Part 29

=Case 110.= Mr. McKenzie[18] was a retired merchant of 42 years whose complaint was that he tired very easily, could not make his legs go where he wished, was unsteady and felt a numbness in his legs. These symptoms had been in progress for a few months only when the examination was made. This disclosed Argyll-Robertson pupils, absent knee-jerks and ankle-jerks, Romberg sign, unsteady gait, moderate ataxia and dysmetria. The W. R. was negative in the blood serum but positive in the spinal fluid with 0.2 cc., and there were 107 cells per cmm. With the symptoms and signs it was therefore easy to make the diagnosis of TABETIC NEUROSYPHILIS (“tabes dorsalis”).

The patient was given five intraspinous injections of mercuric chloride in blood serum (mercurialized serum) according to the method of Byrnes. The dose was 0.001 gm. of mercury. Two weeks after the first injection the cell count was 58 cells per cmm., the Wassermann was positive only with 0.4 cc. After the fourth injection there were but 18 cells and the Wassermann reaction was negative even with 1½ cc. of spinal fluids. The symptoms had improved to such a degree that the patient had no complaint whatsoever and considered himself cured.

1. What are the unpleasant results of intraspinous therapy? Frequently there is an exacerbation of symptoms and pain may be quite severe after intraspinous injections. This, however, lasts only a short period, that is, as a rule less than 24 hours. There may be other symptoms of cord irritation as retention of urine or lack of sphincter control. A rise of temperature is not unusual.

=Treatment may alter the W. R. to negative in blood and spinal fluid in TABES DORSALIS.=

=Case 111.= Ivan Rokicki was a baker, 43 years of age, who came complaining of exceedingly severe attacks of abdominal pain with vomiting. He described these attacks as having occurred periodically for a number of years, lasting sometimes as long as a week, during which time Rokicki could not eat or get relief short of large doses of morphine.

Upon his arrival, Rokicki was seen in one of his attacks; he was curled up with excruciating pain, and the abdomen was rigid, though it was impossible to produce additional pain by external pressure. There was spasmodic vomiting, frequently followed by slight relief from the pain, which however shortly recurred and caused the patient to cry out in his suffering. The condition was controlled by opiates but lasted a full week. The leucocytes remained normal and there was no rise of temperature. The attack ceased spontaneously.

Save for the pain, Rokicki’s =mental examination= proved entirely negative. =Physically=, Rokicki was fairly well developed and nourished. His pupils were slightly irregular: the left markedly larger than the right; both pupils failed to react to light, and the left pupil also failed to react in accommodation. There were no other reflex disorders evident to systematic examination, nor was there sensory disturbance or speech defect. The heart seemed somewhat enlarged but there were no murmurs; blood pressure: systolic 150; diastolic 110.

The correct symptomatic diagnosis in Rokicki’s case proved to be gastric crises, and this diagnosis must perforce be the first to entertain in view of the chronicity, the periodicity, the non-relation to diet, and the spontaneous cessation of the seizures. The observation of Argyll-Robertson pupils was naturally held to substantiate the diagnosis of TABES DORSALIS.

The possibility of abdominal inflammation could be shortly dismissed on account of the absence of tenderness (the rigidity in this case was not accompanied by tenderness), fever, and other characteristic signs. There was no diarrhoea, such as is found in lead colic, and there was no other sign of plumbism. Jaundice was absent and there was no special radiation of pain from the abdomen. One had to think of gastric ulcer and hyperchlorhydria, and possibly malaria or gastroenteritis.

The pupillary reactions pointed to a syphilitic condition despite the fact that the lack of reaction to accommodation (over and above the Argyll-Robertson phenomenon) in the right pupil is not entirely typical. Accordingly, although there was no areflexia, Romberg sign, or ataxia, resort was had to the W. R. This however proved negative, in blood and spinal fluid; nor was there any globulin or excess albumin; there were 5 cells to the cmm., in the spinal fluid.

We are left, accordingly, with characteristic gastric crises; Argyll-Robertson pupils, slightly irregular; and a somewhat enlarged heart.

Upon investigation, it appeared, however, that a year before the attack above described, the patient had been examined and both blood and spinal fluid found positive to the W. R. At that time, treatment, consisting of intravenous injections of salvarsan and intraspinous injections of salvarsanized serum (Swift-Ellis), had been instituted. Whereupon the laboratory tests had become negative, as above stated, and there had been no alleviation of the symptoms.

1. How can Rokicki’s normal deep leg reflexes be explained? The abolition of the deep reflexes is of course due to lesions properly localized. It is probable that this particular case of tabes dorsalis is more truly “dorsal” than most cases; for most cases exhibit lesions involving regions lower than the dorsal. Both in these dorsal cases and in certain rare cases of cervical tabes, the deep leg reflexes are preserved. (See cases Green (30) and Halleck (31).)

2. What is the mechanism by which a characteristic gastric crisis is produced? The mechanism is unknown. Some endeavors have been made to meet gastric crises by surgery of the posterior roots, on the assumption that the irritation causing the pain was located either in the posterior ganglion or in the passage of the nerve through the meninges. In only a few instances, however, has the result been what was desired. In many instances the gastric crises and pain continued uninterrupted and in addition came discomfort due to the lack of sensation in the part supplied by the severed nerve. At present this treatment is seldom carried out.

3. Should antisyphilitic treatment be continued in such a case? As far as our present knowledge of syphilis goes one would hesitate to suggest further antisyphilitic treatment, feeling that the active process had been entirely stopped as suggested by the absence of any positive findings either in the blood serum or in the spinal fluid. We should perhaps conclude that there was no more activity in this case and that the crises were due to the changes that had already taken place in the nerve tissue and which could no longer be changed.

=The literature is in doubt concerning (in fact is preponderantly against) the success of treatment in PARETIC NEUROSYPHILIS (“general paresis”). Our experience has yielded a number of apparently successful results through systematic intensive intravenous salvarsan therapy. Example.=

=Case 112.= Albert Forest had always been a successful salesman, but in the middle of March, in his 46th year, he was arrested for grabbing a purse from a woman in front of a theatre and running down the street with it. In court, Forest acted strangely and he was sent to the Psychopathic Hospital for observation. Upon investigation, it appeared that his wife thought he had been showing mental changes for about a year. For example, he would embrace his wife on a street car, or refuse to pay her fare. He once attempted to hit his son on the head with a red-hot poker. Now and then he would become sleepy and stupid. He looked rather older than his age and had a coarse tremor of the hands. Otherwise, no change could be detected in the physical examination, either neurologically or otherwise. As for the manual tremor, Forest’s wife gave a history of considerable alcoholic indulgence on his part.

For several days, nothing abnormal could be detected in the man; and in particular, his memory for both remote and recent events was very good and his knowledge of current events was good. Simple arithmetic was easy to him.

One evening his temperature was found to be 104° F. and no cause could be discerned for this. The next morning, Forest was discovered in a stupor, with a complete right hemiplegia. The Babinski reflex, the Oppenheim reflex, and ankle clonus had appeared on the right side, and the right arm was spastic.

However, all symptoms of this paralysis had disappeared by four o’clock in the afternoon, and the paralytic phenomena were replaced with violence. The patient fought with the attendants and for some time remained extremely difficult to manage, being confused and subject to outbreaks of violence with destruction of furniture and other property about the ward.

=Diagnosis.= At first we were naturally inclined to dismiss the case with a diagnosis of alcoholism. The transient hemiplegia at once raised a considerable question of brain syphilis or of brain tumor.

The W. R. of the serum was doubtful. The spinal fluid yielded, besides marked excess of albumin and much globulin, also a “paretic” gold sol reaction and 75 cells per cmm. The W. R. was positive.

=Treatment.= The patient was given injections of salvarsan, 0.6 gram, twice a week, with potassium iodid. After a few weeks improvement followed, and after several months all the laboratory tests became negative, the patient was apparently perfectly normal mentally and was discharged from the hospital, and has remained well for 18 months without further treatment. The serum W. R. has continued to be negative.

1. What is the significance of the so-called “doubtful” W. R.? Where there is not a complete uniformity the results of the strong and weak antigens (see appendix on technique of Wassermann reaction) the result is reported as doubtful. In the majority of instances repetitions will give a strong positive reaction.

2. Is the case of Forest to be regarded as one of general paresis? Sometimes such cases are termed in the literature _syphilitic pseudoparesis_ (see case Burkhardt (58)). The differential diagnosis of this group is entirely therapeutic. There are, unhappily, no laboratory tests which will suffice in the present stage of knowledge to differentiate a case of so-called pseudoparesis from general paresis. We are inclined to term the case one of GENERAL PARESIS, with recovery, or, at all events, with remission.

=The literature is in doubt concerning (in fact is preponderantly against) the success of treatment in PARETIC NEUROSYPHILIS (“general paresis”). Our experience has yielded a number of apparently successful results through systematic intensive intravenous salvarsan therapy. Example.=

=Case 113.= We present the case of Gussie Silverman, a housewife, 35 years of age, among other reasons, for its social interest. The case is, on the whole, sufficiently typical of GENERAL PARESIS. =Physically=, for example, the pupils failed to react to light and accommodation and were unequal, the right being larger than the left. The knee-jerks were sluggish though equal. The ankle-jerks could not be obtained. The abdominal reflexes were not obtained. Otherwise, there was no reflex disorder.

From the =laboratory= point of view, the W. R. was positive in the blood and in the spinal fluid. There were 80 cells per cmm. and there were an appropriate globulin and albumin reactions. Mrs. Silverman was rather poorly nourished and had a slight edema of the ankles.

=Mentally=, she was found on admission to be markedly depressed. It appeared that during a recent pregnancy, terminated by the birth of a 7–months child, she had fainted several times a day, that since the confinement she had been very nervous, that she had been asking her husband not to send her away, that she had refused to leave the house, that she had become excited even to the point of injuring herself, especially at night, and that she would go so far as to scratch her husband, shortly afterward being very sorry for her performances. Before this last pregnancy there had been four others and the resulting children were all apparently in good health. Except for the fainting spells during the pregnancy, it would not appear that the story just told is at all characteristic of paresis.

However, in the hospital Mrs. Silverman could hardly be got to answer questions, continually saying, “You know what it is; I don’t have to tell you.” She claimed so marked a degree of confusion as not to know where she was and what she was doing. She would beg despondently that something be done for her, and iterate and re-iterate these claims. There appeared to be a marked degree of amnesia. Some one, she felt, had controlled her thoughts and made her do things she did not want to do and say things she did not want to say, things she did not know she was about to say. She said, “I feel like jumping around. I couldn’t believe myself as if I am me. Some one is making me jump around. I used to hear him talking. I don’t know who it is. I used to keep my eyes open and I couldn’t move. I feel only I would like to talk, and talk, and talk, and talk all the time. It seems to me that some one talks in me. I couldn’t sleep for five minutes. My God, I wish I could sleep! I used to feel something in my heart. I used to faint. It seems to me I used to see a funny thing. What it was I can’t tell. It used to talk to me, make me get out of bed, throw me about, make me do things. O, I don’t know what it was.”

These not entirely characteristic mental symptoms, together with the suggestive physical signs and the laboratory examination, caused treatment to be instituted; under which treatment (intravenous injections of salvarsan) she improved rapidly. Mental symptoms disappeared under the administration of 12 injections of salvarsan within two months. Moreover, the spinal fluid became entirely negative. Two and a half years have now elapsed since her discharge and she has shown no return of symptoms. The serum W. R. has always remained negative although there has been no treatment since leaving the hospital. There has, however, been no change in the reflexes, which remain as on admission. The 7–months baby has continued to be perfectly healthy. Its W. R. is negative, as are the W. R.’s of the husband and the other three children. It must seem surprising that a healthy child could have been born from a mother with generalized syphilis as in this case. However, perhaps there are more instances than we imagine like the case of baby Silverman.

1. May a patient be considered permanently cured although there has been no recurrence of symptoms for 2½ years and although the Wassermann has remained negative? One would hesitate to give a definite statement that the patient was cured until more time had elapsed. It is quite possible that spirochetes may be lurking in some portion of the body without causing the production of symptoms or Wassermann bodies and yet ready to break out at any time. This hypothesis has added weight from the recent work of Warthin already quoted. We advise examination of this patient at intervals of not longer than six months for a good many years.

2. Should the course under treatment cause us to change the diagnosis? It has often been stated that a differential point between cerebrospinal syphilis and general paresis is the reaction to treatment, that is, that a case which recovers could not be general paresis. Head and Fearnsides state that if six months after beginning of treatment the spinal fluid has become negative, the case should be considered as one of cerebrospinal syphilis and not general paresis. We do not feel ready to concur in this view as we know of no similar logic in medicine. We have many cases in which a spinal fluid has remained positive for six months and later become negative, so that where the symptoms shown are those of paretic neurosyphilis, we are inclined to consider the case such until such time as more definite evidence checked by post mortem examination causes us to change this point of view.

3. Do the reflexes change under treatment? The signs of spasticity often do disappear under treatment and also when there is no treatment. A few instances have been reported in the literature where Argyll-Robertson pupils are said to have altered to normal. It has never been our good fortune to see such a change nor have we seen an absent knee-jerk become normal, as has also been reported, except where it is the result of pyramidal tract disease superimposed upon the posterior column sclerosis causing a return of reflex. This, of course, is not to be considered as a return of the normal. (See Case 1.)

=Some RESULTS of systematic intravenous salvarsan therapy are PARTIAL (_e.g._, clinical recovery and persistence of positive laboratory tests).=

=Case 114.= Walter Henry was an undertaker in a small town. He was married and the father of two healthy children. In May, 1914, he began to lose his appetite. He felt restless and seemed to be losing his grip, and in August he repaired to a sanatorium, where he remained for two months. Shortly after leaving the sanatorium, he fainted one day, while digging a grave, during a spell of great heat. Since that time there had been numerous “weak spells,” with headaches and general debility, insomnia, and loss of weight.

In February, 1916, Mr. Henry came to the hospital for advice, but the trip from a distant part of the state was apparently such a strain for him that shortly after admission he collapsed. There were no convulsive movements in this collapse, but the patient was confused and his breathing was rapid and stertorous. The semi-stupor lasted for about 48 hours. Upon recovery from the stupor, Henry was found entirely disoriented, much confused, and laboring under the belief that he was digging a grave. After a time he again fell into a stupor and his temperature rose to 103° F.

The emaciation of this man was striking and unusual, but systematic =physical examination= showed no special disease. =Neurologically=, there were marked tremors, and there were purposeless movements of the arms. There was a marked speech defect. The pupils were dilated, regular, and equal, and reacted, though slightly, to light. Nothing abnormal was noted upon systematic examination of the reflexes.

The W. R. was strongly positive in the blood and in the spinal fluid; the gold sol reaction was typically “paretic”; there were 16 cells per cmm., globulin was present, and albumin was greatly increased.

The =diagnosis= GENERAL PARESIS was accordingly made, and treatment instituted. Intravenous injections of arsenobenzol, at first, and later of diarsenol, were given, as a rule twice a week (usual dose, 0.6 of a gram). Mercurial injections and potassium iodid were also given. This treatment was continued as the patient began to improve. The improvement was of such a degree that at the end of four months, Mr. Henry returned to his home and his work. He had had 30 intravenous injections of salvarsan substitutes. Despite the treatment and the clinical improvement, the laboratory tests remained essentially unchanged. The W. R.’s of the blood and spinal fluid remained strongly positive, as well as also the globulin and albumin; the gold sol reaction was still “paretic”; the cells stood at one per cmm. The patient has continued antisyphilitic treatment since leaving the hospital, and has remained apparently well, with good insight into his condition.

1. What is the significance of a temperature of 103° in a paretic without signs of infection and a normal leucocyte count? Temperatures of this type are not infrequent in the course of general paresis. They are usually spoken of as “paretic temperatures.” Their meaning is not understood, but they are often stated to be due to a disturbance of the heat-regulating mechanism. Such temperatures may remain elevated for a considerable period of time, but the elevation may be very transitory. At times they vary, like septic temperatures.

2. What can be argued from the fact that the cell count became normal? If thorough antisyphilitic treatment is vigorously given, it will be found that in the vast majority of cases of neurosyphilis the cell count will return to normal. It matters not whether the treatment be intravenous or subdural. It is very difficult, however, to obtain this result in general paresis by the use of mercury alone. It cannot, however, be urged that this finding has any great prognostic significance as it occurs in the cases which do poorly as well as in those which recover symptomatically.

3. Is it safe to give large doses of salvarsan to a patient in a stupor? It is not a good plan to give a large dose to such a patient on account of the danger of sudden death. This is probably due as much to the strain put on the heart as it is to any effect on the nervous system, or specific arsenic effect. In this particular instance, a dose of 0.15 gm. was the initial injection and this was increased five centigrams per injection.

=IMPROVEMENT IN PARETIC NEUROSYPHILIS (“general paresis”) may become evident only after several months of intensive treatment.=

=Case 115.= Henry Ryan was a shipping clerk, 54 years of age, who was brought to the hospital following a convulsion. For a few months preceding this period, Mr. Ryan had been failing in his abilities. He had been very forgetful, showed no energy, and had become very irritable. He also complained of insomnia and of feeling nervous.

On admission to the hospital, the most striking feature in the mental situation was that he claimed that he had not slept a wink for three months, and each day he would solemnly affirm that he had not slept at all the preceding night, although the records might show that he had slept eight hours. Argument was of no avail against this conviction. In addition, his memory was very poor; he showed little knowledge of current events, and had no ability with arithmetical problems.

=Neurologically= viewed, the points of chief significance were contracted immobile pupils and a speech defect, especially noticeable on the repetition of test phrases. The whole picture was suggestive of general paresis, and this diagnosis was confirmed by the laboratory findings. It was found that the W. R. was positive in the blood and spinal fluid, that there was a pleocytosis, positive globulin reaction, excess of albumin, and a “paretic” gold sol reaction. Consequently, the diagnosis of GENERAL PARESIS seemed justified, although the patient denied any knowledge of a syphilitic infection.