NeiD Journal for · 4 EARLY RECOGNITION OF GENERAL PARESIS. diagnosis was made. Secondly, the...

THE EARLY RECOGNITIONOF GENERAL PARESIS

(PROGRESSIVE DEMENTIA).

BY

B. SACHS, M. D.,Professor of Mental and Nervous Diseases inthe New York

Polyclinic j President of the New YorkNeurological Society, etc.

REPRINTED PROM THE

"NeiD Yovtt Journalfor July 2 and 9} 1898.

Reprinted from the New YorTc Medical Journalfor July 2 and 9, 1898.

THE r

EAELY RECOGNITION OP GENERAL PARESIS(PROGRESSIVE DEMENTIA).*

By B. SACHS, M. D.,PROFESSOR OF MENTAL AND NERVOUS DISEASES IN THE NEW YORK POLYCLINIC

PRESIDENT OP THE NEW YORK NEUROLOGICAL SOCIETY, ETC.

When the president of this section requested me toprepare a paper on some subject of general interest, itoccurred to me that it might not be unprofitable to dis-cuss the difficulties besetting the early diagnosis of gen-eral paresis. As conscientious physicians we can not failto recognize the grave results that may accrue to thepatient and to those dependent upon him if the diagnosisof paretic dementia has been established. If the patientis a man of affairs, it becomes the physician’s duty toadvise the family that such person is no longer ableto manage his own estate or his own business; or, ifhe is on the point of undertaking some important stepin life, the physician is under the necessity of acting notonly in his medical capacity, but also as counselor andfriend. It is for these various reasons that promptrecognition of general paresis is far more important than

* Read before the Section in Neurology of the New York Academyof Medicine, February 18, 1898.

Copyright, 1898, by I). Applbton and Company.

2 EARLY RECOGNITION OE GENERAL PARESIS.

the early diagnosis of many other diseases. The opinionis common that the suspicion of general paresis in agiven case is usually well founded—if there is reason tosuspect the disease, it is already beyond suspicion; hutthere are exceptions to this rule as to all others, and thepractitioner errs more frequently in failing to recognizedementia paralytica than in mistaking it for some otherdisease.

As in tabes dorsalis, so in progressive dementia, thefull-fledged form of the disease bears its unmistakablesymptoms; but, like tabes, it is often most insidious in itsonset, and its earliest symptoms are variable and poorlydefined. If a patient presents a history of an active,successful life up to the age of thirty-five or forty years;if, after a period of slight irritability or of moodiness, hehas become unmindful of his duties toward his family;if he has become lax in his morals and in his businessmethods; if he fails to keep important engagements;if a change in his mental attitude is indicated by a turnfrom rational economy to wasteful expenditure; if in anyone of a thousand ways he proves that his judgment isdefective and his memory poor, we may well suspectsome form of general paresis. Let the patient, in addi-tion, be in a distinctly exalted mood; let him uttermarked delusions of grandeur, or be subject to epilepticand apoplectic seizures; let him present a distinct reflexiridoplegia, tremor of the face and hands, the peculiarstammering and tremulous speech, knee-jerks that areeither exaggerated or absent, and no one can doubt thediagnosis of the disease. The problem is complicated bythe fact that in point of time the physical symptomsoften, though not invariably, precede the mental, or thephysical signs may be well marked while the mental

EARLY RECOGNITION OF GENERAL PARESIS. 3

phenomena are still in doubt; yet no one should ventureto make the diagnosis of general paresis from the physi-cal symptoms alone, unless there be at least some indica-tion of a change in the person’s psychic state. Ourattention will naturally be directed to those early physi-cal symptoms which are most characteristic of the dis-ease, and which, if they occur in conjunction with anymental defect, point to the existence of progressivedementia.

Before proceeding to the discussion of these details,let us take note of the change which, according to differ-ent authors, is said to have come over the clinical type ofgeneral paresis. Krafft-Ebing * states that such achange has been noted during the past decade; an opin-ion which is shared by Mendel and otherEuropean alien-ists. In this country this same sentiment has been voicedin a recent article by Collins.f That the eases of generalparesis differ somewhat from those observed fifteen andtwenty years ago may well be granted. In a general waythe present writer had been impressed with the truth ofthis view, but a calm and careful study of sixty-two his-tories taken from his private records has shown thenecessity of making a more guarded statement. Thechange referred to has been exhibited in several ways:First, and this seems to be the most important point,paretics appear to live, or to linger, longer than theyformerly did. Hot more than half of my patients suf-fering from progressive dementia have died within aperiod of three years; nine of them have exceeded theperiod of six years, and one is leading a blissful, thoughdemented, existence for more than ten years since the

* Die progressive allgemeine Paralyse, Vienna, 1894.f Medical Record ,

February 5, 1898.

4 EARLY RECOGNITION OF GENERAL PARESIS.

diagnosis was made. Secondly, the unusually long re-missions have often been surprising, and a few recover-ies in cases in which the early diagnosis seemed to bebeyond doubt have been particularly noteworthy. Third-ly, marked delusions of grandeur do not appear to be ascommon as in former days. I have seen fewer of thosewho owned sufficient ships to build a bridge reachingfrom New York to Liverpool, and it is a long while sinceI have met a paretic whose imagination was as vivid asthat of an architect whom I treated some ten years ago,who believed himself commissioned by the King ofBavaria to fill Munich with elegant palaces, each palaceto have windows of diamond instead of common glass.The patient with great poetical talent, with tremendouscapacity for manipulating the stock market, the manwho is going to buy up all the vacant lots to build homesfor the poor, still cross my path. The patient who wasready to pay Dr. Granger one hundred thousand dollarsfor his home and give him a million in addition, and theman who had patented a contrivance by which peoplewere to be brought back from the other world, have am-bitious rivals. There is almost as frequent mention inmy histories of that happy and exalted frame of mindwhich is the paretic’s only blessing. “Never felt betteran my life,” spoken in a way that the phonograph alone-can reproduce, has the same ominous ring as of old. Thedelusions may be less marked, but the euphoria and theexalted mood are as typical as ever. Possibly the hypo-chondriacal state is a little more common in the earlierhistory of the disease; but, if so, the fact can be provedonly by larger statistics than are at my command.

The older writers insisted that that form of generalparesis which was characterized by an exalted mood


took a less violent course than those that were char-acterized by the hypochondriacal state, or b}7 the earlydevelopment of dementia. It would seem, therefore,if the disappearance of the delusion of grandeur as acommon symptom of general paresis mean anything atall, it means that the disease, as we see it nowadays,is less favorable than that of two or three decades ago;and yet the greater longevity of the paretic of to-day isopposed to this interpretation. The better care of thepatient and the earlier removal from his surroundingsmay, however, be factors of some importance. I can notagree altogether with the view that a motor type of thedisease is more prevalent at the present time. Themotor or, better said, the physical symptoms were fullyappreciated by the prominent alienists of twenty orthirty years ago, and had been given due weight in thelater writings of Mendel,* Westphal,fMickle, # Blandford, j | aud others; nor should we disre-gard the fact that in a number of instances the typicalphysical signs have not appeared until after the fulldevelopment of mental symptoms.

Before deciding whether a disease has changed in thecourse of years, it is well to ask whether we are notpossibly beginning to note minute differences between atype as established years ago and other affections whichresemble a given type so closely that it is difficult to dif-ferentiate between them. All of us remember the timewhen progressive muscular atrophy represented a verydefinite and, as we supposed, a spinal disease. We have

* Die progressive Paralyse der Irren, Berlin, 1880.f Archiv fur Psychiatric , i; Gesammelte Abhandlungen , i, p. 204.\ Festschrift , Hamburg, 1891.# General Paralysis of the Insane, London, 1886.

J| Twentieth Century Practice of Medicine , xii.

EARLY RECOGNITION OF GENERAL PARESIS.

passed through the stages in which these atrophies havebeen subdivided into many different groups, so that atthe present time the term “ progressive muscular atro-phy ” conveys no definite meaning as to the part of thenervous system specially affected. The term is now ge-neric rather than specific. Just so some of us recognizethe fact that the clinical type of tabes dorsalis mayrepresent morbid conditions that are to be distinguishedfrom posterior spinal sclerosis. In like manner, too, theterm “general paresis ” includes a number of clinicaltypes dependent upon varying morbid processes. Beforewe decide that general paresis is not so fatal a diseaseas it was a decade or two ago, or that it is at the presenttime more amenable to treatment than it was in formeryears, we must make sure that we are not confoundingwith this disease other diseases whose clinical symptomsare so much like those of general paresis that we find itconvenient to include them under one heading, althoughthe symptoms are due to morbid processes which resem-ble each other chiefly in this, that they lead to the sameterminal condition. How else could we explain the verymarked resemblance in the clinical features between gen-eral paresis of the classic order and syphilitic and alco-holic dementia, or between the former and traumaticmeningo-encephalitis terminating in atrophy of the cor-tical elements?

The problem may be simplified by assuming thatthere are different morbid processes producing the ordi-nary symptoms of progressive dementia. Of the vascularorigin of the disorder in the majority of instances therecan be little doubt; it is not difficult to conceive that thecirculatory disturbance may follow upon acute infec-tions, upon cerebral injuries, or upon a specific endartcr-

EARLY RECOGNITION OE GENERAL PARESIS. 7itis. During the vascular stage the cellular elements(chiefly of the cortex) may exhibit impaired function,but need not be permanently destroyed. Under suchconditions remissions and recoveries are possible, butthey are not conceivable at a time when the long-contin-ued vascular disturbance has led to a destruction of alarge, or of the largest, number of brain cells and fibres.In some few cases the cellular elements may be primarilydiseased and yet not be beyond the possibility of improve-ment and recovery. A recent French writer, Marandonde Montyel,* distinguishes between a progressive and aretrogressive form of paretic dementia, the latter beingsynonymous with pseudo-paresis,which, as he claims, “isalways of toxic or infectious origin, and after an un-usually violent onset reaches a climax and then recedestoward complete recovery.” Such a form, this authorsupposes, never passes beyond the hypochondriacal stage.Further studies in the morbid anatomy of paretic de-mentia must reveal the truth or falsity of these theories.The terminal stages of the morbid process are sufficientlywell known; the earlier stages need investigation. Leav-ing all theories aside, the clinician must decide whetheror not he can recognize those forms which are certainto be fatal and those which hold out a fair prospect ofimprovement or recovery. If this is to be done at all,it must be done when the first signs of cerebral diseaseappear.

In the earlier stages of every form of progressive de-mentia the physical signs arrest our attention. Chiefamong these are, in the order of their importance: (1)The stammering, tremulous speech; (3) the tremor ofthe facial muscles and of the tongue; (3) the pupillary

* Quoted in Revue neurologigue, January 30, 1898.


symptoms; (4) the change in the individual’s handwrit-ing; (5) the exaggeration or the absence of the reflexes.

1. The disturbance of speech is unquestionably oneof the earliest symptoms, and is so characteristic thatone is not infrequently tempted to make the diagnosis ofprogressive dementia if a patient who has shown somemental change has in addition that peculiar stammer-ing utterance which makes the use of words of manysyllables, or of sentences in which there is any allitera-tion, particularly difficult. Yet it occurs at times in per-sons whose mental deterioration is of distinctly alcoholicorigin. I have the histories of two such patients in whomI was led to make the diagnosis of general paresis fromthis and other symptoms, and with the recovery that setin the disturbances of speech disappeared.

2. The tremor of the facial muscles, which occursonly in progressive dementia and in chronic alcoholism,is a symptom of the greatest value. If alcoholism canbe excluded, it is unquestionably a grave symptom, andmay well support the diagnosis of general paresis.

3. The pupillary symptoms have by many writersbeen placed first among the physical symptoms. In sev-eral patients of mine they have not been developed untillong after the appearance of the characteristic speechdisturbances and of the facial tremor. The typicalArgyll Eobertson pupil is common enough, and particu-larly in those forms associated with tabetic symptoms.The complete immobility of the pupils, both to light andduring accommodation, is present in a large number ofcases, and is often associated with inequality of the pu-pils and with the history of preceding ocular palsies, allof which occur more commonly in those who have beenexposed to the syphilitic contagion. The irregular con-


tour of the pupil has been described as occurring inparetics. It is not dependent upon a preceding iritis, ismore probably due to defective innervation, and is, bythe way, often seen in persons with constitutional syphi-lis and also in some young and healthy persons.

4. The changes in the handwriting are of specialvalue, not only as illustrating the tremor of the fingersand of the hand, but as giving the first evidences of thatmental dissolution which is most marked in acts whichhave been performed with the greatest skill. The drop-ping of letters from words that were written with easeand almost unconsciously, the omission of syllables, therunning together of words that should be separated, andthe entire failure to punctuate, may be the first signspointing to serious mental defect. Too much importanceshould not he attached to the tremor alone, for in otherdiseases, and particularly in multiple sclerosis, very simi-lar physical disturbance occurs.

5. The reflexes invite close attention, for, if absent,they may be part of the symptoms of a tabetic processwith which progressive dementia is frequently associated.If exaggerated, great care should be taken not to formu-late the diagnosis of general paresis unless a purely neu-rasthenic condition can be safely excluded.

Without underrating the great value of these varioussymptoms we must remember that the diagnosis of pa-retic dementia can be established only if some one ormore of these physical signs are present in associationwith mental symptoms, however slight these may be.

The normal individual near or past middle life isthe creature of well-established habits of thought andaction. Any departure from the standard which theindividual has established for himself should at all times

10 EARLY RECOGNITION OE GENERAL PARESIS.

be regarded with considerable suspicion, and any changesin the person’s bearing that are not in keeping with hisposition in life—extravagances which he has no right toindulge in, or economy which he need not practise, in-difference to his family, which was not his wont, or sud-den devotion to persons whom he formerly would haveshunned—should be taken to be symptoms of grave men-tal disorder. Defective judgment and, above all, de-fective memory, of which the patient himself frequentlycomplains, are the mental signs that can well be placedin the same scale with the physical signs to which I havereferred above. I need not, however, dilate furtherupon the psychic symptoms of the disease, for I may wellsuppose that they are familiar to all.

In spite of our accurate knowledge of the disease, itsearliest stages may easily be confounded with otheraffections. The first of these is cerebral neurasthenia.The question of differential diagnosis between this recov-erable affection and progressive dementia comes up oftenenough.

Many years ago the late Dr. Mackenzie sent A. M. E.to my office with the request for a diagnosis as to hismental condition. The patient was thirty-nine years ofage, was an excessively hard worker, and had been hap-pily married for five years. His previous history, so faras I could gather it from him and from his wife—a tal-ented lady—was that he had been nervous for a year,■ever since a partner went to Europe, leaving great re-sponsibilities upon the patient’s shoulders. He com-plained of headaches, of difficulty in remembering occur-rences of years ago, of a difficulty in concentrating histhoughts, and also stated that without provocation heseemed extremely irritable; was drowsy all day long.He wras puzzled and anxious about his condition. Hehesitated a little in speech, but this might well have been


due to the excitement of the examination, for he re-peated test sentences that were given him with ease andcorrectly. His pupils were equal and reacted promptly.There was marked tremor of the hands and of thetongue, but not of the facial muscles; the knee-jerkswere lively. I made the provisional diagnosis of cere-bral neurasthenia, but suspected the possible beginningof dementia paralytica. Four days after my first exam-ination I was summoned hastily from my office with thestatement that this patient had had an epileptic seizure.Before I could reach him other physicians, who had beencalled in, had begun to catheterize him, evidently sup-posing that the epileptic attack was due to a urgemiccondition. But to me, who had seen the man a few daysbefore and had had doubts as to his mental condition,the diagnosis was clear at once, and the epileptic sei-zure proved that the man was doomed to general paresis.The epileptic seizures recurred several times during thefirst week, the dementia developed rapidly, and the pa-tient was taken to Bloomingdale, where he died within *aperiod of a few months, having developed all the typicalsymptoms of dementia paralytica.

The patient has appeared to me to be an excellenttype of the most rapidly developing form of generalparesis, and one can hardly suppose that the same mor-bid process underlies the disease in such a case as whenit runs the slow course it does in so many others, coveringa period of years, with remissions of varying duration.

The differential diagnosis between cerebral neuras-thenia and general paralysis is not always established asquickly as it was in the history of the case above men-tioned.

Another patient, whom I saw in September, 1890,complained of sleeplessness and inability to concentratehis mind upon any one thing. He was a well-bred, in-telligent man of twenty-nine years of age; he stammered


a little under excitement, but claimed that this was notnnnsual. The suspicion of dementia paralytica wasaroused, but his pupils reacted promptly; deep reflexeswere normal, and his memory was excellent; the slighttremor of the hands and tongue was not more markedthan in neurasthenic patients. After resting from busi-ness and sojourning in a well-known sanitarium he wasmuch improved. Nine months after the first examina-tion the pupils were still normal. In September, 1891,after a hot summer, he seemed to break down completely;the pupils became unequal, and the right reacted poorlyto light; there was marked facial tremor; knee-jerkswere a little subnormal; speech defect very marked;memory slightly impaired. Upon this there followeda period of improved health, so that he took up bicycling,and showed considerable interest in music and in cur-rent affairs. One day he returned with the statementthat he “ is getting happier all the time,” and from thatday onward there was a continuous and rapid progres-sion of the disease to a fatal issue, about four years afterthe onset. It is noteworthy that in this case, too, thesupposed neurasthenic condition lasted nearly a year,and the mental symptoms appeared long before the pu-pillary phenomena were in evidence.

As a rule, in cerebral neurasthenia the complaints areof a hypochondriacal order; the loss of memory is moreapparent than real, the patient having all his dates andfacts accurately in mind. In writing, his hand maytremble, but he does not omit letters or syllables, and ifperchance he does, he quickly recognizes the omission,and his pupils do not present the altered reactions asso-ciated with the classic form of dementia.

In cases in which the diagnosis is at all doubtful ashort period of observation will generally clear up themystery, for the neurasthenic recovers rapidly under theinfluence of rest; the paretic may quiet down a little, but


the physical symptoms once established do not disap-pear.

Progressive dementia may well be confounded withalcoholic conditions, and the question arises whether wecan distinguish in the earlier stages between alcoholicand other forms of progressive dementia. The questionis well worth considering, for the prognosis differs somaterially that much depends upon a correct answerto this question. A single history will illustrate thepoint.

M. F. D. consulted me in January, 1896. He was amerchant, aged forty-four years, single. Four weeksprevious to his visit to me he was taken with a chillwhile in Chicago, and from that time on had not feltwell. He began to notice that he could not find wordsto express himself. He was much alarmed over thiscondition, returned hurriedly to the city, and on his wayto Hew York noticed that his left arm and leg werenumb. His extremities seemed weak, but he was at leastable to walk. He also felt a numbness in the right handand experienced considerable difficulty in writing. Heacknowledged that he had been drinking for many yearswithout ever becoming intoxicated, and had stoppeddrinking four weeks ago. When asked whether he wastroubling himself about anything, he stated: “I am notfretting about anything. lam one of the healthiest menin town.” He conceded that he was not able to attendto his business, but did not think that it was of any con-sequence, although he was dependent upon it. He statedthat his memory was very good, but when questioned asto the day of the week or the day of the month, waswholly at sea. He was unable to find words for ordinaryobjects, such as paper weight, writing desk, or radiator.When given test sentences, he made a horrible jumble ofthem. When asked to write his name and address, hedid it correctly. He wr rote Philadelphia, Syracuse, andRochester without a mistake, but failed utterly in writ-


ing Constantinople, which he made Constinoble andcould not be made to see that it was not spelled correctly.On attempting to read a paper aloud, he omitted words,and hesitated in reading words of more than two syl-lables, His speech was tremulous and exactly like thatof patients with progressive dementia. The knee-jerkswere absent. There was no Romberg symptom and no-girdle sensation. The grasp was good.

The physical and mental symptoms were so muchlike those of the classical form of general paresis thatone might well have made that diagnosis, but the sub-jective sensations of numbness in the arm and legs, andthe tremor of the hands, which was typically alcoholic,led me to give a somewhat more favorable prognosis thanthe symptoms at the time seemed to warrant. The pa-tient was isolated, put under strict surveillance, and inthe course of the next three months improved to suchan extent that all but the physical symptoms, includingthe speech disturbance, disappeared. His mind seemedclear; he began to interest himself in daily occurrences,and within a period of nine months after my first exam-ination he was able to return to his work, to which he hasattended regularly ever since. As the history reads atthe present time, the diagnosis of an alcoholic dementiaseems evident enough, but there are other cases in whichthe diagnosis is not so readily made, particularly if thealcoholic condition is a more acute one, and if some ofthe other signs of chronic alcoholism are wanting. Ifsuch symptoms as marked tremor of the tongue andhands, paresthesia, and signs of peripheral neuritis arepresent in a case exhibiting some of the typical symptomsof dementia paralytica, one may well hope that thesymptoms are due to the effect of alcoholic poisoningupon the cortical cells, a condition from which the pa-tient may recover, and the diagnosis of dementia para-lytica should be held in abeyance for a considerablelength of time.

In this connection I wish to speak of another patientwho is still under observation, who has been seen by two

EARLY RECOGNITION OE GENERAL PARESIS. 15

other neurologists of this city, and in whose case thephysicians in attendance were called upon to decidewhether the man should he allowed to marry. The obli-gations which a correct diagnosis of the disease in ques-tion entails upon the physician have been brought homevery forcibly to me and others in connection with thisspecial patient.

H. Y., a merchant, aged twenty-nine years, of robustbuild, and a man of high temper. Had always en-jbyed very good health. He had been exhibiting markedchanges in his behavior for four or six weeks be-fore the time of my first examination, in November,1896. He had been smoking on an average fifteen cigarsa day, and stated that during the past few months he hadbeen drinking considerably, but denied having any spe-cial craving for it, having indulged in spirituous liquorsmerely in order to stimulate himself for an excessiveamount of work on his hands. His memory seemed tobe impaired, though he had been filling a responsibleposition in a fairly satisfactory fashion. The mostcharacteristic symptom was a peculiar, rapid, syllabic,stammering speech, which reminded one of the speech ofa paretic, but was claimed to be a mere accentuation of astammering habit which had been evident in former daysduring periods of excitement. During the examinationhe showed considerable impatience, was very certain thatthere was not much the matter with him, did not knowwhy he should be subjected to so many examinations, andfelt that, if he could leave his work for a time and marry,he would soon improve. His mental condition wassomewhat suspicious, but the pupils reacted well; theknee-jerks were a little subnormal, speech was character-istic, and there was a marked facial tremor. After care-ful consideration of the mental and physical symptoms,the condition was diagnosticated by me as a neuras-thenia; the remark was noted in my history that therewas no positive evidence of “general paresis.” Otherphysicians concurring in this opinion, the patient mar-


ried and went abroad. On the trip across the ocean hebecame exceedingly irritable, and after spending a fewdays on the Continent, where he developed a fit of mostviolent temper and extreme irritability, he became thor-oughly confused and was removed, at the suggestion of awell-known alienist, to a private institution. He wasisolated for a number of months, during which time hisreason was supposed to have been entirely gone, and pre-sented symptoms which by some were believed to betypical of progressive dementia, while others claimedthat the symptoms were not characteristic and that re-covery was possible. About ten months after his mar-riage he had so far recovered that he was allowed toreturn to this country and was urged to go back to busi-ness. He has been under my observation again sinceNovember, 1897. His mental condition is even at thepresent time far from normal. He is, on the whole, inan exalted frame of mind, for, although dependentupon his work for his livelihood, he is not in the leastworried about it, and expresses in a rather perfunctoryway his anxiety to return to work. He has had several.attacks of violent temper, but for each one there hasbeen some distinct, though not always sufficient, cause.During the past month he has attempted to make somebusiness connections, but every one whom he meets ap-pears to be repelled and to have his doubts aroused bythe patient’s peculiar stammering speech. He is thor-oughly informed on the subjects of the day, reads thepapers, and can converse intelligently about everythingthat is going on. His speech has, however, undergone oflate some improvement, and it is rather of the stammer-ing than of the syllabic order. A few weeks ago hispupils were noted to contract well under strong light,but were quickly dilated. The contour of the left pupilwas irregular, that of the right entirely normal. Bothpupils reacted well during accommodation. His knee-jerks were normal. He still presents distinct tremor ofthe facial muscles and of the tongue. Up to the presenttime no further changes have occurred, except that the

EARLY RECOGNITION OF GENERAL PARESIS. 17pupils react well and that the facial tremor and thetremor of the tongue have become less than they were.About four weeks ago he had a spell during which hewas not able to speak.

Under the circumstances there is good reason tohesitate in making the diagnosis of a progressive de-mentia, although the mental symptoms argue in favorof it. It is well to note that in this case, in which excess-ive alcoholism, previous syphilitic infection, and possi-bly excessive smoking have been of some etiological im-portance, the ordinary physical symptoms of paretic de-mentia are not fully developed, the tremor and the speechdisturbance being such as we could explain on the sup-position of an alcoholic affection. I refer to the patientin this instance in order to prove that even after thelapse of a year and a quarter there may still be reason-able doubt as to the diagnosis of paretic dementia, and Ithink it well not to give up all hope of the patient’sultimate recovery until further and indubitable signsshall have been developed.*

The differential diagnosis between progressive de-mentia and syphilitic brain disease resembling it calls forcareful discussion. It would be desirable if we couldomit the consideration of brain syphilis, which to manyseems to be more or less of a bugbear, but we might aawell attempt to discuss multiple neuritis without refer-ring to alcoholism as to discuss the aetiology of tabes dor-salis or of general paresis without referring to the syphi-litic contagion. Whether the circumstance be a fortu-nate or an unfortunate one, the fact remains that theclose relationship between specific infection and generalparesis can no longer be doubted. It has been proved inso many different ways that it would seem almost super-

* Since this was written a decided change in the mental symptomshas occurred, leaving little doubt as to the grave character of thedisease.


fluous to insist on this point again and again, if it werenot for the constant reiteration of the doubts of somewho seem to be much troubled by this relationship.Eieger has compiled the results of eleven independentstatistical records showing that in one thousand non-paretics only forty presented the history of a precedingsyphilitic infection, whereas of a thousand patients suf-fering from paretic dementia four hundred had beenpreviously infected with syphilis, and practically allother recent writers have come to similar conclusions.Mendel states that there is a history of a preceding syphi-lis in seventy-five per cent, of paretics from the higherwalks of life and in only 18.6 per cent, of patients suffer-ing from other forms of mental derangement. In thesyphilitic wards of Professor Lang, in Vienna, sixty-three cases exhibiting late manifestations of syphiliswere examined with reference to the history of a specificinfection, and of these positive statements as to the ini-tial lesion were obtained in fifty-four per cent., probablestatements in 9.5 per cent., and in 36.5 per cent, therewas no satisfactory history of specific infection, althoughdistinct signs of late syphilis were in evidence. A com-parison of these figures would show that the previous in-fection has been proved more satisfactorily in the statis-tics of mental cases than in statistics collected of dis-tinctly syphilitic patients. The causal relation betweensyphilis and general paresis has been established by anexperiment which is convincing enough, but which I be-lieve will not be imitated so readily in this country.Recognizing the immunity which syphilitic patients en-joy, a physician in Vienna has attempted to reinoculatewith the syphilitic virus nine patients suffering fromparetic dementia. The experiments were conducted in a


thoroughly scientific fashion and the patients were ob-served, with one exception, for a period of one hundredand eighty days after inoculation. Not a single one ofthese paretics exhibited any reaction upon this attemptat reinoculation. It is evident that this immunity wasdue to the fact that the patients so inoculated had beenpreviouslyaffected with constitutional syphilis. It wouldappear that similar experiments, made as long ago as1854 with other insane patients who had not been pre-viously infected, yielded similar results.*

Although duly impressed with the importance of suchexperimental data, I would not claim syphilis as the solecause of paretic dementia. It is in all probability themost important predisposing condition, but need not besufficient to produce the disease unless some other excit-ing cause, such as overwork, sexual excesses, and aboveall intense worriment and excitement cooperate with it.

If we indorse the preceding statements the questionmay well be raised whether it is necessary to attempt adifferentiation between syphilitic dementia and the typi-cal progressive dementia. But there is need of such adifferentiation; while the large majority of cases of thisaffection may be remotely due to syphilitic disease, thereare evidently some which are directly due to the specificprocess, and which are developed, as a rule, within areasonably short period of time after the initial infec-tion. The conditions are similar to those which obtainin tabes dorsalis. A very large percentage of tabeticpatients have had syphilis in earlier years, yet it maywell be doubted whether the posterior spinal sclerosis is

* For a full statement of these facts see Arbeiten aus dem Gesammt-gehiet der Psychiatric und Neuropaihologie, von R. von Krafft-Ebing, 2tesHeft, Leipzig, 1897.

20 early recognition of general paresis.

primarily specific in origin, although such may by fur-ther researches be proved to be the case. Yet there aresome cases of syphilitic spinal disease which so closelysimulate tabes that they are generally included underthis clinical designation, and these are due, as has beenshownby several autopsies, to a specific meningo-myelitisvery different from the ordinary posterior sclerosis.

It is probable that certain changes occurring in thebrain, particularly in the cortex, in the early stages ofa syphilitic infection give rise to a series of symptomswhich resemble the classical type of paretic dementia soclosely that it is difficult to differentiate between them.For years I have attempted to get at the signs whichwould help us to recognize the truly specific forms of de-mentia, and for this purpose have always regarded thosesymptoms which point to the existence of a generalcerebro-spinal syphilis (such as pupillary immobility,ocular palsies, preceding and transitory apoplexies) asmost valuable signs.

With regard to the prognosis and the treatment thisdifferentiation would be one of the greatest significance.My own experience has shown that the distinctly syphi-litic cases at times yield a more favorable prognosis thanthe others, but do they always recover under antisyphi-litic treatment? Let me refer in this connection to asingle case which is anomalous enough in many respectsto suggest that the morbid process underlying the condi-tion is different from that in the classical type.

In July, 1888,1 had the first opportunity of examin-ing a young chemist, aged twenty-five years, who hadbeen at work for several years, and had managed achemical factory with considerable skill. Nothing outof the ordinary was observed until some months before


the examination, when his relatives noticed that helaughed a great deal and seemed silly and irritable. Theseparation from a partner seemed to have been the spe-cial cause of bringing matters to a focus. There was adistinct change in his manners and in his habits; hewould often do the very reverse of things he had been ac-customed to do, and showed a general defect of Judgmentand some lapse from a former high state of morals.To some old friends he wrote long letters about thesilliest books, took baths at open windows, and to agoverness in his home began to speak in rather shamelessfashion about the syphilitic disease which he had ac-quired while a student abroad. He was fond of present-ing flowers to ladies of his acquaintance, but they veryoften reached the wrong person; he sent them again andagain to elderly ladies, when he had intended them fortheir daughters. At the time of his examination it wasfound that he was well nourished, but had a peculiarvacant stare and presented a marked disturbance ofspeech, halfway between a lisp and the ordinary pareticutterance. He stated that his nerves were shattered andthat he would have them repaired; also that he had beenreading a great deal. When asked what, he statedStevenson’s Arabian Tour and could not be made to givethe correct title. He claimed that he could speak fivelanguages, and each so perfectly that a native could notdetect the difference between his pronunciation and thatof a nativeborn. He knew the common chemical formu-lae, but had evidently forgotten a few of the more com-plicated ones which I could ask him to write. He didnot seem to find it strange that I should be questioninghim. He knew that I was a physician, but could notrecollect my name, although it was mentioned to him anumber of times. He evinced some interest in publicquestions, and when pressed for a statement as to anyparticular one, rattled off a trite phrase regarding freetrade. The physical examination showed a very markedtremor of the facial muscles. The pupils were dilated,unequal, and did not react well either to light or during


accommodation. There was distinct tremor of thehands. Both knee-jerks were exaggerated, and ankle-clonus was present on both sides. This patient was undermy observation for several years, during which time hebecame absolutely demented. Tie had several apoplecticseizures, after which he was so much weakened thatrecovery was not looked for. Yet he rallied from theseand has lived on and on, although he is practically forthe past eight years but a simple vegetating organismwithout the slightest trace of any mental action. I sawhim, quite casually, about a year ago; he was in goodphysical condition, could not utter a single word, made alading sound, but could not appreciate anything that wassaid to him, nor did he evince as much intellect as achild of six months would.

The diagnosis of paretic dementia was concurred inby Spitzka, who saw the patient many years ago, and,although that diagnosis was fully warranted, it is fair tosay that the condition is due to a distinctly syphiliticprocess, possibly a meningo-encephalitis, which has ledto a complete atrophy of the greater part of the cortex.From a clinical point of view, it is important to notethat it is ten years since my first examination, and atleast eleven years since the disease began.

I have the records of another distinctly specific case,which has now lasted over five years. I will not wearyyou with the details, but suffice it to say that the patientwas brought to me some years ago with distinct symp-toms of paretic dementia, exhibiting marked delusionsof grandeur, characteristic paretic speech, immobilepupils, exaggeration of the reflexes, and all the typicalphysical signs. The patient has been confined in severalinstitutions during periods of excitement, in which hewas so unmanageable that he could not be retained athome. lie has had several apoplectic seizures, in one ofwhich all hope of his recovery was abandoned. I gave themost unfavorable prognosis, and the patient was takenhome to the South to end his days there, but, as I under-stand, has recovered so fully that he is once more able toattend to business and is apparently well.


Such histories could be multiplied still further frommy own records, but there would be very little gain inso doing. There are several patients under my observa-tion at the present time whose future is very problemati-cal, including a physician whom I shall not so readilyforget, because he came to me for examination of hisown symptoms shortly after a consultation with himover another patient. This physician developed all thesigns of paretic dementia within a relatively short periodafter his specific infection, which he had acquired duringobstetrical manipulations. For over a year and a half Ihave been watching his condition, hoping almost againsthope that the poor fellow would after all not suc-cumb to the disease, and that in view of the distinctspecific history, and of the fact thatall his symptoms haddeveloped within a few years after a specific infection, along period of remission and usefulness would possiblyensue, or that he would recover wholly from the disease.At the present time his memory is remarkably defective;his knowledge of medicine has dwindled down to such .anextent that he can not remember the names of the mostordinary diseases; he has forgotten the dosage of thesimplest drugs, and he pleases himself by reading overand over again the notes which he took some fifteenyears ago when a student of medicine. lie has the typi-cal speech, and pupils that do not react either to lightor during accommodation. His knee-jerks are exagger-ated and there is slight indication of ankle-clonus. Hereads the papers, but does not know what they contain,and is depressed over the fact that he can not return towork. He is conscious of his illness, but thinks it isnothing more than ordinary nervousness.

It is such cases as this last one that lead one to doubtwhether a distinctly specific form of general paresis ismore favorable than those in which the disease has beendeveloped years after the infection. On the other hand,there are some patients who surprise one by the unusualremission, whose recovery is so perfect that one feels in-clined to doubt the correctness of the original diagnosis,


and in whom it is fair to say the preceding specific infec-tion has been in one sense a boon. Allow me to refer toanother patient, whose career has been most instructiveto me. He was a man of great wealth, carrying unusualresponsibilities; it was necessary, therefore, at the onsetof the disease to take steps to protect his own large inter-ests and his family’s welfare. In this instance I feltcalled upon, after the diagnosis had been made, to askhis lawyer to takefull charge of his affairs and to preventthe patient from participating in the management ofthem. To my great surprise, this patient has recoveredso fully that he seems for the time being to have beliedthe diagnosis, and has pointed the lesson that it is wellto take a more hopeful view of certain forms of pareticdementia than we have been in the habit of doing.

This patient was seen by me first in the summer of1895. He was then forty-three years of age; he hadmade an unusual success in business, and had practicallybeen well with the- exception of a specific infection yearsago. At the suggestion of his family physician he hadgone to Europe to take a course of waters at Kissingenand Carlsbad. During a stay in Berlin he became soexcited that his behavior attracted general notice, andhe was about to be sent to an institution, when his wifedecided that she would take all risks and bring him home.In former years he had been extremely careful in all hisinvestments, but during the summer of 1895 had in-vested extravagantly and began to talk of his greatwealth, a fact which he was in former days most anxiousto keep from every one. He took special delight in hav-ing the most elegant surroundings wherever he was, andalways insisted on having every room in which he wasbrilliantly lighted, whether it was day or night, for hethought this was befitting his station. He was constant-ly making memoranda, and as soon as made would tearthem up; kept every letter that he received, and a dozentimes a day assorted them. He became careless in theuse of money and could not finally state whether billsthat were presented to him were correct. He showed a

great inclination to drive in most extravagant fashion,and could never find horses that appeared to be spiritedenough. At the time of the examination it was notedin addition to the mental symptoms that his knee-jerkswere absent, and he presented distinct Argyll-Eobertsonpupils. There was slight tremor of facial muscles, butno marked tremor of speech. He was isolated in a quietcountry place, put under the care of a competent nurse,was separated from all friends, and above all was pre-vented from going to the city to carry out the huge specu-lations which were on his mind and were entirely con-trary to his former custom. When asked to give me asimple statement of his case in order to test his writing,he wrote a letter in which he said nothing about himself,but went into the most absurd statements as to the greatpleasure he experienced in being under my care, etc.At the present time he seems to me to take a much moresober view of this privilege. His condition had so farimproved that I felt warranted in sending him to Cali-fornia in the Avinter of 1895 in order that he might beseparated from his family and from all former associa-tions. There he did well for several months, but shortlybefore his return home, in the spring of 1896, he passedthrough another period of excitement followed by a con-dition of distinct depression, in which he imagined him-self persecuted by others; thought that he had lost hisentire fortune, that his house was not his own, and thatevery one had forsaken him; but from this depression hesoon recovered, and since that time has been practicallyAvell. I compelled him to abstain from business untilthe fall of 1896, but thenceforward for a period of a yearand a half he has been attending to business actively,and with the exception of the fact that he does not shoAVso much anxiety in the management of his business ashe formerly did (which may in part be due to the vari-ous injunctions given him) he is entirely well. He re-ports to me every few Aveeks, but presents only a fewphysical symptoms. The pupillary reactions have beensomewhat altered as compared AA'ith the test of the first



examination. The left pupil does not react to light norduring accommodation; on the right side there is dis-tinct Argyll-Eobertson pupil. The right knee-jerk cannow be obtained by reenforcement; the left knee-jerkis still absent.* Such changes in the condition of thepupillary reflexes and in the knee-jerks I have observedonly in specific cases.

The improvement in this patient may or may nothave been due to the repeated treatment by inunctionsand by the iodides, but we may suspect the specific char-acter of the entire morbid process, which in this casemay end in complete recovery. I should be willing toconcede the possibility of a prolonged period of remissionin the course of a paretic dementia, but in this patientall the mental symptoms have disappeared to a wonder-ful degree, and the pupillary symptoms as well as theknee-jerks have undergone changes which to my knowl-edge have not been observed in the classic form of gen-eral paresis. Paretic dementia may be mistaken for mul-tiple sclerosis, and vice versa, but this subject I willleave for another occasion.

' Before concluding, it may be well to refer to anotherclass of patients in whom the suspicion of general paresisis justly aroused, and yet the cases go on to full recovery.

In 1890 I saw in conjunction with Dr. StephenSmith a young man of twenty-one years who had justfinished his college career with highest honors. He hadalways been a hard student. Shortly after his gradua-tion he went to the South and was pushed into workwhich was not altogether congenial to him. He cameNorth to escape the intense heat, when he was taken downwith what Dr. Smith pronounced “ a typhoidal con-dition.” On the fourth day he became excited and devel-oped magnificent schemes. Ten days after the onset ofthese symptoms I found him without fever but in anextremely restless condition, speaking of colonizingschemes in Africa. He was about to go to MadisonSquare Garden to start a great project involving hun-

* The left knee-jerk can now be obtained by reenforcement.


dreds of millions, but lie was willing to let only a fewfriends into the scheme. His pet idea was to givelarge sums to every fraternity in every college, for whichhe knew the fraternities would be duly grateful. Hispupils were sluggish, his hands tremulous, his speech wasexcited, and test sentences could be repeated with diffi-culty. Reading was accomplished with less trouble.His knee-jerks were lively. He seemed entirely uncon-scious at the time of the examination, boasted of hisunusual vigor, of his great mental superiority, having afeeling of pity for all others, and could not understandwhy physicians had been called in to examine a man whocould give others a lesson in good health. The physicalsymptoms, taken in conjunction with the maniacal state,would have led to the diagnosis of paretic dementia ifit had not been for his extreme youth and for the entireabsence of any history of preceding or hereditary syphi-lis. After two weeks in an asylum his mind seemed toclear up, but he still developed extravagant schemes,boasted of his great historical attainments, and for along time was certain that a history which he contem-plated writing would startle the students of history hereand abroad. In the course of another few weeks hebecame much more sober in thought, his pupils began toreact promptly, but a slight tremor of the hands and ofthe tongue continued for some months. In November,1890, he had recovered to such an extent that he wasallowed to begin to study law, in which career he hasmade a considerable success. I heard nothing more ofhim until he became prominent in the recent reformmovement in this city and was nominated for an impor-tant office. I venture to believe that his enthusiasm forthis cause can not be interpreted as a sign of failing in-tellect.

I have had two other similar experiences, and, whileparetic dementia does occur, though rarely, in youngerindividuals, I referred to this special history in order tocall attention to the fact that delusions of grandeur as-


sociated with the usual physical symptoms, if occurringin younger persons, need not portend the development ofthis dreaded disease.

The preceding remarks should be interpreted as abrief statement of opinions that have been formed andbased upon a very considerable number of cases of pareticdementia seen in private practice. My hospital and dis-pensary histories I have not had time to analyze. Theconclusions presented for your consideration are:

General paresis or, better still, paretic dementia isa convenient designation for the clinical manifestationsof a number of different morbid processes affecting thebrain, and leading ultimately to an atrophy and destruc-tion of cerebral (chiefly cortical) elements. The clas-sical type of paretic dementia represents the severest ofthese diseases, and is fatal in fully ninety-five per cent,of the cases; but it is well to bear in mind that there areother forms of disease closely resembling the main type,which can scarcely be differentiated from it, and yetseem at times to yield a more favorable prognosis.

It is for this reason and from the result of actualobservation that I would urge a careful consideration ofthe earlier stages of every form of paretic dementia, andwould insist that the possibility of prolonged remissionsor of a complete recovery be kept in mind.

It may be conceded that the disease has undergonesome changes, but in all probability some of these aredue to the fact that other forms of disease are becomingmore frequent or that we recognize them more readilythan we once did.

The greater duration of life after the disease hasbeen recognized may be due to the better care which thepatient receives, but possibly also to the earlier recogni-


tion of the first symptoms. Among these symptoms theevidences of mental derangement are of first importance,for on the strength of the physical symptoms alone adiagnosis is not warranted. Bnt with the appearance ofany evidence of the characteristic mental derangementthe importance of the physical symptoms can not be over-estimated, and among these early signs the facial tremor,the stammering, tremulous speech, and the abnormitiesin pupillary reaction are the most characteristic. Thepupillary reflexes should be studied carefully with refer-ence to the evidence that they supply of a distinct andactive alcoholic or specific poison.

Finally, I would venture the statement that thesymptoms commonly interpreted as those of a progressivedementia do not necessarily indicate the presence of afatal disease, whence it follows that in every instancethe patient should be given the benefit of proper treat-ment. Absolute mental rest, total abstinence, separationfrom an irritating environment, mild sedatives, and, insome instances, a rigorous antisyphilitic regimen will beof distinct value.

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