Art. IV.?ON GENERAL PARALYSIS IN COMBINATION WITH OTHER DISEASES OF THE BRAIN. By T. CLAYE
Lecturer
011
Slental
SHAAV, M.D. Lond., M.R.C.P. ;
Diseases, St. Bai-tholomew's Hospital; Medical Superintendent, Leavesden Hospital.
It is a common saying, and textbooks on the subject declare it, that general paralysis of the insane is a disease sui generis, never grafted upon any other form of insanity; so well-known as to its vagaries by the initiated that although at one time all the signs of melancholia may be present, at another those of mania (whilst either of these conditions may precede the full development of late symptoms, and the disease be said to remain tor a longer or shorter time in what are called the earlier stages), still the melancholia or mania of the general paralytic are not melancholia or mania proper, but are only the first a definite history. Hence the has of a disease which stages of find general paralysis in any known difficulty
they
placing
system of classification,
pathological
or
phenomenal.
That it is a distinct disease no one who has had practical experience will dispute, though in what pathological change the peculiarity consists we do not know. Being a distinct disease there must be a definite pathological area, which does not show itself until after the brain has attained a certain stage of development; but a condition of mania, melancholia, or dementia, in the ordinary way of speaking, may arise at any time of life, and I have no more difficulty in seeing how a person might suffer from the pathological condition which sets up any of these, and then have the special lesion (if there be one) which connotes " general paralysis," than I have doubt that such does occur. I know that it is said that these antecedent states are only the primary stages of general paralysis, and that between the lines the special features of the latter may be read ; but I contend that the conditions may be distinct, and that, to take an example, a person with melancholia of a typical form, deluded and suspicious, refusing food, and with suicidal tendencies, may either end in recovery, death, or fatuity, or may pass into a state of general paresis with delusions of the most exalted kind, and with the greatest difficulty in articulation. Nor is a state of general paralysis incompatible with an originally weak brain. I have never seen it certainly in very strongly pronounced idiocy or imbecility, but a moderate degree of the latter may be
54:
ON
GENERAL PARALYSIS IN COMBINATION
often seen. Imbecility is, of course, a relative term, and a man, the sum total of whose faculties would not entitle him to be
held compos, may still have strong development of some parts of his nervous system, parts susceptible of the special degeneration which causes general paresis. We cannot therefore confine general paralysis to a degeneration attacking intellects Nor does coarse pathology give us any reason once complete. for saying that general paralysis is a distinct disease, incompatible Avith the presence of the others now named, though it is said that we are soon to have some northern light thrown on this subject. Taking the coarse appearances of post-mortems, there are scores of brains where it is impossible to say, without a knowledge of the previous history, but judging only from appearances, that the patient suffered from chronic mania, melancholia, dementia, or general paralysis, and the microscopical appearances cannot as yet be said to be more decisive. There.is no difficulty in seeing how, even granting that there is a special lesion in general paralysis, another affection causing mental and motor symptoms should not precede, accompany, or follow. The curious thing is that the fact is denied, and that
supreme monopolising pathological condition called general paralysis has been set up, fenced-in by boundaries which are fallacious, making a fetish of it which larger experience shows must be demolished as being useless. Arguing from analogy, we can see how vain it is to give this exclusive character to general paralysis; for it is no uncommon thing to have two or more intercurrent diseases in the lungs at the same moment,
a
as constitutional and physical skin diseases may co-exist. Is not general paralysis a compound of signs produced by physical, i.e. mechanical and chemical, and vital changes, and do not the extreme vascularity of the brain, its large amount of connective tissue, andthe intimate relationship of its component parts almost defy such an isolation to exist as many suppose when they speak of the singularity of general paralysis ? In the Annates Medico-Psychologiques, September 1874, Eey has described general paralysis as coexisting with locomotor ataxia, i.e. two distinct nervous affections of the cord, distinct as far as the objective symptoms go; and quite recently Dr.Clouston has described the coexistence of chorea with general paralysis. If we find a person in a melancholic condition for years in whom eventually large delusions and lesion of speech supervene, that I take to be an instance of general paralysis following on a case of true melancholia by virtue of a progressive and perhaps different pathological condition, and I should not consider the primary melancholic state to be in any way connected with the subsequent general paralytic condition from the point
just
WITH OTHER DISEASES OE THE BRAIN.
55
of view
generally held, viz. that the patient had simply an unusually prolonged period of the depressed state which, according to ordinary belief, frequently ushers in the well-pronounced, peculiarities of the general paralytic's condition. If, again, there is a case of mania" of the exalted form, resembling in "
most delusions of the Grand all that is deemed characteristic of the general paralytic, but without the hesitation of
some years the special signs of themselves and the patient dies in the condition of " paralytic dementia," that I take to be a case of general paralysis grafted upon one of ordinary mania, and I should not connect the two in any other way than as coexisting but not necessarily correlated. There are without any doubt cases which are general paralytics from the outset: we can predict a definite course with tolerable certainty. There are others where we cannot be so the most that can be said is where and certain, that " he will become a general paralytic," as often is said ; but those who make this statement have, I think, the idea present to them that they are talking of the disease as a unit, and if they were asked whether or not the patient, who is excited or depressed as the case may be, was in the ordinary condition " " of one whom they would call maniacal" or melancholic,'' would say " no," failing to recognise that there may be an exalted or depressed state which may pass through as such to the end, or which may later on take up the special symptoms of general paralysis.' It is important torecognisetliis superposition of one disease upon another, and the clinical truth that they may be distinguished as running together, at times clashing and confounding one another, at times modifying their respective symptoms in such a way that only the most accurate observation can detect the differences. General paresis has a natural history of its own as yet not thoroughly defined, and " sporting" at times in a most perplexing manner. The handwriting does not declare it. I was told the other day of a person whom most would consider a classical case of melancholia; yet this man's speech is perfect, though his handwriting exactly corresponds with what is considered pathognomonic of general paresis, viz. the uncertainty in the up-strokes of the letters. To me the affection of certain muscles about the eyes has " seemed the most useful line of demarcation for saying now which This I this person is in a state of general paresis." sign, first described in the St. Bartholomew's Hospital Reports, I have found of the greatest service, for it is never absent, and according to my observation precedes the tremor of the mouth muscles and the slip in the articulation of words. Closely regarding a general paralytic in the earliest stages of motor
speech,
and if after
general paralysis
'
announce
56
ON
GENERAL
PARALYSIS
IN
COMBINATION
defect discovers a tremor in the levator labii superioris muscle, and a contraction of the grief-muscles : the corrugatives become supercilii and the occipito-frontalis; these latter become in fact extraordinary muscles of articulation and serve as a point d'appui for the formation of words by the lips, especially words
As a consequence general containing explosive consonants. paralytics often assume a melancholy air; and until I found out the rationale of the expression, I was much puzzled by the coincidence of gay delusions with a sentiment of grief expressed in the face. This is, indeed a false expression, and the diagnosis between it and the real sentiment of grief is that the former is transient whilst the latter is persistent. After his effort to talk the face of the general paralytic assumes a placidity, or rather vacuity, which is never seen in the ordinary most long-standing dement. In masticating food the same grief-expression,
due to the same cause, may be seen. Let me now give one or two instances in proof:? J. A., male, aged 49 years, furniture maker, but never was very good at his business, small in stature, and of low type of cranium. Has extravagant delusions and marked hesitation of speech. Has been five years in the Asylum, exhibiting the same symptoms on admission as he does now. This is an instance of
general paralysis occurring in a man with congenital acquired imbecility, liis frontal and occipital excess being very small, and the measurement of his palate indicating an approach to the idiotic type. He performs a certain amount of manual work, and seems likely to continue some time in his present state. Case of J. P., aged 55 years. Admitted in December 1871, with large delusions and extravagant conduct, saying that he was the Prince of Wales, Archbishop of Canterbury, &c. Head well developed, and he has evidently been a man of considerable mental power. Some doubt existed for a longtime as to his classification in the pathological series, and no motor lesion supervening he was put down as a case of chronic mania with large delusions. So he continued from December 1871 to June 1875, employing himself quietly. At that time I noticed a slight elevation of the eyebrows in talking, and his speech is now distinctly affected, and no one would hesitate to call him a general paralytic. I prefer to call this case one of general paralysis supervening on mania of the amoenic form? rather one of primary general paralysis in which the precursory stage of mental exaltation has preceded for an unusual length of time the motor affection ; he has, in fact, been a general paralytic only since June 1875, when the combination
or
of mental and motor symptoms
was
noticed,
and I should be pre-
57
"WITH OTHER DISEASES OF THE BRAIN.
to find in his brain the appearances of an ordinary case of chronic insanity, with or without those which have been said to of be peculiar to general paresis. Now that the combination mental and motor signs exists it is possible to speak with tolerable accuracy as to the probable duration of this man s life ; but I should be inclined to give him a much longer span months in the view that his paretic condition began twelve since than I should on the hypothesis that general paresis is he never " grafted" on to another form of insanity, but that has been condition for many yeais, in the "paralysed'' really I should add that his ?and that his first stage has been slow. in were eyes examined by Professor Liebreich, who found them all respects normal before the motor signs appealed. Ihis may not be much of a guide to show that he was not suffering from general paralysis, for the fact of the fundus of the eye is disbeing at all at any time affected in general paralysis In tie woith. is it what tor puted; still it maybe taken fundus ot t le case oi J. no affection of the above A.5 quoted^ eye ever did or does exist. He was also examined by Liebreich. and has attempted AN B., male, aged 41 years. Depressed, suicide, refused food, and had to be fed forcibly; in fact, exhibited all the features of a typical case of melancholia. In ?January last he had a maniacal attack lasting threethedays, first giving vent to large delusions, and then I noticed for of affection time the and slight twitching about the eyebrows contwo discriminate to is it speech. Even now possible ditions in this man; one being that of ordinary melancholia, the other of general paresis, the signs varying in intensity from day to day. From what I can find out of the previous history, this man lias been suicidal and "melancholy a long time; llnd though he is now undoubtedly paralysed," there is, I think, but a casual connection between the two states. I he last case to which I will refer is J. K., aged 52 years, ballast man. This man was first admitted as an imbecile ^ doubt. m March 1873, and of his imbecility there could be no He could neither read nor write, was of very low cranial type, work. At and quite incapable of any but the merest routine the request of some of his friends he was discharged, but was re-admitted in May 1874, after a twelvemonth's absence, when his speech was markedly hesitating'. Soon afterwards laige delusions supervened, and he is now in the full swing of general paresis. I mention this case chiefly to show that on an which defective structure a disease may be engrafted
pared
.
the^
originally
ls
generally supposed
objects
to select more
its victims. However desirable it is
intellectually advanced
as
theoretically
to
adopt
a
pathological
58
OX
GENERAL
PARALYSIS.
" " we cannot as yet get rid of mania melancliolia." General paralysis I should reserve as a term for the combination of certain mental and motor symptoms, and I think that in a true case this combination will always be found; whilst we shall gain much by recognising that conditions of general or particular excitement or depression may precede or coexist. Especially shall we gain with reference to prognosis. A person who long remains in the exalted or depressed state without the motor affection has a much better life than the general paralytic; but the advent of speech-hesitation gives a prophetic insight into the probability of the duration of life which was before impossible. I have simply recorded the results of clinical observation, without attempting an explanation of the facts. Why do delusion^ of the grand kind, in all " respects similar to those of a general paralytic," go on for years without much impairing the condition of the patient whilst their associations with motor lesions of muscles not very necessary to the maintenance of the vital processes connotes a speedy deatli ? There must surely be two different processes at work. At all events, precision in diagnosis and prognosis
classification, practically and
"
will be
subject
mania
may have in the same insanity of general delusional melancholia and that of general paralysis.
gained by admitting at the
or
same
that
moment the
we
Lecturer
011
Slental
SHAAV, M.D. Lond., M.R.C.P. ;
Diseases, St. Bai-tholomew's Hospital; Medical Superintendent, Leavesden Hospital.
It is a common saying, and textbooks on the subject declare it, that general paralysis of the insane is a disease sui generis, never grafted upon any other form of insanity; so well-known as to its vagaries by the initiated that although at one time all the signs of melancholia may be present, at another those of mania (whilst either of these conditions may precede the full development of late symptoms, and the disease be said to remain tor a longer or shorter time in what are called the earlier stages), still the melancholia or mania of the general paralytic are not melancholia or mania proper, but are only the first a definite history. Hence the has of a disease which stages of find general paralysis in any known difficulty
they
placing
system of classification,
pathological
or
phenomenal.
That it is a distinct disease no one who has had practical experience will dispute, though in what pathological change the peculiarity consists we do not know. Being a distinct disease there must be a definite pathological area, which does not show itself until after the brain has attained a certain stage of development; but a condition of mania, melancholia, or dementia, in the ordinary way of speaking, may arise at any time of life, and I have no more difficulty in seeing how a person might suffer from the pathological condition which sets up any of these, and then have the special lesion (if there be one) which connotes " general paralysis," than I have doubt that such does occur. I know that it is said that these antecedent states are only the primary stages of general paralysis, and that between the lines the special features of the latter may be read ; but I contend that the conditions may be distinct, and that, to take an example, a person with melancholia of a typical form, deluded and suspicious, refusing food, and with suicidal tendencies, may either end in recovery, death, or fatuity, or may pass into a state of general paresis with delusions of the most exalted kind, and with the greatest difficulty in articulation. Nor is a state of general paralysis incompatible with an originally weak brain. I have never seen it certainly in very strongly pronounced idiocy or imbecility, but a moderate degree of the latter may be
54:
ON
GENERAL PARALYSIS IN COMBINATION
often seen. Imbecility is, of course, a relative term, and a man, the sum total of whose faculties would not entitle him to be
held compos, may still have strong development of some parts of his nervous system, parts susceptible of the special degeneration which causes general paresis. We cannot therefore confine general paralysis to a degeneration attacking intellects Nor does coarse pathology give us any reason once complete. for saying that general paralysis is a distinct disease, incompatible Avith the presence of the others now named, though it is said that we are soon to have some northern light thrown on this subject. Taking the coarse appearances of post-mortems, there are scores of brains where it is impossible to say, without a knowledge of the previous history, but judging only from appearances, that the patient suffered from chronic mania, melancholia, dementia, or general paralysis, and the microscopical appearances cannot as yet be said to be more decisive. There.is no difficulty in seeing how, even granting that there is a special lesion in general paralysis, another affection causing mental and motor symptoms should not precede, accompany, or follow. The curious thing is that the fact is denied, and that
supreme monopolising pathological condition called general paralysis has been set up, fenced-in by boundaries which are fallacious, making a fetish of it which larger experience shows must be demolished as being useless. Arguing from analogy, we can see how vain it is to give this exclusive character to general paralysis; for it is no uncommon thing to have two or more intercurrent diseases in the lungs at the same moment,
a
as constitutional and physical skin diseases may co-exist. Is not general paralysis a compound of signs produced by physical, i.e. mechanical and chemical, and vital changes, and do not the extreme vascularity of the brain, its large amount of connective tissue, andthe intimate relationship of its component parts almost defy such an isolation to exist as many suppose when they speak of the singularity of general paralysis ? In the Annates Medico-Psychologiques, September 1874, Eey has described general paralysis as coexisting with locomotor ataxia, i.e. two distinct nervous affections of the cord, distinct as far as the objective symptoms go; and quite recently Dr.Clouston has described the coexistence of chorea with general paralysis. If we find a person in a melancholic condition for years in whom eventually large delusions and lesion of speech supervene, that I take to be an instance of general paralysis following on a case of true melancholia by virtue of a progressive and perhaps different pathological condition, and I should not consider the primary melancholic state to be in any way connected with the subsequent general paralytic condition from the point
just
WITH OTHER DISEASES OE THE BRAIN.
55
of view
generally held, viz. that the patient had simply an unusually prolonged period of the depressed state which, according to ordinary belief, frequently ushers in the well-pronounced, peculiarities of the general paralytic's condition. If, again, there is a case of mania" of the exalted form, resembling in "
most delusions of the Grand all that is deemed characteristic of the general paralytic, but without the hesitation of
some years the special signs of themselves and the patient dies in the condition of " paralytic dementia," that I take to be a case of general paralysis grafted upon one of ordinary mania, and I should not connect the two in any other way than as coexisting but not necessarily correlated. There are without any doubt cases which are general paralytics from the outset: we can predict a definite course with tolerable certainty. There are others where we cannot be so the most that can be said is where and certain, that " he will become a general paralytic," as often is said ; but those who make this statement have, I think, the idea present to them that they are talking of the disease as a unit, and if they were asked whether or not the patient, who is excited or depressed as the case may be, was in the ordinary condition " " of one whom they would call maniacal" or melancholic,'' would say " no," failing to recognise that there may be an exalted or depressed state which may pass through as such to the end, or which may later on take up the special symptoms of general paralysis.' It is important torecognisetliis superposition of one disease upon another, and the clinical truth that they may be distinguished as running together, at times clashing and confounding one another, at times modifying their respective symptoms in such a way that only the most accurate observation can detect the differences. General paresis has a natural history of its own as yet not thoroughly defined, and " sporting" at times in a most perplexing manner. The handwriting does not declare it. I was told the other day of a person whom most would consider a classical case of melancholia; yet this man's speech is perfect, though his handwriting exactly corresponds with what is considered pathognomonic of general paresis, viz. the uncertainty in the up-strokes of the letters. To me the affection of certain muscles about the eyes has " seemed the most useful line of demarcation for saying now which This I this person is in a state of general paresis." sign, first described in the St. Bartholomew's Hospital Reports, I have found of the greatest service, for it is never absent, and according to my observation precedes the tremor of the mouth muscles and the slip in the articulation of words. Closely regarding a general paralytic in the earliest stages of motor
speech,
and if after
general paralysis
'
announce
56
ON
GENERAL
PARALYSIS
IN
COMBINATION
defect discovers a tremor in the levator labii superioris muscle, and a contraction of the grief-muscles : the corrugatives become supercilii and the occipito-frontalis; these latter become in fact extraordinary muscles of articulation and serve as a point d'appui for the formation of words by the lips, especially words
As a consequence general containing explosive consonants. paralytics often assume a melancholy air; and until I found out the rationale of the expression, I was much puzzled by the coincidence of gay delusions with a sentiment of grief expressed in the face. This is, indeed a false expression, and the diagnosis between it and the real sentiment of grief is that the former is transient whilst the latter is persistent. After his effort to talk the face of the general paralytic assumes a placidity, or rather vacuity, which is never seen in the ordinary most long-standing dement. In masticating food the same grief-expression,
due to the same cause, may be seen. Let me now give one or two instances in proof:? J. A., male, aged 49 years, furniture maker, but never was very good at his business, small in stature, and of low type of cranium. Has extravagant delusions and marked hesitation of speech. Has been five years in the Asylum, exhibiting the same symptoms on admission as he does now. This is an instance of
general paralysis occurring in a man with congenital acquired imbecility, liis frontal and occipital excess being very small, and the measurement of his palate indicating an approach to the idiotic type. He performs a certain amount of manual work, and seems likely to continue some time in his present state. Case of J. P., aged 55 years. Admitted in December 1871, with large delusions and extravagant conduct, saying that he was the Prince of Wales, Archbishop of Canterbury, &c. Head well developed, and he has evidently been a man of considerable mental power. Some doubt existed for a longtime as to his classification in the pathological series, and no motor lesion supervening he was put down as a case of chronic mania with large delusions. So he continued from December 1871 to June 1875, employing himself quietly. At that time I noticed a slight elevation of the eyebrows in talking, and his speech is now distinctly affected, and no one would hesitate to call him a general paralytic. I prefer to call this case one of general paralysis supervening on mania of the amoenic form? rather one of primary general paralysis in which the precursory stage of mental exaltation has preceded for an unusual length of time the motor affection ; he has, in fact, been a general paralytic only since June 1875, when the combination
or
of mental and motor symptoms
was
noticed,
and I should be pre-
57
"WITH OTHER DISEASES OF THE BRAIN.
to find in his brain the appearances of an ordinary case of chronic insanity, with or without those which have been said to of be peculiar to general paresis. Now that the combination mental and motor signs exists it is possible to speak with tolerable accuracy as to the probable duration of this man s life ; but I should be inclined to give him a much longer span months in the view that his paretic condition began twelve since than I should on the hypothesis that general paresis is he never " grafted" on to another form of insanity, but that has been condition for many yeais, in the "paralysed'' really I should add that his ?and that his first stage has been slow. in were eyes examined by Professor Liebreich, who found them all respects normal before the motor signs appealed. Ihis may not be much of a guide to show that he was not suffering from general paralysis, for the fact of the fundus of the eye is disbeing at all at any time affected in general paralysis In tie woith. is it what tor puted; still it maybe taken fundus ot t le case oi J. no affection of the above A.5 quoted^ eye ever did or does exist. He was also examined by Liebreich. and has attempted AN B., male, aged 41 years. Depressed, suicide, refused food, and had to be fed forcibly; in fact, exhibited all the features of a typical case of melancholia. In ?January last he had a maniacal attack lasting threethedays, first giving vent to large delusions, and then I noticed for of affection time the and slight twitching about the eyebrows contwo discriminate to is it speech. Even now possible ditions in this man; one being that of ordinary melancholia, the other of general paresis, the signs varying in intensity from day to day. From what I can find out of the previous history, this man lias been suicidal and "melancholy a long time; llnd though he is now undoubtedly paralysed," there is, I think, but a casual connection between the two states. I he last case to which I will refer is J. K., aged 52 years, ballast man. This man was first admitted as an imbecile ^ doubt. m March 1873, and of his imbecility there could be no He could neither read nor write, was of very low cranial type, work. At and quite incapable of any but the merest routine the request of some of his friends he was discharged, but was re-admitted in May 1874, after a twelvemonth's absence, when his speech was markedly hesitating'. Soon afterwards laige delusions supervened, and he is now in the full swing of general paresis. I mention this case chiefly to show that on an which defective structure a disease may be engrafted
pared
.
the^
originally
ls
generally supposed
objects
to select more
its victims. However desirable it is
intellectually advanced
as
theoretically
to
adopt
a
pathological
58
OX
GENERAL
PARALYSIS.
" " we cannot as yet get rid of mania melancliolia." General paralysis I should reserve as a term for the combination of certain mental and motor symptoms, and I think that in a true case this combination will always be found; whilst we shall gain much by recognising that conditions of general or particular excitement or depression may precede or coexist. Especially shall we gain with reference to prognosis. A person who long remains in the exalted or depressed state without the motor affection has a much better life than the general paralytic; but the advent of speech-hesitation gives a prophetic insight into the probability of the duration of life which was before impossible. I have simply recorded the results of clinical observation, without attempting an explanation of the facts. Why do delusion^ of the grand kind, in all " respects similar to those of a general paralytic," go on for years without much impairing the condition of the patient whilst their associations with motor lesions of muscles not very necessary to the maintenance of the vital processes connotes a speedy deatli ? There must surely be two different processes at work. At all events, precision in diagnosis and prognosis
classification, practically and
"
will be
subject
mania
may have in the same insanity of general delusional melancholia and that of general paralysis.
gained by admitting at the
or
same
that
moment the
we
