Who was first to diagnose and report neuropathic arthropathy of the foot and ankle: Jean-Martin Charcot or Herbert William Page?

In November 1883, Jean-Martin Charcot and Charles Féré reported on bone and joint disease of the foot in cases of tabes dorsalis, and referred to the condition as 'pied tabétique'--a disabling neuropathic osteoarthropathy that we usually now refer to as the Charcot foot. Charcot had originally described neuropathic osteoarthropathy in more proximal joints in 1868, and in his 1883 paper with Féré stated that involvement of the short bones and small joints of the foot had not yet been described. They emphasised in the paper that one of their cases was the first ever observed, two years earlier, in 1881. It is relevant, however, that it was in this same year that involvement of the foot by tabetic arthropathy was presented to the International Medical Congress in London by an English surgeon, Herbert William Page. We believe that Page was the first to diagnose and to report a case of tabetic neuropathic osteoarthropathy in which the bones of the foot and ankle were involved. He was also the first to propose a link between the tabetic foot and disease of the peripheral nerves, as opposed to the central nervous system.

What lessons can history teach us about the Charcot foot?

Regrettably, physicians today receive very little instruction in the history of medicine. Most health care providers have a very limited, contemporary knowledge of the condition that we know of as the Charcot foot. Yet, historical concepts of the pathogenesis and natural history of this condition provide us with important lessons that enhance our understanding, recognition, and management of this rare but debilitating neurogenic arthropathy. It is my belief that knowledge of the history of medicine provides us with a better understanding of present-day issues and clearer vision as we look to the future. This article describes some of the important lessons learned from the history of the Charcot foot.

Jean-Martin Charcot (1825-1893). The man behind the joint disease.

Jean-Martin Charcot was one of the most celebrated French physicians of the 19th century. A masterful teacher and a captivating lecturer, Charcot created the foundations of neurology as an independent discipline, and transformed the Salpêtrière hospital, in Paris, into one of the world's greatest teaching centers for clinical neurologic research. His name is attached to the distinct pathologic entity, Charcot's joint disease, that he so meticulously described. This article reviews the highlights of Charcot's career and his clinicoanatomic studies of patients with tabetic arthropathies.

The diabetic foot: basic mechanisms of disease.

There remains vast truth in the statement "neuropathy causes diabetic foot pathology." However, if it were really that straightforward and if our understanding were complete, it is doubtful that ulceration and neuroarthropathy would be the public health problems that they are today. Diabetes is an insidious disease, and almost every component of the spectrum of hyperglycemic complications is active in creating foot lesions. These include dramatic alterations in all components of the peripheral nerves, the mechanical characteristics of bones and soft tissues, gait kinematics, the vasculature at both a microscopic and a macroscopic level, the immune system, and the fundamental processes of wound healing. Clinical treatments that address the biologic aspects of the problem without considering the mechanics, or vice versa, can sometimes be effective but fail to take advantage of all of the potential means to succeed. The greatest potential for future clinical advance lies in understanding and simultaneously addressing the many synergistic factors that cause both ulceration and neuroarthropathy.

The enduring mark left by Jean-Martin Charcot on rheumatology.

Although Charcot is remembered above all as an outstanding neurologist, he also left a lasting imprint on the study of rheumatic diseases, primarily in two fields. a) He performed a pathologic-nosographic confrontation based on principles that remain relevant in today's era of imaging techniques. His vision as a pathologist allowed him to establish links between nonspecific lesions, which led him to develop a unified concept of chronic rheumatism. At the same time however, his experience as a clinician gave him a sense of the nosologic distinctions that are widely accepted today. b) He analyzed osteoarticular dystrophies associated with neurologic disorders, most notably tabetic arthropathies with epiphyseal fragmentation and in some instances spontaneous fractures. In addition, a constellation of alterations of the synovial membrane, ligaments, and muscles identified in those analyses foreshadowed today's concept of reflex sympathetic dystrophy syndrome.

A short history of neuropathic arthropathy.

Neuropathic joint disease or Charcot joints are a chronic form of a degenerative arthropathy that is associated with decreased sensory innervation of the involved joints. Neuropathic arthropathies are considered to be an accelerated osteoarthritis that is precipitated by trauma to a joint not protected by its proprioceptive or nociceptive reflexes. This process continues until destruction of the joint occurs. The etiology of Charcot joints has varied throughout history as different diseases have become less or more prominent. Some of the offenders include diabetic neuropathy, tabes dorsalis, syringomyelia, and congenital indifference to pain. The morphology and pathogenesis of this condition has intrigued the minds of many, including Musgrave, Charcot, Virchow, Eloesser, Soto-Hall, and Key.

Neuropathic bone and joint disease.

The pathogenesis of the neuropathic joint has been a subject of controversy for many years. Two main theories of pathophysiologic pathways have evolved: (1) the neurotraumatic, which states that the changes result from mechanical trauma and repetitive injuries to an insensitive extremity or joint and (2) the neurovascular, which states that the changes result from a neurally initiated vascular reflex that leads to hyperemia, angiogenesis, and very active bone resorption by osteoclasts. Through clinical, radiographic, and pathologic observation, it appears evident that both pathways contribute to neuropathic bone and joint disease. Initially, the alteration of sympathetic control triggers a persistent hyperemia, leading to active bone resorption. There may or may not be associated pathologic fractures and subsequent repair. This depends upon the degree of joint insensitivity and whether or not it is subjected to continued weightbearing. If so, the neurotraumatic mechanisms come into play, but only secondarily.

Hip replacement in a Charcot joint: a case report and historical review.

A 61-year-old woman with a neuropathic joint was treated by total hip arthroplasty. Histologically, the absence of any osseous repair process in this hip is consistent with reports that neuropathic fractures of the femoral neck generally fail to unite. The fragments of necrotic bone found in the synovium are characteristic of the pathologic process. The above-described patient is typical of the preataxic stage. The seven-year successful result of total hip arthroplasty is most likely explained by the fact that the patient was not ataxic. Because of the unpredictability of neurosyphilis, seven years should not be considered the touchstone of success. From this experience there is no recommendation or even a suggestion that hip arthroplasty should be performed on ataxic patients.

[Nervous arthropathies]. / Arthropathies nerveuses.

In 1868, J.M. Charcot published his memoir "sur quelques athropathies qui paraissent dépendre d'une lésion du cerveau ou de la moelle épinière "based on 4 cases of tabes dorsalis. This work was subsequently developed and presented in London in 1881 when Sir James Paget acknowledged the significance of this entity. "Charcot's joint" has come in use to mean that particular joint disease which results, from nervous lesions, whatever its nature. A controversy lasted between those who thought, e.g. Volkman, that mechanical trauma is the significant pathophysiological factor and those, e.g. Charcot, who ascribed a trophic role to the spinal cord. At the beginning, Charcot thought that lesions of the anterior horn were responsible but finally concluded that be could not bring a precise answer to that point. Subsequently Raymond, Dejerine (who insisted on the absence of pain), Foix and Alajouanine, Alajouanine and his collaborators extended Charcot's observations. Alajouanine proposed that lesions of the sympathetic nervous system played a major pathophysiological role. In 1960 Castaigne and Cambier proposed a unified theory with complementary roles ascribed to trauma and to nervous lesions. In 1936, diabetic neuropathy was described by W. Jordan. In 1942, Thevenard described "acropathie ulcéro-mutilante" now widely known as hereditary sensory neuropathy. Amyloidosis, familial dysautonomia, congenital insensitivity to pain were added to the list of causes of Charcot's joint. Less well known are the works of Charcot on joint diseases in hemiplegics. These were later studied by Alajouanine and Thurel and de Sèze and Ryckewaert have proposed to call them "algoneurodystrophies décalcifiantes réflexes" a topic which nowadays arises much interest in rheumatology.

[Osteoarticular diseases from J.M. Charcot to the present time]. / Les affections ostéo-articulaires de J.M. Charcot à nos jours.

In foreign countries Charcot's main contribution in the field is known as Charcot's joint. A special paper is devoted to this topic by Dr Hubault in this issue of the Revue. It is perhaps less widely known that Charcot's thesis was devoted to rheumatoid arthritis and that he wrote also interesting observations on gout. Pierre Marie gave many famous contributions to bones and joints diseases. In 1886 he described acromegaly. In 1890 he described hypertrophic pulmonary osteoarthropathy which he correctly linked with lung diseases although some of the cases he had collected in the literature were instances of pachydermo-periostosis. In 1900 he gave a brilliant description of achondroplasia in 2 of his patients: Anatole and Claudius. On the 11th of February 1898 he reported 2 patients which were the basis of a historical description of ankylosing spondylitis, on which papers by Strümpell and von Bechterew had appeared in 1884 and 1893. The subsequent works at the Clinique des Maladies du Système Nerveux de la Salpêtrière on sciatica and herniated disks are related in Professor de Seze's paper in this issue of the Revue.