A VANISHING DISEASE:
GENERAL
PARESIS
Guy de Maupassant (Wikipedia) |
On January 1, 1892,
the famous French writer Guy de Maupassant, in a delirious state, pointed a
revolver at himself and pulled the trigger. He heard only a click, however, as
his valet, worried about his mental condition, had earlier removed the bullets.
Maupassant then stabbed himself superficially and attempted to jump out a
window before his valet rescued him. Committed to an asylum, Maupassant’s
delusions and irascible behavior intensified and, in July of 1893, after a
series of convulsions, he finally met his end. He was 43 years old. His brother
had met a similar fate not long before.
Years earlier,
Maupassant proudly announced that he had syphilis, undoubtedly related to his
frequent brothel visits. By the late 1880s, he was becoming irritable and complaining
of pains in his stomach, head, and elsewhere, preludes to his later sufferings.
His final years were almost certainly due to a late stage of syphilis, known as
general paresis, characterized by delusions, dementia, weakening or paralysis
of various motor functions, and eventually death.
At the onset of the
nineteenth century even the notion that brain disease could cause mental
illness, rather than the other way around, was unclear. A Parisian medical
student, Antoine Bayle, in his doctoral thesis of 1822, explored the issue with a path-breaking doctoral thesis. He noted
that since so many autopsies in mentally ill patients had shown no brain
pathology, theories had arisen placing the seat of “madness” in other organs.
Bayle then described six cases from the Charenton Insane Asylum, near Paris
(where the Marquis de Sade had been confined), who had progressed from
delusions of grandeur to weakness, dementia, and death. In all cases, he found
inflammation of the tissues covering the brain and attributed the mental
disturbances to the inflammatory changes. Not long after, Bayle published on
over 200 similar cases and other authors made similar reports, all boosting the
theory of organic/structural causes for mental illness.
Alzheimer's drawing of involved areas in general paresis (Hist u Histopath Arbeiten v1,1904, Hathi Trust) |
Alois Alzheimer, the
pathologist who described the brain changes seen in dementia, published, in
1904, detailed findings in the brains of paretics, locating the primary damage
in the frontal lobes and meningeal coverings. Franz Nissl, another noted
neuropathologist and a friend, published similar findings in the same journal
issue. By this time general paresis accounted for about 10 to 30 percent of
psychiatric admissions. A relation to syphilis was considered probable, though
with no organism to point to debate was still vigorous. William Osler, in his
1892 text, acknowledged a relationship to syphilis but felt that in the U.S.
the hectic pace of modern life was a major factor causing general paresis,
along with alcoholism and lead poisoning.
Events accelerated the
following year when Fritz Shaudinn finally sighted the tiny spiral-shaped
organism, now called treponema
pallidum, in a secondary syphilis
papule. The next year the Wassermann reaction for syphilis was published, a
valuable diagnostic tool. Yet another year later, Paul Ehrlich announced the
first “magic bullet”, an arsenical, to treat syphilis. Finally, in 1912 Hideyo
Noguchi at the Rockefeller Institute discovered the treponema in the brain and
spinal cord of patients. Doubts disappeared on the cause of general paresis,
though arguments on organic vs psychologic origins for other mental illnesses carried
on.
Julius Wagner-Jauregg (Wikipedia) |
Therapy remained a
problem. Arsenicals were toxic and required a long, painful series of
injections. Further, the drugs penetrated poorly into brain tissue. Some patients with general paresis, however, unexpectedly improved after surviving an
infection, raising the question: could fever weaken the organism? An Austrian
psychiatrist, Julius Wagner von Jauregg, cautiously investigated the effects of
fever. Starting with tuberculin injections, he turned to malaria infection and
saw improvement in a substantial number of paretics. Eventually, malaria
therapy - infusing patients with the safer vivax strains
- became widespread. A variety of heating devices also sprang up to duplicate the
effects of malaria. The results impressed the scientific world enough to award a Nobel Pirze to Wagner-Jauregg. The treatments, however, were only a partial solution. They often produced
remissions rather than cures and worked best in early cases.
Al Capone (Wikipedia) |
A patient that suffered through the full gamut of ministrations was the notorious gangster, Al Capone. Probably
contracting the disease in his teens, mental symptoms were first apparent in
the Atlanta Penitentiary, where he was sent for tax evasion at age 33. Boasting
extravagant exploits, he was diagnosed with megalomania due to central nervous
system syphilis, confirmed with blood and spinal fluid tests. Bismuth (another heavy
metal) therapy was begun. Two years later, this time in Alcatraz, his
megalomania worsened and he received arsenicals and more bismuth. Near the end
of his prison term he was transferred to the care of Dr. J. E. Moore, a world
expert on venereal disease at Johns Hopkins Hospital. Capone was admitted under
an assumed name and treated with malaria infections and possibly more heavy
metal. The trustees of Johns Hopkins Hospital and a second hospital, learning
the identity of their patient, both forced him to leave, so his family rented a
house in Baltimore to finish treatments. To follow the results of therapy,
repeated spinal taps were done under the watchful eye of Capone’s bodyguards.
In March 1940, Capone
moved to his house on Palm Island, Florida, now a weak man, demented, a shell
of his former self. He seemed to stabilize, grew fat, but did not improve
mentally. In 1945 he was given penicillin, one of the first to receive it,
but it did little good and in January 1947, after suffering a “stroke” he died
quietly.
The former scourge of
syphilis is today under better control and
general paresis, the late stage of
brain involvement, is rare. At an earlier time, however, general paresis was
important in the evolution of psychiatric ideas and in the early development of
therapy of infectious diseases before the modern “magic bullets”.
And now to a lighter
subject: HAPPY HOLIDAYS TO ALL AND A HAPPY NEW YEAR!
SOURCES:
Ropper, A H and Burrell, B D. How the Brain Lost its Mind: Sex, Hysteria, and the
Riddle of Mental Illness. 2019;
Avery Press.
Lerner, M G. Maupassant.
1975; George Allen & Unwin.
Hayden, D. Pox: Genius, Madness, and the Mysteries of Syphilis. 2003; Basic Books.
Solomon, H C. “General Paresis: What It
Is and its Therapeutic Possibilities”. 1923; Amer J Psych 79:
623-46.
Whitrow, M. “Wagner-Jauregg and Fever
Therapy” 1990; Medical History 34:
294-310.
A. J. K. “Joseph Early Moore”
(obituary). 1958; Brit J Vener
Dis 34: 58.
Moore, M and Solomon, H C. “Contributions
of Haslam, Bayle, and Esmarch and Jessen to the History of Neurosyphilis”.
1934; Arch Neurol Psychiat. 32: 804-39.
Bergreen, L. Capone: The Man and the Era. 1994; Simon & Shuster.