Part 23

1. Can we separate the symptoms of Addison’s disease from those of paresis in this case? The extreme cardiac weakness with a characteristic, small low-pressure pulse is in point. The asthenia and apathy are consistent enough with Addison’s disease as well as with paresis itself. It would also be possible to ascribe the gastro-intestinal symptoms to either disease.

2. Of what significance is the persistent thymus? Persistent thymus has been observed in a few cases of Addison’s disease, but that it plays any part in the symptomatology thereof is a matter of doubt.

3. How can the obesity be explained? It is of course of note that the thyroid gland was small, but microscopically there were no peculiar features in this gland.

4. Was the adrenal tuberculosis actually primary? Minute search failed to reveal evidences of tuberculosis elsewhere unless we regard the few adhesions binding the lower half of the lung to the chest wall as indicative of an old tuberculosis. In particular, the mesenteric lymph nodes were normal.

=Neurosyphilis? Secondary stage of syphilis.=

=Case 81.= Florence Fitzgerald, a woman 25 years of age, applied at the police station to be taken care of. She said she had been a prostitute for the last few months, was now ill, and wanted to reform. She appeared physically ill and was sent to the Psychopathic Hospital, where she remained at first almost mute, making answers chiefly by nodding the head. She gave the impression of daze or stupor, and in fact her condition was at first regarded as catatonic. This reaction, after a few days, changed and Florence became quite normal, giving a full account of her condition.

It seems that four months before going to the police station, she developed a chancre, which was locally treated. A careful physical examination showed a fine red macular eruption which was without much question a syphilitic roseola. The spinal fluid yielded a positive W. R. although other tests of the fluid were negative. Curiously enough, no physical sign of involvement of the nervous system could be discovered. We were inclined to regard the mental symptoms as partly due to the syphilitic intoxication, and partly due to a psychic reaction of the nature of defense. As for the positive W. R. in the spinal fluid, in early secondaries various observers differ as to the frequency both of the W. R. and of other changes, percentages being given that range from 25 to 90%. See case Caperson (45). It is of note that clinically there were symptoms referable to a syphilitic involvement of the nervous system; namely, marked headache and malaise. The headaches of the secondary period are frequently the result of meningeal involvement.

=TABOPARETIC NEUROSYPHILIS (“taboparesis”); death from TYPHOID MENINGITIS. Autopsy.=

=Case 82.= Frederick Estabrook was a salesman, who, be it noted, had never had typhoid fever or any disease remotely resembling typhoid fever. He had acquired syphilis at 19; had married at 22; was the father of two healthy children (no miscarriages); had had a certain disturbance of bladder and rectum, but remained a successful salesman to the age of 28, when advancing tabes confined him to bed for a time. At 30, mental signs of PARETIC NEUROSYPHILIS developed, and death followed at 32, after an acute illness of a week.

The details of the history after the first symptoms at 28 are as follows:

At twenty-eight patient lost control of limbs and was confined to the house about two months, under medical care. Three months later he had regained partial control of his limbs but had lost all control of his sphincters. After another month he had returned to work, but did not work steadily and seemed to have lost ambition. In the summer of 1905, his mind became obviously altered. He grew indolent and extravagant and given to buying expensive and useless articles. Loss of interest in things followed, together with loss of memory for recent events, lack of insight into illness, delusions of persecution by wife, irascibility followed quickly by crying. Before admission to hospital, he was euphoric, drawling and tremulous in speech, sprawling in penmanship, alternately depressed and exalted in manner. Knee-jerks were absent, gait ataxic, pupils stiff to light.

The family history was negative with respect to insanity. All the family were reported as nervous. A brother died of peritonitis at twenty-eight, a sister of pneumonia under twenty. Another brother and sister are living. Father and mother died of heart trouble at about sixty-seven and sixty respectively.

The patient was at high school one year and was a fair student. Considerable tobacco was used, and some alcohol. Intoxication denied. There was no history of typhoid fever or other acute disease.

The patient on admission was sallow, poorly nourished, and flat-chested, with a slight lateral curvature. There was slight dulness over right apex in front and in right upper back. Voice sounds were increased over right apex in front and over whole right back. The right chest showed bronchial respiration throughout. The respiration in front of right chest was of an interrupted character. The liver seemed moderately enlarged. The urine showed a very faint trace of albumin. There were a few small nodes in right groin and a scar on dorsum of penis.

=Neurological Examination.= Slight swaying in Romberg position. Slight tremor of protruded tongue and extended fingers. Pupils irregular, left slightly larger than right. Left pupil reacted to light consensually, but not directly. Right pupil reacted very slightly to direct light, not consensually. Knee-jerks and Achilles jerks absent. Ankle clonus absent, abdominal and cremasteric reflexes brisk. Sharp and dull points were recognized in the legs with numerous mistakes. Vocal and facial tremor. Speech slow and drawling. Test phrases repeated well if care was taken. Consciousness clear. Orientation perfect. Calculating ability preserved. Many words omitted in writing. Penmanship clear but shaky.

Hallucinations absent. Memory of recent events poor. Associations of a logical or defining type. Patient denied various statements in commitment papers and had little or no insight into the mental side of his disease—slight euphoria.

After a month’s observation the patient was removed to a quiet ward and set to work a few days in the scullery. One night he began to yell as if assaulted and said later that he had an idea that he was going to die. Before three months had passed he had become untidy, disorderly, and imperfectly oriented.

The general degeneration continued rapidly. One week before death the temperature rose to 103 degrees F., and the patient succumbed to what seemed clinically like a bronchopneumonia. Unconsciousness two days before death.

Note with respect to history of typhoid.—Inquiries of his physicians, wife, employer, and brother tend to show conclusively that the patient never had a disease even remotely resembling typhoid fever.

The =autopsy= findings were as follows:

Acute conditions:

Hypostatic pneumonia, with early serofibrinous pleuritis and without lymph node swelling; =enlargement of mesenteric lymph nodes=; =acute cerebrospinal leptomeningitis=; multiple small hemorrhages of spleen.

Other findings:

=Scar of penis=; =sclerosis of aortic arch= (Heller’s type?) and slight coronary arteriosclerosis; =calvarium= thin and =dense=; =dura mater thickened= and adherent to calvarium; calcified arachnoidal villi; =chronic= cerebral and cerebellar =leptomeningitis=; =atrophy of frontal lobes=; =granular ependymitis=; =sclerosis of posterior columns= of spinal cord; emaciation; unequal pupils; slight parietal fibrous endocarditis, slight mitral sclerosis; gastro-intestinal atrophy; chronic cystitis; chronic abscess of prostate.

The description of the head findings is as follows:

Skin exceedingly loose, and the whole skull cap thinned. The diploë are absent. Adhesion with dura easily separated. The dura somewhat thickened, but not distended. Along the longitudinal sinus extensive calcareous granulations adhere to it. The longitudinal sinus does not contain blood, and the inner surface is normal in color. The pia is extensively thickened and opaque and a general subpial exudate exists which is more marked over the vertex where it lifts the pia from the brain surface to the extent of three centimeters in Rolandic, superior frontal, intraparietal, and mesial precentral sulci on each side. The arteries at base are free from atheroma. The temporal lobes are much bound down by adhesions, as is the cerebellum. Post mortem softening is evident. The hemispheres show no asymmetry, but the frontal convolutions are markedly atrophic. The corpus callosum is united to the cortex by old adhesions and has to be dissected away from it. Lateral ventricles contain some slight amount of cloudy fluid, and the pia along the vessels is opaque. Some granulations in ependyma. Brain weight, 1305 grams. Pons and cerebellum, 195 grams.

Cord.—Dura much thickened, and the pia corresponds to its appearance in brain with a like exudate. Cross sections of cord show sclerosis of posterior columns.

Bacteriologically the _typhoid bacillus_ was cultivated _from the meninges and from the swollen mesenteric lymph nodes_. The blood was negative; the intestines were negative so far as lesions were concerned.

The microscopic examination confirmed the clinical diagnosis of GENERAL PARESIS and of TABES, since there was not only an extensive chronic encephalitis, with the usual lymphocytic and plasma cell deposit and irregular gliosis, but also a well marked posterior column sclerosis, not unusual save in its extreme degree.

It might be surmised that some difficulty would arise in distinguishing the effects of paretic meningoencephalitis from those of the more recent typhoidal process. The well-known tendency of typhoidal processes to escape polynuclear exudation, at least until frank necrosis has set in, gave rise to the idea that the two mononuclear pictures—that of general paresis and that of typhoidal processes—might be confusing.

The picture presented by the meninges was scarcely what might be expected. Although numerous mononuclear phagocytic cells are everywhere found, yet the predominant picture is that of a polynuclear exudation.

The polynuclear leucocytes occur in greatest numbers in the tissue spaces, especially in the meshes of the lumbar arachnoid and in the spaces of the frontal and paracentral pia mater. In the lumbar region of the spinal arachnoid wide fields occur in which the cells are almost one hundred per cent polynuclear leucocytes. In places phagocytic cells occur, and in a few fields, even in the open tissue spaces, the number of phagocytic cells may arise to fifty per cent. Edema is a considerable feature in the meninges. Fibrin is found chiefly in the cerebral meninges and appears in numerous delicate strands in the tissue spaces.

Moloch, horrid king, besmeared with blood Of human sacrifice, and parents’ tears; Though, for the noise of drums and timbrels loud, Their children’s cries unheard that passed through fire To his grim idol.

Paradise Lost, Book I, lines 392–396

IV. MEDICOLEGAL AND SOCIAL

=Neurosyphilis in a public character: eloquence, reformatory efforts, notoriety.=

=Case 83.= Major Isaac Thompson, M.D., was a character. He had been regarded as eccentric for many years prior to his death at 63. In fact, it seems that there had been more or less definite symptoms and signs about his fortieth year. The doctor himself had a ready explanation for his Argyll-Robertson pupils; he explained that he had had a peculiarly heavy smallpox at about the age of 27 (which would be about 1872).

The doctor had a good secondary education, he had gone through the Civil War as a hospital steward, went into business after the war, married, and then went to the medical school, graduating at the age of 34. He continued in practice for a dozen years, and then gave it up. For years he had been especially interested in certain literary lines and he had published any number of pamphlets, all of a somewhat striking description, often with a political color and intended to stir up reform measures. The doctor never bore a very good reputation, and years later it was recalled that certain books disappeared from libraries and their loss was almost certainly traced to Dr. Thompson. In general, however, he was considered to be a rather worthy local figure.

It is possible that a fall on the ice in his 61st year actually started the fatal process, since after that time the patient had difficulty in walking, and a few months later developed periods of excitement with peremptory insistence on obedience to his wishes. Whereas formerly the doctor had finished up one literary piece of work after another, he now began to do very scattering work. He appeared in public to denounce certain financial schemes with great force and unusual eloquence. His eloquence was greatly complimented, and these compliments induced the doctor to a remarkable crusade against a certain corporation; there was so much truth mixed with the fiction of his eloquence that he obtained a considerable following in his campaign. He wanted to start a bureau of information for the instruction of the public on these matters, and he planned to put up a building adjoining his own home for the accommodation of the various clerks and writers in this bureau. However, before the building had been actually started, an outbreak occurred.

One morning the doctor was very excitable and noisy over the telephone, ordering typewriters and giving directions to mechanics. He repaired to Boston in connection with certain resources that he supposed (and gave others reason to believe) had been supplied by the Government and by a large newspaper. One evening he returned very late. It appeared that he had had a fracas at a hotel and had knocked down one or two colored porters, acting as though drunk. Upon being put to bed, the doctor talked incessantly of religious matters, proposing to undertake a Sunday School class. His interlocutor did not exhibit a particular interest in this scheme, whereupon Dr. Thompson threatened him with violence. Police and doctors were called in and a constant stream of conversation lasted for hours. The patient was finally brought to Danvers Hospital upon representation by physicians, to whom he told that his luck had turned, that he was about to be made senator from the district, and that he and Roosevelt were going to break up the trusts, and that, as a matter of fact, he was a relative of Mr. Roosevelt.

Upon admission, the patient was a well preserved and well groomed man with gray hair and beard. He was somewhat pallid but his teeth were well preserved and well cared for, and there was little or no physical change except a slight hypertension. He claimed that he had suffered from kidney disease for some years, and there was in fact a trace of albumin in the urine.

=Neurologically=, the plantar and Achilles reactions could not be obtained, but there were no other reflex disorders except the bilateral Argyll-Robertson pupil. The doctor’s explanation for these stiff pupils, which he described as existing for many years, was frank and circumstantial, so that the unlikelihood of Argyll-Robertson pupils due to smallpox was rather frowned upon by him. Without entering upon a detailed description of the clinical symptoms and course of the disease which led to death a little over a year after admission, it may be said that the differential diagnosis lay between the expansive form of general paresis and a maniacal condition, presumably the maniacal phase of manic-depressive psychosis. From the data of a special staff meeting held upon the case, we learn that the diagnosis of manic-depressive psychosis was entertained more strongly than that of general paresis. Thus, for general paresis alone was the somewhat gradual onset with increasing excitement, accompanied by expansive delusions concerning unlimited finance, personal over-importance, and Argyll-Robertson pupils. Dismissing the Argyll-Robertson pupils from consideration, the diagnosticians were led to see in the constant motor activity displayed in conveying an enormous number of thoughts on paper, inconsistent talking with digressions, a manic-depressive psychosis. There was no amnesia and no other sign of mental deterioration. There was a certain improvement early in the hospital stay of the patient. Consciousness was clear and orientation perfect. The delusions themselves, though extravagant, were not inconsistent or fantastic. The hallucinatory disorder was hardly characteristic either of manic-depressive psychosis or of paresis.

The patient might be described as “interesting.” A good preliminary training with years of travel and variety of occupation, furnished him with a fund of knowledge. An excellent memory, prompt replies and repartee, endless digressions with voluntary return to the original topic, caused him to be an amusing and even instructive interlocutor. However, his commitment and confinement in the institution seemed always entirely wrong, and he expressed mixed feelings about the family, now being bitter against them, and again condoning their mistakes. The patient’s conduct was good and he was tidy in habits, and tried as far as possible to conform to the requirements of the hospital. The doctor showed a marked antipathy toward a certain male attendant, who had removed articles from his clothing upon admission and had reclaimed a book on rules and regulations. The doctor prepared a list of 327 different acts of abuse, lack of care, and insubordination which he said he had observed in the hospital.

In the last weeks of the patient’s illness, his ideas became more expansive and extravagant, dealing with a grapevine system of wireless communication and delusions of unlimited wealth. He would at times keep his room flooded with urine and water for the purpose of keeping down the plague which he said was infecting the hospital. Later he mixed food with urine and other ingredients, claiming that he was constructing an elixir of life.

The =autopsy= showed few changes of the calvarium or of the dura mater, nor was the pia mater more than slightly thickened and milky over the frontal poles, along the longitudinal fissure and over the sulci. There were fairly firm adhesions of the pia mater to the dura mater along the longitudinal fissure and over the frontal poles and at the temporal tips. The hemispheres were firmly interadherent, and the cerebello-pontine tissues were covered with a firm leptomeningitis. The floors of the ventricles were smooth and the basal vessels showed little beyond a few spots of sclerosis. There was a generalized increase of consistence. The frontal gyri were rather prominent with wide sulci, but upon section no very marked atrophy of the gray matter could be shown. The rest of the brain failed to show any flaring of sulci or any special evidence of cortical atrophy. The brain weighed 1250 grams; a possible diminution of 100 grams, considering the patient’s body length. However, it must be remembered that he was at this time 63 years of age.

=Microscopically=, the diagnosis of GENERAL PARESIS was confirmed on the basis of plasmocytosis, lymphocytosis, gliotic changes and nerve cell destruction. There was an unusual variation in the degree of the destructive process, which picked out, for example, certain regions of the right side for maximal lesion (cornu ammonis, gyrus rectus, and superior frontal gyrus).

If the patient’s own estimate of 35 years’ duration for his Argyll-Robertson pupils can be trusted (and in general his memory was extremely good), we may well conceive an unusual duration for the process in his case. There was, however, in the body at large no very marked degree of changes. There was a slight old tuberculosis. There was a slight interstitial nephritis, with cardiac hypertrophy and fibrous myocarditis. There was also a sclerosis of the mitral and aortic valves; there were chronic changes in the spleen, liver, and bladder; there was generalized arteriosclerosis of mild degree; there were two round gastric ulcers near the pylorus. The liver weighed but 800 grams, and its left lobe was somewhat rough.

This case is placed among the medicolegal and social cases because the phenomena that ushered in his last illness were mistaken by the local public for meritorious social reform measures. They were regarded as not markedly different from the variety of steps taken by the very active doctor in previous years; indeed the public eloquence that he displayed a year before his death was quite in line with previous habits, despite the suspicious over-brilliance of language. It is an important question, how far the eccentricity and literary overactivity of the latter half of the doctor’s total life can be explained on the basis of a mild syphilitic irritation of the nervous system. In this connection we are tempted to recall the suggestions of Mœbius concerning a portion of the literary products of Nietzsche. Our doctor was by no means so brilliant an exemplar of syphilitic literature as was Nietzsche, if we grant the hypothesis of Mœbius to cover our doctor’s case as well as that of Nietzsche. In the future, important studies of character change under the influence of syphilis will doubtless be made. With modern diagnostic methods, of course, the diagnosis would have been rendered almost at once in the case of Major Isaac Thompson, M.D., and much of his past life would have been brought under special review in connection with the syphilis which doubtless the blood serum or at any rate the cerebrospinal fluid would have shown.

This case illustrates but one of the many social complications arising as the result of paresis. When one recalls that the onset is often insidious and not correctly understood for a period of time, it is readily seen that many unfortunate acts may be committed by a patient. As hypersexual desire is not an infrequent early symptom and as judgment is early disturbed, loose morals may ruin the patient’s reputation. The poor judgment and expansive delusions often lead to foolish business deals wherein the patient’s family is left destitute. At other times the onset is sudden and then the danger of false commands or acts by a person in a responsible position, as a steamship captain, an engineer or chauffeur, may lead to loss of life and property.

=Sudden grandiosity: debts. PARETIC NEUROSYPHILIS (“general paresis”): Question of liability.=

=Case 84.= Lester Smith was a salesman, 31 years of age, who, while on a business trip, accompanied by his wife, suddenly developed grandiose ideas. He originated a scheme of cornering the phonograph market. His prospects seemed so certain to him, that he hired an expensive suite of rooms in a hotel at something over $35 a day. As at the first presentation of his bill it was found that he had no money to meet these charges, he was taken into custody and at once transferred to a hospital for the insane, where it was discovered that he was suffering from GENERAL PARESIS.

1. What is the patient’s responsibility for these debts? Legally the patient or his estate is responsible for debts accruing from services rendered or goods received. As he is adjudged _non compos mentis_ contracts entered into would not hold, and he would not be considered liable for criminal acts.