms appeared despite three injections of salvarsan, injections of mercury and mercury by mouth. Under intensive treatment (meaning injections of salvarsan twice a week and continued injections of mercury), complete recovery occurred in a few weeks.

The second case is that of a waiter with signs and symptoms of neurosyphilis in whom the diagnosis lay between the diffuse and paretic forms.[148] This patient developed his symptoms in spite of continuous antisyphilitic treatment during the six years since his infection. This treatment had been comparatively mild, consisting in great part of mercury by mouth. However, he had had courses of injections of mercury and several injections of salvarsan. Under a systematic course of intravenous injections of salvarsan twice a week for a number of months, all symptoms disappeared and the spinal fluid tests became negative as well as the W. R. in the blood serum.

A final case is offered which indicates that antisyphilitic treatment may occasionally be of service in improving the mentality of a FEEBLEMINDED CONGENITAL SYPHILITIC.[149]

No attempt has been made in this section to give a per cent evaluation of the results of treatment in any one group of neurosyphilis. Two charts (charts 25 and 26), however, are appended which give an indication of some of our results. It seems to us, however, that it is too early to make any definite statements as to how far treatment will take us in the groups of neurosyphilis. We do feel decidedly, however, that many patients, in whatever group of neurosyphilis the diagnosis may place them, will respond to intensive systematic antisyphilitic treatment. =It is unfair to give an entirely grave prognosis in any case of neurosyphilis until the effect of treatment has been tried.=

In a separate section, entitled NEUROSYPHILIS AND THE WAR, we have presented fourteen cases selected from British, French and German writers in the war literature of 1914–16. Most of these cases were naturally somewhat inadequately reported under the critical conditions of literature made in the war. We present the cases for what they are worth: at all events they draw attention to the extraordinary interest of the neurosyphilis problem in relation to the war.

Such cases as A, one of tabes dorsalis apparently developing paresis by a process akin to shell-shock, is of value in the interpretation of the development of paresis in civil life. By “shell-shock” we commonly refer to a condition in which there is no actual traumatic injury of the brain. The hypothesis must be then that the explosion in some way indirectly caused an alteration of living conditions of the spirochetes, permitting the development of paresis.

Case B similarly seems to be a case in which a latent syphilis has turned shell-shock into tabes dorsalis.

Cases C, D, E bring up the question of aggravation of neurosyphilis _by_ service and _on_ service, respectively.

Case F likewise shows how, in the determination of amount of pension, the probable duration of the neurosyphilitic process is important.

Case G seems to show that war stress alone, without the emotional or physical effects of shell-shock, may kindle a latent syphilis into paretic neurosyphilis.

Case H similarly suggests that the “gassing” process may effect the same result.

Case I seems to show that the neuropathically tainted person may have latent epilepsy brought out through syphilis, the syphilis in this case having been acquired during the first summer of the war.

Case J was an interesting case of a syphilitic who, after the stress of the Battle of Dixmude, became an epileptic.

Syphilitic root-sciatica was developed in Case K at work in the war zone.

Case L is one of a civilian who apparently would not have developed paresis at precisely the moment when he did, if he had not been discharged as a German Jew from his long-held bank position in London.

Two cases, M and N, are cases of shell-shock, non-syphilitic; yet the picture of paresis in the one case and of tabes in the other was for a long time almost convincing to the examiners. They are better termed cases of pseudoparesis and pseudotabes, using the prefix “pseudo”, as usual, to signify a non-syphilitic imitation of the disease in question.

To sum up in the most general way the lessons of this book, we may emphasize again (1) _the unity-in-variety of the phenomena of neurosyphilis_, (2) _the value of a hopeful approach to the therapy of all cases of neurosyphilis_, _even the paretic form_, and (3) _the value of applying syphilis tests to every case of neurosis or psychosis_.

(1) RE _unity-in-variety of neurosyphilitic phenomena_.

The unity of these phenomena is confirmed, theoretically, by the common factor of spirochetosis: practically, by the Wassermann reaction, positive in serum or spinal fluid! Almost at this point the unity of phenomena ceases. Neither chronicity, nor evidence of mononuclear cell deposits, nor evidence of serious structural damage to the nervous system, nor presence of other positive tests than the W. R.,[150] nor existence of mental or nervous symptoms or signs, is a common feature of neurosyphilis. Sometimes the nervous system appears to harbor spirochetes in the most cordial manner as guest-friends (_paresis sine paresi_.) Again, perhaps as an expression of elaborate processes of immunity, the spirochetes take effect in relatively huge gummata. Sometimes the neurosyphilitic process rises as if by a regular process of siege from spinal nerve-root to spinal nerve-root (tabes dorsalis and diffuse neurosyphilis). Again, the nervous system is taken by storm, as it were (disseminated encephalitis). Very frequently the neurosyphilis is simply an indirect effect of blood-vessel disease, and huge masses of tissue are scooped out in necrosis with dependent secondary degenerations; and later the extinct lesions of vascular origin may or may not betray evidence of their syphilitic origin. Sometimes diffuse processes run on, apparently, with perfect fatalism to a mortal issue in a few years both with and without treatment. Again treatment appears to accomplish much (see fuller discussion under 2). The laws governing the preference of processes to lodge in membranes, vessels, and parenchyma, and in all combinations of these, have not been worked out. Hardly a case of neurosyphilis, properly studied ante mortem and post mortem, but would throw important light on our medical approach to one of the great problems of civilization, the problem of syphilis as a whole.

(2) RE _value of a hopeful approach to the therapy of neurosyphilis_.