Tabes dorsalis in the 19th century. The golden age of progressive locomotor ataxia.

Tabes dorsalis, a late neurological complication of syphilis, is nowadays almost extinct. The path to understanding this disease and its pathophysiology was long and winding, spanning multiple centuries. The 19th century was a crucial period for understanding it. In the first third of the century, German and French physicians defined the semiology of tabes dorsalis, renamed in France "ataxie locomotrice progressive [progressive locomotor ataxia]." Nevertheless, the multiplicity of ancient and recent terms and the description of sometimes unclear nosological concepts (tabes nervosa, tabes spasmodic, nervo-tabes, etc.) were a hindrance to understanding it. Tabes dorsalis was a fertile ground for the description of many clinical signs that have become classics in medicine. No real treatment was available and various unusual therapies were performed. For a long time, the etiology of this disease remained unknown. The link between syphilis and tabes dorsalis was slowly established in the second part of the 19th century from epidemiologic observations. We present an overview of the concept of tabes dorsalis in the medical context of the 19th century and discuss the medical observations of some famous patients suffering from the disease such as Édouard Manet (1832-1883) and Alphonse Daudet (1840-1897).

Édouard Manet's Tabes Dorsalis: From Painful Ataxia to Phantom Limb.

Édouard Manet (1832-1883) is considered the "father" of impressionism and even of twentieth century modern art. Manet's genius involved getting away from the classical narrative or historical topics and replacing them by the banality of daily life. Technically, he erased volumes into flat two-dimensional coloured planes, and distorted conventional perspective with often gross brushstrokes intentionally giving an "unfinished" aspect to the work. It is little known that Manet had a very painful second part of his life, due to excruciating limb and chest pains, which developed in parallel with proprioceptive ataxia and gait imbalance. Manet always remained discreet about his private life, and we mainly know that his future wife was his family piano teacher, with whom he had a liaison at the age of 17 years. Later, the great but platonic passion of his life was the painter Berthe Morisot (1841-1895), who married Manet's brother Eugène. In fact, we do not know whether he had a mistress at all, although he had several elegant "flirts" in the mundane and artistic milieu. Thus, while Manet's progressive painful ataxia from the age 40 years yields little doubt on its tabetic origin, how he contracted syphilis at least 15-20 years before will probably remain a mystery. It is fascinating that Manet's daily struggle against pain and poor coordination may have led his art to become one of the most significant of modern times, opening the way to twentieth century avant-gardes, along with another victim of syphilis, Paul Gauguin (1848-1903). Manet never showed any sign of general paresis, and like his contemporary, the writer Alphonse Daudet, his clinical picture remained dominated by paroxysmal pain and walking impairment. Difficult hand co-ordination made him quit watercolour painting, and during the last 2 years of his life he had to focus on small format oil works, the subject of which was nearly limited to modest bunches of fresh flowers, now often considered to be his maturity masterpieces. Having become bedridden, he had to be amputated of one leg, which was developing gangrene, probably associated with ergot overuse. While he died shortly thereafter, we have some witness anecdotes suggesting that he experienced a phantom limb: when Claude Monet (1840-1926) visited him and sat down on his bed, Manet violently shouted that he was sitting on his (absent) leg, which provoked terrible pains. With its facts and mysteries, the subtle interaction between Manet's illness and his work output remains one of the most intriguing stories in the neurology of art.

Édouard Manet's Tabes Dorsalis: From Painful Ataxia to Phantom Limb.

Édouard Manet (1832-1883) is considered the 'father' of Impressionism and even of XXth century modern art. Manet's genius involved getting away from the classical narrative or historical topics and replacing them by the banality of daily life. Technically, he erased volumes into flat two-dimensional coloured planes, and distorted conventional perspective with often gross brushstrokes intentionally giving an 'unfinished' aspect to the work. It is little known that Manet had a very painful second part of his life, due to excruciating limb and chest pains, which developed in parallel with proprioceptive ataxia and gait imbalance. Manet always remained discreet about his private life, and we mainly know that his future wife was his family piano teacher, with whom he had a liaison already at age 17. Later, the great but platonic passion of his life was the painter Berthe Morisot (1841-1895), who got married to Manet's brother Eugène. In fact, we do not know whether he had any mistress at all, although he had several elegant 'flirts' in the mundane and artistic milieu. Thus, while Manet's progressive painful ataxia from age 40 yields little doubt on its tabetic origin, how he contracted syphilis at least 15-20 years before will probably remain a mystery. It is fascinating that Manet's daily struggle against pain and poor coordination may have led his art to become one of the most significant of modern times, opening the way to XXth century avant-gardes, along with another victim of syphilis, Paul Gauguin (1848-1903). Manet never showed any sign of General Paresis, and like his contemporary the writer Alphonse Daudet, his clinical picture remained dominated by paroxysmal pain and walking impairment. Difficult hand coordination made him quit watercolor painting, and during the last 2 years of his life, he had to focus on small format oil works, whose subject was nearly limited to modest bunches of fresh flowers, now often considered to be his maturity masterpieces. Having become bedridden, he had to be amputated of one leg, which was developing gangrene probably associated with ergot overuse. While he died shortly thereafter, we have some witness anecdotes suggesting that he experienced a phantom limb: when Claude Monet (1840-1926) visited him and sat down on his bed, Manet violently shouted at him that he was just sitting on his (absent) leg, which provoked terrible pains. With its facts and mysteries, the subtle interaction between Manet's illness and his work output remains one of the most intriguing stories in neurology of art.

Nerve-stretching in the 19th century.

Operative nerve-stretching was first described in 1872 to relieve incurable pain from sciatica and tabes dorsalis. It became popular for 20 years and numerous articles were published on the subject. It had many complications but relief was only transient and, consequently, it fell into disuse. This paper analyses the literature, contemporary views on the benefits of nerve stretching and its influence on more recent neurological practice.

Historical review: suspension therapy for the treatment of tabes dorsalis.

BACKGROUND: Suspension therapy was developed by a Russian doctor, A. Motschutkovsky and at the end of the 19th century it was a popular treatment for tabes dorsalis. It was endorsed by Jean-Martin Charcot in France and Weir Mitchell in the United States; but after 10 years, it was abandoned because it proved to be useless and some patients developed paralysis. SUMMARY: The effect of suspension upon a spinal cord affected by tabes dorsalis and a healthy spinal cord has been analyzed in the light of current knowledge. The benefits of suspension were thought to be due to an improvement in the blood supply to the spinal cord and due to the suggestibility or the placebo effect. Key Message: Analysis of the contemporary literature in the light of current research shows that suspension therapy was a powerful weapon that could cause impairment to the conductivity of the spinal cord and this has important implications for current therapy such as the use of Harrington rods.

Douglas Argyll Robertson (1837-1909) and his pupil.

Douglas Argyll Robertson's (1837-1909) experimental work with physostigmine in 1863 sharpened his knowledge of the innervation of the internal muscles of the eye. So he was ideally prepared in 1869 to analyse the conundrum when he saw patients with spinal cord disease who had lost the response to light even though accommodation to near objects was normal. By translating his knowledge of basic science to a clinical problem he drew attention to this phenomenon, known subsequently as the Argyll Robertson pupil that came to be considered pathognomonic of tabes dorsalis, general paresis and neurovascular syphilis.

The Romberg sign and early instruments for measuring postural sway.

In the first half of the 19th century, European physicians-including Marshall Hall, Moritz Romberg, and Bernardus Brach-described loss of postural control in darkness of patients with severely compromised proprioception. Romberg and Brach emphasized the relationship between this sign and tabes dorsalis. Later, other neurologists evaluated the phenomenon, which is now known as Romberg's sign, in a broader range of neurologic disorders using a variety of simple but increasingly precise and sensitive clinical tests. In the late 19th century, neurologists also developed instruments to measure and record postural sway in patients with neurologic disease. Principal contributors included Philadelphia neurologist Silas Weir Mitchell and his trainees Morris Lewis and Guy Hinsdale. The efforts of these neurologists anticipated later physiologic studies and ultimately the development of computerized dynamic platform posturography.

The history of tabes dorsalis and the impact of observational studies in neurology.

It is common for a particular aspect of scientific knowledge to undergo a great advance in a brief period of time after the discovery of new investigational procedures that broaden research horizons. Knowledge of neurosyphilis increased markedly during the second half of the 19th century. As revealed by the example of tabes dorsalis, this progress was not related to new research methods but instead to the impetus of careful clinical observations.

James Joyce: steps towards a diagnosis.

Authors whose scholarship is in the golden realm of English literature have not hesitated to make pronouncements on James Joyce's health. A publication in this genre claims he had tabes dorsalis. One feels that an authoritative comment, accepting or rejecting a diagnosis of neurosyphilis, should be provided by the Journal of the History of the Neurosciences.

Neurosyphilis, or chronic heavy metal poisoning: Karen Blixen's lifelong disease.

BACKGROUND: Since the 1490s, the treatment of syphilis has consisted of heavy metals--first mercurial and later arsenic and bismuth preparations. Tabes dorsalis, as described by Duchenne in the 1850s, is made up of various characteristic neurologic symptoms. "Gastric crises," sudden stabbing pains followed by vomiting and diarrhea, was originally included by Duchenne, but later, syphilologists disputed its relevance to syphilis. Poisoning by heavy metals, including mercury, may produce similar pain reactions and tabes-like neurologic symptoms. METHODS: According to an earlier published pathography, the Danish author Karen Blixen (1885-1962), also known under the pseudonym Isak Dinesen, suffered from a lifelong disease described as tabes dorsalis. She got syphilis in 1914 and took mercury pills for a year, after which she experienced a severe mercurial intoxication. The Wassermann reaction (WR) in peripheral blood was positive only once, in 1915, before treatment with arsphenamine (Salvarsan), which she received during hospitalization in Copenhagen in 1915 to 1916. Her spinal fluid was examined several times from 1915 to 1956. Apart from an increased number of cells in 1915, the fluid remained unremarkable and the WR was always negative. RESULTS AND CONCLUSIONS: It was postulated that her illness, ending with a cachectic state, was the result of heavy metal poisoning from the various treatments and not a monosymptomatic tabes dorsalis with negative serology.