CHAPTER XV. ETIOLOGY. While a large amount of definite knowledge has been the product of extensive investigations into the etiology, clinical history and pathology of this dis- ease, there remains much to be explored in these sev- eral fields, and even at this late day it may be said that the comparative value of many vexatious ele- ments continues to be a subject of dispute. Doubt- less the following concise summary of causes as given by Chapin^ finds wide concurrence: 'The his- tory of the large majority of cases is one of intemper- ence, licentiousness, sexual excess, syphilis or some nervous exhaustion incident to excessive application to business, or the great strain attending reverses.' This view to be acceptable to some authors needs to be qualified by the revision that the results of heredity should be acknowledged as an important predisposing cause. There are prominent writers who claim syphilis as the sole etiological factor; again, others (and they appear to be numerous) who estab- lish this 'unitarian' view on a neuropathic basis, meaning by this term a susceptibility to an invalid brain, which may be either inherited or acquired. <Callout type="important" title="Important">Heredity is often underestimated in the etiology of general paresis.</Callout> Berkley holds the same view, basing his opinion upon the errors of development of brain convolutions and of the defective growth of the hemispheres, and especially upon the microscopical evidences of irregular con- struction and anomalies in the cortical cells. Folsom says: 'My experience leads me to the conclusion that in those cases of general paresis without a previous history of syphilis the vast majority occur in families in which there have been cases of insanity, epilepsy or apoplexy.' Regis thinks the most important predisposing cause of gen- eral paresis is the congestive or cerebral tendency, usually the result of heredity. He adds that the dis- ease has its source in an heredity that is not vesanic but cerebral, arthritic, or congestive and cites as authority Lunier, Doutrebente, Baillarger, Ball, Lemoine and Pierret. As to paretics who are the offspring of insane parents he notes that this particu- larity shows itself in the vesanic, remittent or circu- lar form, that is to say, 'It is imposed on general paresis by the predominance in the subjects of the paralytic insanity over the paralytic dementia.'* He speaks also of having found many times consan- guinity in the ancestors of general paretics. The family history in all types of insanity is always of importance, for while the disease may not reappear in <Callout type="risk" title="Risk">the same form</Callout>, a record especially of apoplexy, epi- lepsy, or alcoholism, should put the physician on his guard; for there can be no doubt that defect, deteri- oration, or vitiated quality of brain, the necessary re- sults of these conditions, are strong predisposing agents toward paresis. And, on the other hand, from paresis is transmitted, not usually a tendency to the same disease, but a general tendency to organic and functional mental disorders. The percentage of heredity is lower in private than in pauper cases, and lower in males than in females. One fact should be noted in this connection, that paretics usually leave small families ; about one-third of the marriages are sterile and the families of the remaining two-thirds average only one and one-half child. <Callout type="beginner" title="Beginner">Heredity plays a significant role in general paresis, even if it is not always evident.</Callout> GENERAL PARESIS DUE TO HEREDITARY INFLUENCES. Twin brothers, with a strong family history of insanity, both sanguine and keen in temperament, of very active habits, both indulging to great excess in wine and women ; both following a similar occupation — an exciting one — and both were affected with general paresis within a year of one another. (Abstract, Clouston & Savage, Journal of Mental Science, Vol. 34, p. 65.) BOTH PARENTS WERE ALCOHOLICS. Two brothers under treatment for general paresis, their father was an alcoholic and died of cerebral apoplexy, their mother, a highly educated woman of violent temper, was also an alcoholic. In one of them the disease was attributed to sun-stroke. They were of good physique, keen, ambitious and passionate, both alcoholic and one of them, at least, excessively sexual. They followed the same occupation, a very trying and exciting kind of life and were conspicuously successful. Both acquired general paresis between the ages of forty and forty-five years. (Abstract, G. R. Wilson, Journal of Mental Science, Vol. 38, p. 33.) 190 ETIOLOGY. BOTH PARENTS WERE ALCOHOLICS. A. B.'s ancestors had had numerous breakdowns from neuroses of the higher levels. His father was a shrewd, steady, successful business man ; his mother an energetic pious wife. He had a full cousin of both sides who died in the Crichton Institution, Dumfries. The fathers were not strikingly alfke but the mothers were. At the height of a busy, immoral life the case ended in general paresis. (Abstract, G. R. Wilson, loc. cit., p. 34.) A CASE OF GENERAL PARESIS IN A DEGENERATE. Young man of 25, always regarded as simple-minded, but tall and well developed, after business worry, mani- fested considerable exaltation, followed by a period of comparative well-being, with 'faulty and slight mental enfeeblement,' succeeded by a state of acute resistive excitement, ending fatally. (Abstract, Hotchkis, R. D., Glasgow Medical Journal, June, 1897.) DEGENERATION AND GENERAL PARESIS. A degenerate man, under M. Magnan's care for some time, who developed general paralysis, was ultimately admitted under Professor Joffroy's care as an illustration of the view which he holds, that a morbid heredity, more or less marked, or degeneration more or less obvious, is a fre- quent if not indispensable factor in the etiology of general paralysis. The patient, a foundling born in 185 1, of feminine build and habits in youth, became strongly addicted to sodomy after the age of 15, and led a life of debauchery in Paris. In 1893 his memory began to fail and he was arrested for occupying some one else's bed (mistaking ( ?) the story of the house). He was sent to the asylum ; delusions, loss of memory, unequal pupils and slight affection of speech were noticed. In May, 1895, he came under Professor Joffroy's care. His condition did not vary much till 1897, during which interval he had been working as a tailor in the asylum, I HEREDITY. I9I now he became more and more demented with fleeting delusions. In November, 1897, he took to his bed, and had general tremors with dirty habits. In Januar}% 18989 his speech became incomprehensible and he died after getting weaker physically and intellectually, in March, 1898. (Abstract, Revue de Psychologic, 98, No. 10.) THE DAUGHTER OF A PARETIC INHERITS TABES. Heredo-tabes in a young girl with marked congenital syphilis whose father died of general paralysis but with no history or signs of syphilis. (Abstract, Mott, Journal of Mental Sciences, Vol. 55, p. 693.) A PARETIC THE SON OF AN INSANE FATHER AND PARETIC MOTHER. J. R. B., set. 38, married, no children, a hard drinker, developed general paresis which made slow progress; father died of acute mania, mother of general paresis. A CASE OF PARESIS WITH A NEUROPATHIC DIATHESIS. J. H., aged 32, married, no children. First attack, duration two years ; he was admitted in February and died in October. He was a hard drinker ; grandfather insane ; his father a steady man, died of phthisis. A CASE OF PRECOCIOUS GENERAL PARESIS OF NEUROPATHIC HEREDITY. A boy of neuropathic heredity, both paternal grand- parents having had paralytic troubles, a cousin having been insane and his father formerly intemperate. No evidence of syphilis. During childhood he was healthy and a good scholar. At 14 he was put to work. After a month, his intelligence began to fail and he had to be told everything that he had to do ; he wrote badly and could not make arithmetical calculations; he seemed changed, taciturn and silly ; he stammered at times and his hands trembled when tired. On admission, he had wet his bed for a month; backward in physical development; slight 192 ETIOLOGY. evidences of puberty though 17. His expression was dull, walk clumsy, all movements awkward. His mind was much enfeebled, he seemed apathetic and indifferent. Memory poor, no delusions ; tremor of tongue and lips, extending at times to other facial muscles ; articulation imperfect, especially when tired and with the lingual consonants ; tremulous hands, clumsiness of handwriting with tendency to omit and misplace ; inequality of pupils ; attacks of formication, beginning in right foot and involv- ing the whole right side ; headache, general muscular weakness, no localized paralysis, knee-jerks exaggerated. (Abstract, Charcot, Archiv de Neurologie, March, 1892, vide American Journal of Insanity, Vol. 49, p. 76.) GENERAL PARESIS IN MOTHER AND CHILD. General paralysis in a child of 11 and in the mother at 45. There was a neurotic family history and the mother had had syphilis. One younger child died at an early age of convulsions, said to have been caused by congenital syphi- lis. The mental affection first was present in the daughter, who, up to the age of 10 or 12, was a very promising girl. The first symptoms were those of inability to skate as well as usual. The writing soon became affected and her dullness gradually increased to helpless dementia. The mother began to be affected a few years later than the daughter. The first symptom was extreme jealousy. Later she became very indolent and careless of her per- son, she began to drink freely and was unmindful of her household duties. Paresis of the muscles of articulation was an early symptom and the disease progressed in typi- cal fashion. (Abstract, Mueller, Allgemeine Zeitschrift fiir Psychologie, 55, 98, p. 151.) GENERAL PARESIS IN CHILD AND TABES IN MOTHER. The father was alcoholic and infected the mother with what was apparently syphilis, three or four years before the birth of the child. When aged forty-four the mother developed tabes and at the age of seven, following a severe attack of scarlatina with nephritis, the child showed signs k SYPHILIS. 193 of beginning dementia. Her disposition changed and she developed a general fine tremor. Later she had an epi- leptiform attack and subsequently developed typical gene- ral paresis. (Abstract, Grannelli, Rivista, Psich. Neuro- pat., 2, 98, p. 213.) Syphilis. — In the estimation of many authorities syphilis is regarded as the most common cause of general paresis. Bonnet and Anglade have held that in seventy to ninety cases out of a hundred in general paresis syphilis has existed. Bannister gives the percentage as 89, Houghberg, 75.7; Mendel, 75; Berkley, 50, and Graf, 40 per cent. According to Kraepelin the subjects of syphilis are from sixteen to seventeen times more liable to general paresis than others not so affected. The tendency of the age is to regard the cases of general paresis where syphilis has existed as a parasyphilitic disorder. The exact relationship betw^een syphilis and general paresis has not been solved, although it has been under active discussion for a long time. Mickle quotes the statistics of Lewin in which, out of 20,000 cases of s)'philis, only one per cent, became insane and not one case of gen- eral paresis developed. The pathological processes of syphilitic brain disease and general paresis are dif- ferent. In syphilis there are changes in the blood- vessels, and the formation of gummata, or diffuse meningeal infiltration. The first and third occur about the base of the brain, while the second is more apt to appear in the cortical region. On the other hand, in general paresis there is a chronic meningitis of the convexity with atrophy of the cortex. Some years ago Peterson made a study of syphilis as an etiological factor of paresis, which comprised an ex- amination of the contributions of no fewer than seventy authors and his conclusions in this connec- tion are interesting, (i) A history ot syphilis is found in sixty to seventy per cent, of cases of general paralysis of the insane. (2) The fact must not be lost sight of that in thirty to forty per cent, of these cases no history of syphilis, congenital or acquired, is to be found. (3) Antecedent syphilis is seven to ten times more frequent in general paralysis than in other forms of insanity. (4) Syphilis is, therefore, to be looked upon as a frequent, but not constant, fac- tor in its production. (5) But paralytic dementia is not a form of specific disease, not a late syphilitic manifestation, nor is it a form of degeneration de- pending upon the syphilitic poison for its origin. (6) The relationship of syphilis to general paresis lies in the facts that it is a widespread disorder in all communities, that it weakens the constitution and vitiates the blood in many in whom it infects, and that the system is thus prepared in many cases for the direct operation of the final etiological factors of general paresis, viz., alcoholism, excessive venery, heredity and mental overstrain and excitement. The failure of syphilitic remedies to arrest the course of general paresis even when there is a his- tory of syphilis preceding is further evidence of the difference of the processes. The two prominent sup- positions current, explaining the mode of infection in syphilitic cases, are: (i) That the paresis is not due to the direct action of the syphilitic virus but that it is caused by a parasyphilitic poison the result of some remote nutritional or tissue changes, initiated by syphilis. (Fournier.) (2) That the cerebral tissues, profoundly exhausted by the infection of syphilis, are less resistant to the influences of ordinary causes. 'It is significant,' says Dercum, 'that, for the most part, paresis in syphilitic subjects is a late develop- ment. In Houghberg's cases, eighty-one in number, I SYPHILIS. 195 the onset occurred in from five to nineteen years after infection.' GENERAL PARESIS FROM SYPHILIS BEFORE MARRIAGE. WIFE INFECTED. William B. J.; married; set. 36; photographer; no neurotic history ; first attack. He contracted syphilis before marriage ; he had but slight secondary symptoms and married two years later. His wife had no children but developed secondary syphilis and has for years been a martyr to all sorts of troubles due to this source. She now has syphilitic laryngitis. The patient has had no cranial nerve paralysis but has been greatly distressed by his wife's sufferings and also by business worries. Eighteen months before admission, he began to lose his memory ; four months before admission, he had severe headaches; hallucinations of sight; right pupil large; walk feeble, tottering ; knee reflexes brisk. On admission, he showed confusion and a weak mind ; restless and inco- herent ; pupils unequal ; skin greasy ; labial tremors and twitchings ; great physical weakness ; loss of vesical con- trol ; exaltation ; optic discs hazy, probably due to old syphilitic retinitis. He had cystitis and once hemorrhage from the urethra and hematoma in right ear. Discharged uncured after a year. (Abstract, Savage, Transactions Ninth International Medical Congress, Vol. 5, p. 409.) GENERAL PARESIS DUE TO CONGENITAL SYPHILIS. J. B., aet. 18, paternal grandfather died in asylum; patient's father had been a 'show case' of syphilis and he is now convalescing from an attack of hemiplegia and is pathologically exalted on every point, especially on his syphilis. The patient, as a small and sickly infant, had convulsions a few hours after birth, but had good health till fourteen ; active, intelligent, with considerable musical talent. When sixteen years old he had convulsions for two days, followed by slight mental deterioration. At sixteen, fits returned producing more marked mental change, insomnia, change of temper and loss of memory. Nine 196 ETIOLOGY. months before admission he had four very severe epilepti- form seizures, and two months later he had one fit which left him 'paralyzed.' He has had several further attacks during the six months before admission. On admission, physiognomy characteristic of congenital syphilis ; head small and misshapen, with other signs. His pupils were irregular and unequal, responding sluggishly to accom- modation and light and very slightly to the sympathetic reflex. There is general tremor and twitching of facial muscles, plantar, knee-jerk and cremasteric reflexes very exaggerated and ankle clonus well marked ; gait is un- certain, hasty and tottering. There is general cutaneous hyperesthesia ; tongue movements jerky, and its extrinsic muscles tremulous ; speech characteristic of general par- esis. Patient smiles and grimaces. It is difficult to arrest his attention, as he is busily engaged in gathering up and secreting any rubbish that is about; he shows marked dementia. He can tell his name but almost nothing else. He says he is 'very happy' and in a silly way spars with those about him, but a moment later he cringes as in fear and whimpers like a beaten cur. Two months after admission he had a slight epileptiform seizure, followed by paresis of right side, and spastic rigidity of right side ; control over rectum and bladder lost; pupils widely dilated, unequal and sluggish to
Key Takeaways
- Heredity is a significant factor in the development of general paresis, even if it may not always be obvious.
- Syphilis is frequently cited as the primary cause of general paresis, but hereditary factors should also be considered.
- The relationship between syphilis and general paresis remains complex and not fully understood.
Practical Tips
- Be aware that a family history of mental illness or alcoholism may indicate a higher risk for developing general paresis.
- Recognize the importance of hereditary factors in assessing potential cases, even if there is no clear history of syphilis.
- Consider the possibility of congenital syphilis when evaluating patients with neurological symptoms.
Warnings & Risks
- Do not assume that a patient's condition is solely due to syphilis without considering other hereditary factors.
- Be cautious in attributing all cases of general paresis to syphilis, as many cases may have no history of the disease.
- Understand that the relationship between syphilis and general paresis remains complex and not fully understood.
Modern Application
While the historical techniques for diagnosing and treating general paresis are outdated, understanding hereditary factors and recognizing the importance of a comprehensive family medical history can still be valuable in modern emergency response. The knowledge that certain neurological conditions may have genetic components is crucial for early intervention and prevention strategies.
Frequently Asked Questions
Q: How does heredity play a role in general paresis?
Heredity plays a significant but often underestimated role in the development of general paresis. Cases where there is no history of syphilis are frequently found to have a family history of insanity, epilepsy, or apoplexy, indicating that hereditary factors can predispose individuals to this condition.
Q: What percentage of cases of general paresis are attributed to syphilis?
Many authorities estimate that between 60% and 90% of cases of general paresis have a history of syphilis, making it the most common cause. However, there is also evidence suggesting that in about 30-40% of cases, no history of syphilis can be found.
Q: Why is the relationship between syphilis and general paresis still not fully understood?
The exact relationship between syphilis and general paresis remains complex. While it is widely accepted that syphilis significantly increases the risk of developing general paresis