Part 10

A sudden outburst of violence brought Mr. Donovan to the Psychopathic Hospital; he was very surly, combative, and difficult to manage, standing 6′ 2″, and weighing 210 pounds. He was oriented only fairly well and his surliness was streaked with humor. He facetiously said that the Psychopathic Hospital was the largest hospital in the country, and that it was, in fact, a horse hospital; that he had come because he liked the surroundings, not to make money; that he was the healthiest man in the world, never having been sick; that the Psychopathic Hospital was a club, for which you have to get somebody to propose your name. There was amnesia and no knowledge of current events. He regarded the food as poisoned, refused to eat, and was very irritable and untidy.

=Physically=, there were few abnormalities, but the pupils failed to react either to light or accommodation, and the knee-jerks and ankle-jerks were absent. There was a slight Rombergism. There was a marked speech defect to test phrases. Both serum and spinal fluid W. R.’s were positive; the fluid showed 41 cells per cmm., there were large amounts of globulin and albumin, and the gold sol reaction was of the “paretic” type.

=ATAXIA OR INCOÖRDINATION=

NEUROSYPHILIS LESION OF PERIPHERAL SENSORY NERVES DIVISION OF POSTERIOR ROOTS TUMORS OR CHRONIC SCLEROSIS OF POSTERIOR COLUMNS SUBACUTE COMBINED DEGENERATION VESTIBULAR ATAXIA FRIEDREICH’S ATAXIA FAMILY PROGRESSIVE HYPERTROPHIC NEURITIS THROMBOSIS POSTERIOR INFERIOR CEREBELLAR ARTERY MARIE’S HEREDITARY CEREBELLAR ATAXIA LESIONS OF CEREBELLUM, TUMORS, ETC. WRITERS’ CRAMP PREHEMIPLEGIA MULTIPLE SCLEROSIS PSEUDO-SCLEROSIS HYSTERIA

CHART 18

=CONDITIONS IN WHICH VERTIGO IS FOUND=

NEUROSYPHILIS HEAD TRAUMA CEREBRAL ANEMIA AND HYPEREMIA MENOPAUSE ARTERIOSCLEROSIS RENAL DISEASE CEREBRAL HEMORRHAGE AND THROMBOSIS INTRACRANIAL TUMORS MULTIPLE SCLEROSIS EPILEPSY (AURA) TOXIC CONDITIONS: alcohol, tobacco, constipation PSYCHONEUROSIS OCULAR DISTURBANCES EAR DISEASE MÉNIÈRE’S DISEASE MIGRAINE

CHART 19

Salvarsanized serum was injected intraventricularly through a trephine opening in the right frontal region. Injections were made through the corpus callosum into the third ventricle. There was progressive symptomatic improvement after each of four injections. In fact, after the fourth injection the patient was allowed to leave the hospital despite the fact that there was only a slight improvement in the spinal fluid findings. The speech defect had entirely disappeared. (Speech defect, according to many authorities, including Kraepelin, is of very grave diagnostic significance.) His memory returned. Mr. Donovan is now able to handle figures rather extraordinarily well. He now has a good insight into his delusions and tells stories about them with great humor.

1. What is the definition of a remission in general paresis? Remissions form a foil to seizures; just as seizures mark a sudden advance in the severity of the disease or may even lead to death; so remissions may cause a sudden cessation of both mental and nervous phenomena in the disease. Whereas the seizures occur most often, according to Kraepelin, in the demented types of paresis, the remissions occur in all cases except in the terminal phase. Kraepelin quotes Hoppe as observing pronounced remissions of long duration in 17% of male and 15% of female paretics. Gaupp observed marked improvement in less than 10%, and very marked improvement indeed in only 1% of his cases. Kraepelin states that such improvements are most frequent in agitated and especially in expansive forms of paresis, and that they are rarer and less complete in the depressive and demented forms. Sometimes the improvement occurs over night, although the full extent of the remission becomes complete only gradually, perhaps in the course of months. The sensorium clears, the disorientation disappears, the delusions retreat, and the former delusions are treated as dreams and imaginations. There is often a good deal of persistent uncertainty as to events during the height of the disease. The nervous disorders are far more obstinate than the mental. Still, both speech and writing may often greatly improve.

Cotton in New Jersey found, among 127 cases of paresis diagnosticated by modern methods during seven years, that remissions occurred in but five, or about 4%, lasting from a half to three years.

2. Does a remission ever amount to a cure? The classical case quoted in this connection is one observed by Tuczek. This case developed a picture of paresis in 1876, at the age of 36; and a remission, or cessation, of symptoms, occurred in 1878; but in 1883, at 43 years, the patient developed a tabes without any trace of mental disorder, which tabes gradually advanced. By the middle of 1898, when the patient was 58, certain symptoms of excitement and confusion occurred, which led to death with dementia, 22 years after the beginning of the disease. Nissl pronounced the cortex to be undoubtedly the characteristic cortex of a paretic. This observation seems to indicate that a clinical remission tantamount to a clinical recovery may occur without the death of the spirochetes engaged. This observation is to be held in mind in connection with all therapeutic work with neurosyphilis.

Nonne states that during his clinical experience of 19 years he had followed 10 cases of paresis with apparent recovery; but of these ten cases, four had to be thrown out by Nonne because the apparent recoveries turned out to be only long and almost complete remissions, finally issuing in characteristic dementia. Of the remaining six cases, perhaps two should hardly be counted as paretic and Nonne rather preferred to term them cases of syphilitic dementia in the sense of a non-paretic cerebral syphilis. At the end, therefore, of his review of observations, Nonne found himself with four cases of true recovery from paresis.

Spielmeyer holds that there is no theoretical reason why paresis might not be cured, since all the different changes that have been described in the disease can be halted, and many of them can be repaired. In particular, he reminds us that the acute infiltrative process, the neuroglia reaction, and the phagocytic action of the large mononuclear cells are distinctly removable processes. (See discussion below under Section V, for apparent cures and remissions occasionally secured under treatment.)

=REMISSIONS of identical appearance occur in PARETIC (“general paresis”) and in DIFFUSE (non-paretic) NEUROSYPHILIS.=

=Case 24.= Michael O’Donnell, a laborer of 48 years, came home, one day, at 5:30, complaining of severe headache. His wife told him he should lie down and, taking him by the arm, tried to help him to the bed. At this moment, O’Donnell lost control of both left arm and left leg, and fell, unable to move but with consciousness preserved. The wife noted that the left side of his face was drawn up and that he drooled. He was at once carried to a general hospital, remaining there for about three weeks, talking at random in a delirious manner and tied in bed. Two intraspinous injections of salvarsan were given, and O’Donnell showed considerable improvement and went home.

However, upon his return from the hospital, he became very wilful, would not remain in bed, and on one occasion actually took the mattress from the bed, carried it to another room, and then returned to his own room and slept upon the springs. He became irritable and emotional, insisted upon going to the hospital, did not go there but upon returning home insisted that he had been there. That night, O’Donnell left the house only partly dressed.

It appears that O’Donnell had been excessively alcoholic, but that before August 15, when he sustained the left-sided hemiplegia above mentioned, there had been no symptoms except that in February he had once been very dizzy. It appears that there had been another dizzy spell, three nights before the paralysis, accompanied by a fall and unconsciousness for about 15 minutes.

=TRANSIENT OR FLEETING PARALYSES=

NEUROSYPHILIS MYASTHENIA GRAVIS MYOTONIA CONGENITA (THOMSEN’S DISEASE) PARAMYOTONIA CONGENITA MYOTONIA ATROPHICA INTERMITTENT CLAUDICATION OCCUPATION NEUROSES FAMILY PERIODIC PARALYSES ETANY EPILEPSY MINOR HYSTERIA MULTIPLE SCLEROSIS APOPLEXY CEREBRAL THROMBOSIS

CHART 20

O’Donnell was brought to the Psychopathic Hospital some six weeks after the paralysis, complaining merely of a slight headache and desirous of treatment. There were no mental symptoms of any sort. =Physically=, O’Donnell was in general not abnormal (there was a slight pre-systolic murmur and a blood pressure of 190 mm. systolic). The pupils were slightly irregular, the left larger than the right; both reacted sluggishly. Both ears were moderately deaf; the tendon reflexes of the left arm and leg were somewhat more lively than those on the right. The systematic =neurological= examination otherwise revealed no abnormalities. The urine was negative. The serum W. R. was positive but the spinal fluid reaction was negative. There were but 2 cells per cmm., and there was a very slight trace of albumin.

1. How shall we account for O’Donnell’s transient paralysis? We might invoke brain tumor, alcoholic pseudoparesis, or some form of neurosyphilis. The diagnosis of brain tumor seems quite untenable in view of the absence of premonitory symptoms and in the absence of intracranial pressure. As for alcoholic pseudoparesis it is true that the patient was excessively alcoholic.

However, against these two diagnoses and in favor of the diagnosis of NEUROSYPHILIS, are the positive serum W. R. and the pupillary reactions (although these are short of the true Argyll-Robertson phenomenon). Dizziness with retention of consciousness and associated with the paralyses mentioned suggests rather a subcortical than a cortical lesion. We are inclined to regard this lesion as probably THROMBOTIC, and to place it possibly in the region of the internal capsule. We are inclined to regard the phenomenon as purely vascular and as not in this case associated with an encephalitis. We are, however, not entirely satisfied with the diagnosis.

2. What shall be said as to treatment? A full-blown left-sided hemiplegia may be produced even when the thrombotic lesion is itself exceedingly small. It is common to explain this on the basis that there is an area of collateral edema about the small necrotic, thrombotic, or hemorrhagic area responsible for the lesion. In short, numerous neurones are functionally rather than structurally affected, or at all events capable of early restitution of function.

3. What is the prognosis in such cases? It appears that now and again patients run for several years without further trouble, both with and without treatment. We are inclined, however, to advocate treatment rather than absence of treatment for a variety of reasons. In the first place, vascular lesions may at any time become associated with meningitic lesions, and treatment by salvarsan may perhaps be counted on to head off this process; secondly, the treatment with iodids may possibly aid in the resolution of a local thrombotic process.

4. What are the prodromal symptoms of cerebrospinal syphilis? According to Nonne, headache, dizziness, sleeplessness, mental symptoms of the irritability group, loss of capacity as to mental work, whether severe or not, and loss of capacity for difficult thinking; also impairment of memory. Nonne does not regard these phenomena as characteristic of syphilitic vascular disease, and calls attention to the fact that in every organic disease the same subjective symptoms occur. The triad—headache, dizziness, and impairment of memory—is for example now counted as a prodromal symptom complex for arteriosclerotic apoplexy (Cramer). Of course, apoplectic attacks occur without such preliminary symptoms: particularly, according to Nonne, the nocturnal attacks.

5. Can the fleeting paralysis be of service in differentiating the diffuse from the paretic form of neurosyphilis? Probably not. In both forms transient paralyses occur as well as the permanent ones. In general, however, the transient paralyses are more frequent in paretic neurosyphilis, whereas the permanent ones occur more often in diffuse neurosyphilis.

=There are cases of NEUROSYPHILIS in which the laboratory signs are positive but in which there are no clinical signs or symptoms (PARESIS SINE PARESI?).=

=Case 25.= Richard Lawlor[6] was admitted to the Psychopathic Hospital, October 29, 1914, being sent there from a general hospital where he had gone on account of a self-inflicted wound of the wrist, apparently made in a period of depression with suicidal intent. Routine notes follow.

=Family History.= Paternal grandparents both died of heart disease. Maternal grandfather died at seventy-two of dropsy. Moderately alcoholic. Maternal grandmother died of shock at fifty-six. Father died at age of forty, after an illness of eight years, from heart disease. Father all his life was subject to fainting spells and headaches. The only paternal cousin died at thirteen months of brain fever. Mother, aged forty-seven, is, to say the least, eccentric. Says “she has several times been given up from tuberculosis.” Two maternal uncles died of tuberculosis, one from rupture, one from heart disease. One uncle who “doesn’t know anything after he has a teaspoonful of liquor.” Several other uncles and aunts whose history is not obtained. Patient is mother’s only child. Mother was twice married. There were several miscarriages by both husbands; patient child by first marriage.

=Past History.= Patient born thirty-two years ago, full term, normal delivery and development. Measles, mumps, and chickenpox in childhood. Subject to headaches since seven or eight years old. Kicked in the face by horse at seventeen or eighteen, not considered serious. Hit by a baseball three or four years ago, leaving him hard of hearing on left side. Married ten years ago; no children because he says his wife needed an operation. He denies venereal disease by name and symptoms. For past ten years has had attacks of depression lasting but a short time, but quite severe. Never caused him to quit work as a barber and he felt better when working. His married life he says was fairly happy except for his wife’s extravagances, and on this account he left her a little over a year ago, and she has applied for a divorce, which he is willing that she should have, but does not wish to give her alimony. He admits moderate alcoholism.

=Present Trouble.= Patient states that since he left his wife a year ago he has felt sorry a number of times. He has wished he had her back. He has felt lonely. He has had six or eight periods of depression in that time similar to those he has had for many years, lasting two or three days, and sometimes a week. These were always precipitated by some cause for worry. In these attacks he feels nervous, sleeps poorly, has little or no appetite, sweats during his work and everything looks black. Several times in these attacks he has had suicidal ideas. Ten months ago he considered taking corrosive sublimate. For a little over a week before entrance to hospital he had been out of work and had been “sporting.” The day before entrance he had a telephone message from his lawyer which upset him somewhat and he walked the floor all night. He had just been shaving when the idea of suicide came to him. He sat down a minute when suddenly the thought “to hell with the world” came to him; he took the razor and slashed his wrist. He does not remember drawing the razor across his wrist. As soon as he saw the blood he felt sorry, called his mother, and was taken to an emergency hospital and then sent to the Psychopathic Hospital.

=Physical Examination.= Patient is a well developed and nourished man thirty-two years of age. Head is normal as to size and shape; there are no scars or marks of injury. Hair and skin not remarkable in any way. Ears negative to external examination. Teeth well kept; two missing, several gold fillings. Tongue very slightly coated. Throat negative. Tonsils easily visible without evidence of inflammation or exudation. Neck, no thyroid enlargement, no abnormal pulsations, no adenopathy. Chest, symmetrical, expansion good, resonant throughout. Breath sounds transmitted normally. No râles or rubs heard. Heart, no enlargement or cardiac dulness. Sounds of good quality, no murmurs heard. Rate regular. Pulses equal, regular and synchronous, and of good volume and tension. Systolic blood pressure 130, diastolic 65. Abdomen, flat, soft and tympanitic throughout; no masses; no tenderness. Liver edge not felt, below costal margin. Spleen not palpable. Extremities negative, except for incised wound on left wrist.

=Neuromuscular Examination.= Pupils are large, round, regular, equal and react readily to light and accommodation. No nystagmus, strabismus or ptosis. No weaknesses or paresis of facial muscles. The tongue projects medially and shows no tremor. The triceps and biceps reflexes are readily elicited, and are quite active, as are the knee-jerks and ankle-jerks. On one occasion it was thought that the tendon reflexes were slightly more active on the left than on the right. This was never confirmed; always afterwards found equal. There was no tremor of extended hands. Abdominal reflexes not elicited. Cremasteric present on both sides. The plantar response is flexor. There is no Babinski, Gordon or Oppenheim. No Romberg. Coördination tests well performed. No speech defect. No sensory disturbances. Urine examination negative.

Wassermann reaction in the serum: Positive, with cholesterinized antigen; negative, with syphilitic fetal liver antigen.

Wassermann reaction in fluid positive on two occasions. Examination of spinal fluid, November 4: globulin +++, albumin ++, 100 cells per cubic millimeter; large lymphocytes, 8 per cent; small lymphocytes, 90 per cent; plasma cells, 0.7 per cent; endothelial cells, 1.3 per cent. November 11, globulin +++, albumin +++, cells 18 per cubic millimeter. November 26, globulin ++, albumin ++, cells 92 per cubic millimeter; large lymphocytes, 13.1 per cent; small lymphocytes, 82.1 per cent; plasma, 1.2 per cent; endothelial, 3.6 per cent.

Gold sol, November 4, 5555432100.

Gold sol, November 26, 3332100000.

=Mental Examination.= On entrance to hospital patient seemed slightly depressed and a bit irritable. This condition lasted two days, after which he was agreeable and apparently entirely over his depression. Even during his mild depression, however, he talked freely. There was no evidence of retardation. He told his story readily. Orientation was intact. Memory excellent. Educational knowledge well retained. There was no evidence of any hallucinations or delusions.

1. Was Richard Lawlor insane?

There was, then, on the mental and physical examination nothing to make a definite suggestion of a psychosis, and the most one could think of was a psychoneurosis or a cyclothymia of at least ten years’ duration. The findings in the cerebrospinal fluid and the Wassermann reactions, however, give us material for thought. Certainly one cannot call the man insane; all who saw him agreed on this point.

2. If Richard Lawlor should some day develop mental symptoms, what would be the genesis of the new psychosis? Though writers such as Fildes and McIntosh, and Swift, have suggested an anaphylactic or hyperallergic explanation for the development of symptoms after a normal interval; such a hypothesis could hardly obtain in the present case. The hyperallergic hypothesis for the development of tertiary neurosyphilis would run to the effect that in the secondary stages there had been a definite disease of the nervous system, which, however, absolutely cleared up, leaving no inflammatory vascular or parenchymatous relics of its existence. Nothing would on this hypothesis remain except a hypersensitisation of the tissues. In some later period of the now clinically normal person, one or more spirochetes from a lesion outside the nervous system are carried into the nerve tissues and there set up an anaphylactic or hyperallergic reaction. It is obviously difficult to prove the correctness or incorrectness of the hyperallergic theory without numerous examinations of the spinal fluid, in clinically normal persons after the secondaries have passed. The present case, so far from demonstrating a normal fluid, demonstrates a highly pathological fluid, even though there are absolutely no clinical symptoms which could be regarded as of nervous origin. The burden of proof at the present time would seem to lie with those who claim hyperallergy in neurosyphilis. We prefer on present evidence to think that at the conclusion of the secondaries a disease process often remains in the nerve tissues despite clinical quiescence.

3. What is the prognosis in the case of Richard Lawlor? The prognosis _re_ neurosyphilis is doubtful. We have, however, boldly termed the condition _PARESIS SINE PARESI_, meaning thereby to suggest that the patient is in considerable danger of the efflorescence of a true diffuse or paretic neurosyphilis. We have no means of telling, however, whether the positive symptoms would be those of a paretic or a non-paretic neurosyphilis. As data accumulate regarding these cases of _paresis sine paresi_, we may be able finally to come upon some case in which trauma shall bring out the clinical symptoms of neurosyphilis. For discussion of this matter, see the case of Bessie Vogel (52) in Part III of this book.

4. Should Lawlor have been brought to a psychopathic hospital? It is a safe working rule to have any person who attempts suicide observed. A large percentage of suicides occur in psychotic individuals and a suicidal attempt is not infrequently the first recognized abnormality. Immediate observation is a necessary safeguard against another more successful attempt.

=Demonstrates SYMPTOMS and LESIONS of PARETIC NEUROSYPHILIS (“general paresis”). Autopsy.=