Can behavioral variant frontotemporal dementia salvage Semmelweis?
Faraze A. Niazi
Jack E. Riggs
Morgantown, West Virginia, United States
|Ignaz Semmelweis. 1818 – 1865. Age 47 years at death. Via Wikimedia.|
Remember me for the mind I had; not the mind a disease created.
Few physicians have made a more significant observation than did Ignaz Semmelweis.1 In 1847 he took over two obstetric divisions at the Vienna General Hospital. In Division 1, where babies were delivered by physicians and medical students, he noted that the mortality rate from puerperal (childbed) fever was several times higher than in Division 2, where babies were delivered by midwives. Puerperal fever is caused by a bacterial infection, most often originating at the placental site in the uterus. During Semmelweis’ time, this led to sepsis and death within days after delivery. He deduced that the cause for the difference in mortality was delivery by physicians, who often went directly from an autopsy to a delivery, carrying the etiologic agent on their hands. He instituted a policy requiring the immersion of hands in a chlorine solution before performing deliveries, which resulted in an immediate decrease in mortality from 15.4% to 1.8%. Such an important observation and intervention should have resulted in immediate fame and admiration, and though some now refer to Semmelweis as the “Savior of Mothers,”2 such adulation did not occur during his lifetime.3-5
The reasons behind Semmelweis’ failure to gain acceptance for his discovery were complex and all too human.6 Perhaps first and foremost, he did not publish his findings until 1860 despite encouragement from several influential colleagues. He wrote in the preface of his treatise that he had “an innate aversion to everything in the nature of writing.”1 Second, he fell victim to self-destructive pride. When finally offered a permanent position in obstetrics at the Vienna General Hospital in 1850, he felt slighted, turned the job down, and left town because he was not going to be allowed to perform—of all things—autopsies.6
Semmelweis also wrote in his preface, “Because of my aversion to controversy, I did not reply to the numerous attacks on my doctrine . . .”1 However, the very next year he contradicted that statement and published two open letters to his colleagues, causing irreparable damage to his reputation. In those open letters, he accused fellow physicians of being “a partner in this massacre. This murder must cease.”1 He chastised all who “propagate dangerous errors about Childbed Fever,” calling them “ignorant” and their practices “based on the corpses of puerperal women murdered out of ignorance.”1 With the deepest cut of all, he wrote, “You sacrifice individual puerperal women to cadaver infection in order to have more mothers left to murder.”1 It is no wonder that his ideas were not warmly received and accepted by his contemporaries.
Semmelweis would be dead within four years at the age of forty-seven, after four years of increasingly bizarre behavior. “He, who earlier had drunk spirits only at special occasions, now often exceeded the limits of moderation. He began to work more indifferently, his behavior became more unseemly. Although he not infrequently expressed himself freely in earlier times . . . he still led a strictly moral life . . . those around him have noticed a heightened sexual excitement; he, who had been lovingly devoted to his wife, now established relationships with a prostitute and found nothing reprehensible therein . . . He became more negligent in his dress, indecent in his behavior toward both acquaintances and strangers. He became wasteful in his expenses, without providing for his family. He estimated his income high and his expenses low.”7 His wife finally had him committed to an insane asylum on July 30, 1865. He did not survive long, dying on August 13, 1865. Records revealed “. . . three attendants could hardly control him . . . His finger, as well as the back of his entire right hand more swollen. Gangrene grows worse . . . pulse over 100 . . . Tongue hard as a board . . . pyemia? . . . Evening death.”7 At autopsy: “The brain showing shrunken convolutions corresponding to the anterior lobes . . . chronic hydrocephalus. Acute gangrenous osteomyelitis of the middle finger of the right hand . . .”7 The autopsy also “revealed major injuries that could only have been sustained in beatings to which Semmelweis had been subjected while in the asylum.”7 Ironically, the proximal cause of death appears to have been bacterial sepsis, the same final process seen in childbed fever.
Many possible causes for Semmelweis’ decline have been tendered including neurosyphilis,8 Alzheimer’s presenile dementia,9 and manic depressive disorder.5 We suggest that his behavioral dysfunction, neuropathological brain description, and rapid decline are most consistent with behavioral variant frontotemporal dementia (bvFTD). An early-onset and rapidly progressive form of dementia (the average survival after diagnosis is three years), bvFTD is characterized by marked behavioral and personality changes preceding cognitive decline.10-14 Affected patients display early disinhibition and loss of socially appropriate contextual skills and are often initially diagnosed as having a psychiatric disorder.
Had Semmelweis published his work in 1850 instead of 1860, as his influential peers encouraged, his work would have more likely been accepted in his lifetime. Importantly, the socially insensitive vitriol displayed in his open letters published in 1861 had not been evident in the treatise he published in 1860. Semmelweis’ observations, deductions, and interventions regarding childbed fever in 1847-1849 are representative of his true genius, while his behavior from 1861-1865 is representative of his disease, bvFTD.
- Semmelweis IF (translated FP Murphy). The etiology, the concept and the prophylaxis of childbed fever. Birmingham: The Classics of Medicine Library, 1981.
- Kasler M. Ignaz Semmelweis, the savior of mothers. Civic Review 2018;14:385-410.
- Manor J, Blum N, Lurie Y. “No good deed goes unpunished”: Ignaz Semmelweis and the story of puerperal fever. Infect Contr Hosp Epidemol 2016;37:881-7.
- Schreiner S. Ignaz Semmelweis: a victim of harassment? Wien Med Wochenschr 2020;170:293-302.
- Obenchain TG. Genius belabored. Childbed fever and the tragic life of Ignaz Semmelweis. Tuscaloosa: The University of Alabama Press, 2016.
- Nuland SB. The doctors’ plague. Germs, childbed fever, and the strange story of Ignaz Semmelweis. New York: WW Horton & Company, 2003.
- Carter KC, Abbott S, Siebach JL. Five documents relating to the final illness and death of Ignaz Semmelweis. Bull Hist Med 1995;69:255-70.
- Bauer J. The tragic fate of Ignaz Philipp Semmelweis. Calif Med 1963;5:264-6.
- Nuland SB. The enigma of Semmelweis – an interpretation. J Hist Med Allied Sci 1979;34:255-72.
- Piguet O, Hornberger M, Shelley BP, Kipps CM, Hodges Sensitivity of current criteria for the diagnosis of behavioral variant frontotemporal dementia. Neurology 2009;72:732-7.
- Piguet O, Hornberger M, Mioshi E, Hodges JR. Behavioural-variant frontotemporal dementia: diagnosis, clinical staging, and management. Lancet Neurol 2011;10:162-72.
- Manes F. Psychiatric conditions that can mimic early behavioral variant frontotemporal dementia: the importance of the new diagnostic criteria. Curr Psychiatry Rep 2012;14:450-2.
- Ibanez A, Manes F. Contextual social cognition and the behavioral variant of frontotemporal dementia. Neurology 2012;78:1354-62.
- Pressman PS, Miller BL. Diagnosis and management of behavorial variant frontotemporal dementia. Biol Psychiatry 2014;75:574-81.
FARAZE A. NIAZI, MD, is a neurology resident at West Virginia University She is interested in neurocritical care, ethics, and the history of medicine.
JACK E. RIGGS, MD, is a Professor of Neurology at West Virginia University