Charcot disease

Introduction

Charcot's osteoarthropathy is a complication of diabetic neuropathy and some other neurological conditions. Scientific nomenclature should be accurate and specific. However, when describing Charcot's osteoarthropathy up until now, names have been chosen with lack of precision, extraordinary pedantry and frank inaccuracy. If there is confusion over the name of the condition, there is a consequent risk of confusion over diagnosis and treatment, which may be reflected in the alarming morbidity associated with this condition. Here, the author argues that if the term Charcot's osteoarthropathy could be generally adopted, it would put an end to decades of confusion.

KEY WORDS

* Charcot's osteoarthropathy

* Confused nomenclature

* Accurate classification

* Universal terminology

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There is no consensus on the correct name for Charcot's osteoarthropathy, which can lead to imprecise diagnosis and management. This can adversely affect outcomes in patients with diabetic foot problems.

Early descriptions of Charcot's osteoarthropathy

Charcot's osteoarthropathy is a complication of diabetic neuropathy and some other neurological conditions (Table 1).

William Musgrave, writing in Antiquitates Britanno-Belgicae (1748), described cases of neuropathic arthritis and emphasised the need to recognise that arthritis could be secondary to other diseases (Kelly, 1963). In 1831, JK Mitchell of Philadelphia wrote about 12 cases of joint afflictions (arthritis) in patients with lesions of the spinal cord (Mitchell, 1831).

The eminent 19th Century neurophysiologist Professor Jean-Martin Charcot (1868a; 1868b) described a progressive, destructive arthropathy in a group of his patients with neurosyphilis (tabes dorsalis) at La Salpetriere Hospital in Paris, but Charcot himself acknowledged that Mitchell's was the earlier report. However, in 1882, the Rapport du Congres, published in London, first named these distinct pathological changes as 'Charcot's joint' (MacCormac and Klockmann, 1881):

Charcot had a remarkable and admirable ability to see what was new and what was logically linked, and his name is associated with many other medical conditions (Table 2). In 1936, William Reilly Jordan made the first report of Charcot's osteoarthropathy in a patient with diabetes (Jordan, 1936).

Confused nomenclature and lack of consensus

Although over 200 papers and numerous book chapters have been devoted to Charcot's osteoarthropathy--and in spite of the different stages of the condition having been described in considerable detail by Harris and Brand (1966), Lennox (1974), Horibe et al (1988), Sanders and Frykberg (1991), Barjon (1993), Brodsky and Rouse (1993), Johnson (1995) and Dounis (1997)--there is no consensus as to what is the most appropriate or correct name (Table 3). In a review of Charcot's osteoarthropathy by Solomon Tesfaye's group (Rajbhandari et al, 2002), 39 names were used to describe Charcot's osteoarthropathy in the references cited (Table 4).

Furthermore, many authors have allocated different names to Charcot's osteoarthropathy in articles authored or co-authored by them over a period of time. There is no consensus and much confusion.

Discussion

For practical purposes, clinicians managing Charcot's osteoarthropathy need to know:

* the name of the disease

* the stage (acute, bony destruction, resolving or resolved)

* the site affected (in patients with diabetes this will usually be forefoot, midfoot, hindfoot, ankle or, rarely, the knee)

* the type and degree of residual deformity (rocker bottom, medial convexity) after the acute stage has resolved

* the presence of ulceration, infection or instability of a joint.

The name of the condition alone is not enough to guide treatment and predict outcomes.

However, Newman (1987) wrote of Charcot's osteoarthropathy:

There is no consensus on the most appropriate name for Charcot's osteoarthropathy. The International Consensus on the Diabetic Foot (International Working Group on the Diabetic Foot, 1999) suggested the rather unwieldy name of 'neuro-osteoarthropathy (Charcot-foot)', but this name has not been adopted.

It is always desirable to use language to order concepts in a way that is logically sound, elegant and well suited for potential users of the description. Some names are steeped in history: 'diabetes mellitus', the 'honey siphon' of the ancient Greeks, for instance, describes some of the symptoms of untreated diabetes very well, so there is a good reason for preserving it.

Scientific nomenclature should be accurate and specific. Up until now, however, when describing Charcot's osteoarthropathy, names have been chosen with a lack of precision, extraordinary pedantry and frank inaccuracy. In some cases, the desire to cover all possible eventualities has resulted in a 'scattergun' approach, with every possible descriptive term appended together with afterthoughts in brackets.

Few would deny that Professor Jean-Martin Charcot was both deserving of honour and possessed of a beguiling name, as reflected by the fact that there are at least 14 medical eponyms associated with this great physician.

If the first describer of a disease is the one to be honoured, the disease should be called 'Musgrave's' or 'Mitchell's' osteoarthropathy. If we want a specific name for Charcot's osteoarthropathy in diabetes, the disease should be 'Jordan's' osteoarthropathy. However, once a name has become firmly associated with a condition it is hard to change it, so it seems that potential claimants such as Musgrave, Mitchell and Jordan had better bow down to Charcot.

Regarding precise semantics, Charcot's osteoarthropathy is not just an arthropathy; it can also affect bone alone, so it is properly described as an osteoarthropathy. In the early stages it may be hard to tell whether an initial osteopathy will progress to an arthropathy. Some radiologists, therefore, describe it as an osteopathy. However, since it is usually more than just osteopathy, affecting joints as well as bones, arthropathy should be included in the name.

Although Charcot described many other diseases and syndromes (Table 2), he did not describe any other bone and joint condition, so there is no possibility of confusion when we refer to Charcot's osteoarthropathy. However, we should not just call Charcot's osteoarthropathy 'the Charcot foot'.

Conclusion

Imprecise description is unacceptable in diagnosis or treatment, and it should not be acceptable in the nomenclature of Charcot's osteoarthropathy. Many clinicians may be reading or writing about Charcot's osteoarthropathy in a language that is not their own first language. If there is confusion over the name of the condition, there is a consequent risk of confusion over diagnosis and treatment, which may be reflected in the alarming morbidity associated with this condition (Jeffcoate, 2005).

The author has no doubt that Charcot's osteoarthropathy is the most appropriate name, and justly honours a great neurophysiologist and acute observer. If this nomenclature could be generally adopted, it would put an end to decades of confusion.

Barjon MC (1993) Les osteoarthropathies destructices due pied diabetique. In: Herisson C, Simon L (eds) Le Pied Diabetique. Masson, Paris: 77-91

Brodsky JW, Rouse AM (1993) Exostectomy for symptomatic bony prominences in diabetic Charcot foot. Clinical Orthopaedics 296: 21-6

Charcot JM (1868a) Sur quelques arthropathies qui paraissent dependre d'une lesion du cerveau ou de la moelle epiniere. Archives of Physiology and Normal Pathology 1: 161-78

Charcot JM (1868b) On some arthropathies apparently related to a lesion of the brain or spinal cord. Translated and edited by Hoche G, Sanders LJ (1992). Journal of the History of Neuroscience 1: 75-87

Dounis E (1997) Charcot neuropathic osteoarthropathy of the foot. Acta Orthopaedica Hellenica 48: 281-95

Harris JR, Brand PW (1966) Patterns of disintegration of the tarsus in the anesthetic foot. Journal of Bone and Joint Surgery 5: 95-7

Horibe S, Tada K, Magano J (1988) Neuroarthropathy of the foot in leprosy. Journal of Bone and Joint Surgery 70B(3): 481-85

International Working Group on the Diabetic Foot (1999) International Consensus on the Diabetic Foot. International Diabetes Federation, Brussels

Jeffcoate W (2005) Charcot foot--why still so difficult to treat? Diabetic Foot Conference, Edinburgh

Johnson JE (1995) Neuropathic (Charcot) arthropathy of the foot and ankle. American Academy of Orthopedic Surgeons Instructional course. No. 349

Jordan WR (1936) Neuritic manifestations in diabetes mellitus. Archives of Internal Medicine 57: 307-66

Kelly M (1963) De arthritide symptomatica of William Musgrave (1657-1721): his description of neuropathic arthritis. Bulletin of the History of Medicine 37: 372-7

Lennox WM (1974) Surgical treatment of chronic deformities of the anaesthetic foot. In: F McDowell, CP Enna, eds. Surgical rehabilitation in leprosy, and in peripheral nerve disorders. Williams and Wilkins, Baltimore, MD, 350-372

MacCormac W, Klockmann JW (1881) Transactions of the international medical congress. Ballantyne, Hanson & Co., London

Mitchell JK (1831) On a new practice in acute and chronic rheumatism. American Journal of Medical Science 8: 55

Newman JH (1987) Non-infective disease of the diabetic foot. In: AJM Boulton, H Connor, JD Ward, eds. The Foot in Diabetes. John Wiley & Sons, Chichester

Rajbhandari SM, Jenkins RC, Davies C, Tesfaye S (2002) Charcot neuroarthropathy in diabetes mellitus. Diabetologia 45: 1085-96

Sanders LJ, Frykberg RG (1991) Diabetic neuropathic osteoarthropathy: the Charcot foot. In: Frykberg RG (ed) The High Risk Foot in Diabetes Mellitus. Churchill Livingstone, New York, 297-338

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RELATED ARTICLE: ARTICLE POINTS

1 There is no consensus on the most appropriate name for Charcot's osteoarthropathy.

2 Charcot's osteoarthropathy is a complication of diabetic neuropathy and some other neurological conditions.

3 Imprecise description is unacceptable in diagnosis or treatment, and it should not be acceptable in the nomenclature of the Charcot foot.

4 If the term 'Charcot's osteoarthropathy' could be generally adopted, it would put an end to decades of confusion.

RELATED ARTICLE: PAGE POINT

1 Charcot had a remarkable and admirable ability to see what was new and what was logically linked, and his name is associated with many other medical conditions.

RELATED ARTICLE: PAGE POINTS

1 Although over 200 papers and numerous book chapters have been devoted to Charcot's osteoarthropathy, there is no consensus on what is the most appropriate or correct name.

2 Many authors have allocated different names to Charcot's osteoarthropathy in articles authored or co-authored by them over a period of time.

3 Clinicians managing Charcot's osteoarthropathy need to know the name of the disease, the stage, the site affected, the type and degree of residual deformity after the acute stage has resolved, and the presence of ulceration, infection or instability of a joint.

RELATED ARTICLE: PAGE POINTS

1 Charcot's osteoarthropathy is not just an arthropathy; it can also affect bone alone, so it is properly described as an osteoarthropathy.

2 Although Charcot described many other diseases and syndromes, he did not describe any other bone and joint condition, so there is no possibility of confusion when we refer to Charcot's osteoarthropathy.

Ali Foster is Honorary Consultant Podiatrist at the Diabetic foot Clinic, King's College Hospital, London.

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