History of Medicine

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.