Neurogenic arthropathy, also known as Charcot joints, is a rare but severely debilitating disorder of the articular system. Diabetic neuropathy, syringomyelia, trauma, neurosyphilis, and many other diseases can induce this phenomenon. Severe neuropathy and vascular insufficiency lead to a rapidly progressive destruction of the joints with symptoms of swelling and erythema and only mild pain. The diagnosis rests on a detailed patient history, a thorough physical examination, and appropriate imaging studies that determine the underlying cause.
Presentation
Neurogenic arthropathy, initially named as Charcot neuropathic osteoarthropathy, affects the joints and the skeletal system and possesses a chronic and progressive clinical course [1] [2] [3] [4]. The pathogenesis stems from a diminished function of the sensory nerves arising due to significant peripheral neuropathy, which may be encountered in various conditions [2] [5] [6] [7]. By far, the most common etiology is diabetic neuropathy, affecting up to 13% of cases suffering from severe forms of this ailment [5]. Other notable causative factors are syringomyelia, neurosyphilis, trauma to the spinal or peripheral nerves, and several other conditions affecting the nervous system [1] [6] [7]. Virtually any joint can be affected by neurogenic arthropathy. Some studies have established that neurosyphilis present in the knee, whereas the foot and ankle are mainly targeted in diabetic patients [6] [7]. The clinical presentation is distinguished by a somewhat rapid onset of erythema and swelling of the joint with minimal or mild accompanying pain, which is one of the reasons why patients present very late to the physician [5]. A limited range of motion and stiffness is frequently observed, particularly in the shoulder joint [6]. In addition to localized joint symptoms, autonomic dysfunction may be simultaneously present with complaints of constipation, urinary retention, erectile dysfunction, and arrhythmias [2] [4].
Entire Body System
- Collapse
Sudden Trauma A sudden injury such as dropping heavy object on the foot can cause sudden collapse of its arch resulting in the formation of this condition. [primehealthchannel.com]
[…] dx.doi.org/10.1594/essr2015/P-0105 Optimal current management of the acute diabetic Charcot foot requires accurate and timely diagnosis to allow casting for protection of the foot, prior to the development of radiographically identifiable fractures or collapse [posterng.netkey.at]
The condition is named after Jean Marie Charcot, who described the collapse of the bones of the foot in patients who had lost feeling in the feet due to advanced syphilis. [vygizejydybuq.tk]
Symptoms non-painful swelling of a joint Physical exam acutely, may present as non-tender, erythematous, edematous, and warm joint chronically, may present as joint or foot deformity with joint effusion and bony prominences most common deformity is a collapse [medbullets.com]
Two pathologic theories: Neurotraumatic: Repeated minor trauma of pedal bones in patient unable to perceive pain leads to multiple fractures and collapse of normal architecture ( 2 ). [unboundmedicine.com]
- Swollen Knee
It is seen in up to 9% of patients with diabetic neuropathy. [1] Similar pathology can also occur in the knee but is much less common and should be considered in a patient with peripheral neuropathy, who presents with a red, hot, tender, and swollen knee [emedicine.com]
- Ankle Swelling
Swelling There is inflammation in the area of the Charcot foot and ankle. Swelling becomes apparent where the disease makes itself visible. Pain Sufferers usually complain of soreness or pain in the affected foot area. [primehealthchannel.com]
Musculoskeletal
- Arthritis
People rarely develop a second arthritis that is caused by bacteria (see Infectious Arthritis ) and may or may not have the fever or general feeling of illness (malaise) that typically occurs with infectious arthritis. [merckmanuals.com]
People rarely develop a second arthritis that is caused by bacteria (see Infectious Arthritis) and may or may not have the fever or general feeling of illness (malaise) that typically occurs with infectious arthritis. [msdmanuals.com]
(Dorland, 27th ed) Applies To Charcot's arthropathy associated with diseases classifiable elsewhere Neuropathic arthritis associated with diseases classifiable elsewhere Code First Code first underlying disease, as: neuropathic joint disease [Charcot's [icd9data.com]
[…] physical therapy management of psoriatic arthritis can be found on the page Psoriatic Arthritis. [physio-pedia.com]
- Joint Dislocation
Stage 1 : Osseous fragmentation with joint dislocation seen on radiograph ("acute Charcot"). [uwmsk.org]
Stage 1: Osseous fragmentation with joint dislocation seen on radiograph ("acute Charcot"). [en.wikipedia.org]
Ligamentous laxity, muscular hypotonia, and rapid destruction of joint cartilage are common, predisposing to joint dislocations, which also accelerate disease progression. [merckmanuals.com]
- Joint Deformity
[…] variants: also Charcot's joint Medical Definition of Charcot joint : a progressive, degenerative condition that affects one or more joints especially of the foot or ankle, is marked by bone fragmentation, swelling, redness, pain, and joint deformity, [merriam-webster.com]
Radiographs revealed a large joint effusion, calcification within the joint capsule, loose bodies, increased density within the distal humerus, proximal radius and ulnar, joint deformity and destruction (Figure 1 and Figure 2). [nzma.org.nz]
Without that protective response, the child can develop joint deformities and problems with limb alignment (especially in the legs). Arthropathy is another term to mean joint disease. [eorthopod.com]
Jean-Martin Charcot was a French neurologist (of course) who in 1868 recognized a link between neurosyphilis (tabes dorsalis) and a particular kind of deformed joint. [uwmsk.org]
Joint deformity, subluxation and dislocation of the metatarsals lead to a rocker-bottom type deformity in which the cuboid becomes a weight-bearing structure. [radiologyassistant.nl]
- Joint Effusion
Physical exam acutely, may present as non-tender, erythematous, edematous, and warm joint chronically, may present as joint or foot deformity with joint effusion and bony prominences most common deformity is a collapse of the tarsometatarsal joint, with [medbullets.com]
Joint effusion, calcification within the joint capsule, loose bodies and increased density within the distal humerus, proximal radius and ulnar are evident. [nzma.org.nz]
Progressive joint effusion, fracture, fragmentation, and subluxation should raise the suspicion of neuroarthropathy. [emedicine.com]
Hemorrhagic joint effusions and multiple small fractures can occur, accelerating disease progression. [merckmanuals.com]
- Joint Swelling
[…] that form a joint) Swelling Diagnostic tests X-rays confirm the diagnosis and allow evaluation of damage. [medical-clinic.org]
Common manifestations include joint swelling, effusion, deformity, and instability. Pain may be disproportionately mild due to the underlying neuropathy. Diagnosis requires x-ray confirmation. [merckmanuals.com]
[…] of the joint Subluxation (misalignment of the bones that form a joint) Swelling After an injury, the synovial fluid that leaks out of the joint capsule may produce swelling. [drfoot.co.uk]
Workup
The diagnosis of neurogenic arthropathy rests on the ability of the clinician to raise suspicion toward this disease. In order to gain sufficient evidence, a detailed physical examination and a thorough history are of critical importance. The presence of illnesses that could predispose to this condition, such as diabetes mellitus, must be excluded, while recent trauma is also an important risk factor. Once the course of symptoms, as well as their duration and progression, are assessed, the physical exam should cover a complete neurological workup, including reflex testing and evaluation of the sensory nerves [2]. Neuropathy of any origin can be confirmed by using either the Semmes-Weinstein 5.07/10 g monofilament or simple pinprick testing, whereas the neurometer test might also be employed when possible [2] [3]. A lower temperature of the affected joint and extremity is readily noted [2], thus further solidifying the diagnosis of neuropathic origin [2]. To confirm the neurogenic arthropathy, imaging studies need to be performed. Plain radiography, although a very useful method for observing the skeletal system, is of limited benefit due to frequent involvement of soft tissues by local inflammation, which is why magnetic resonance imaging (MRI) is the gold standard [2] [5] [6] [7]. Scintigraphy, use of Doppler probes, electrophysiology studies, and a skin biopsy are also recommended in the workup of individuals in whom neurogenic arthropathy is suspected [2].
Treatment
Treatment 1.7.5 If the multidisciplinary foot care service suspects acute Charcot arthropathy, offer treatment with a non‑removable offloading device. [nice.org.uk]
Diagnosis and Treatment The diagnostic path begins with recognition of the underlying neuropathy. X-RAY can usually confirm the damage to the joint. [beltina.org]
Treatment Pain relief - the immediate treatment goal- may be achieved with analgesics, nonsteroidal antinflammatory drugs, and joint immobilization (crutches, splints, braces, and weight-bearing restrictions). [medical-clinic.org]
There is little strong evidence and consensus concerning the timing of treatment and use of different fixation methods [ 6 ]. Complications Fractures can occur without pain and the absence of treatment leads to deformity. [patient.info]
Treatment of acute Lisfranc fracture and dislocation in patients with neuropathy. Tech Foot Ankle Surg. 2010. 9 (3):115-22. Schlossbauer T, Mioc T, Sommerey S, Kessler SB, Reiser MF, Pfeifer KJ. [emedicine.medscape.com]
Prognosis
A good prognosis therefore depends upon early recognition of the problem and effective management, which includes patient education [ 9 ]. [patient.info]
Anatomic classification systems are the most commonly used and have the added benefit of predicting outcome and prognosis. [emedicine.medscape.com]
Etiology
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. [ncbi.nlm.nih.gov]
By far, the most common etiology is diabetic neuropathy, affecting up to 13% of cases suffering from severe forms of this ailment. [symptoma.com]
Explore these free sample topics: -- The first section of this topic is shown below -- Etiology Diabetic neuropathy Syringomyelia Spinal cord injury Pernicious anemia Tabes dorsalis (neurosyphilis) Hansen's disease (leprosy) Peripheral neuropathy due [unboundmedicine.com]
Epidemiology
Methods: This is an observational, epidemiological study with a retrospective, descriptive, exploratory cohort, following a quantitative approach. [teses.usp.br]
Introduction Clinical definition arthropathy due to underlying neuropathy or trauma, leading to a destruction of joints and bone Epidemiology demographics adults location foot and ankle (most common) shoulder elbows risk factors diabetic neuropathy for [medbullets.com]
Epidemiology Usually occurs 8 to 12 years after diagnosis of DM Seen mostly in the 5th and 6th decades but can be seen in patients as young as 20 years Men > females 30% bilateral incidence 77% prevalence in patients with DM Prevalence without DM 11.7% [unboundmedicine.com]
Epidemiologic studies do not distinguish between acute and postacute disease. Bilateral disease occurs in less than 10% of patients. Recurrence of disease occurs in less than 5% of patients. [emedicine.medscape.com]
Pathophysiology
Prevalence 0.1% in all patients 0.8–8% in with the general DM population Up to 13% in high-risk diabetic patients ( 3 ) Etiology and Pathophysiology Exact cause is unknown. [unboundmedicine.com]
Charcot, observing the posterior column demyelination of tabes dorsalis, suggested the loss of a trophic function protecting joints as a pathophysiologic mechanism. [clinicalgate.com]
Pathophysiology The exact nature of Charcot arthropathy remains unknown, [5] but two major theories exist regarding the pathophysiology of this condition: neurotraumatic and neurovascular. [emedicine.medscape.com]
Prevention
Special shoes and/or braces may be necessary to support the foot to prevent further injury and ulceration of the foot. Sometimes surgery is the only way to make bracing and the use of footwear possible and help prevent amputation. [amputee-coalition.org]
Prevention In Western societies the most important form of prevention would be the prevention of type 2 diabetes and the control of existing diabetes. Diabetes clinics facilitate early detection of problems. [patient.info]
Prevention Avoiding injuries and using protective wear, such as splints or special boots Sometimes neurogenic arthropathy can be prevented by taking care of the feet and by avoiding injuries. [merckmanuals.com]
Prevention Prevention of arthropathy may be possible in a patient at risk. [medical-clinic.org]
Injury prevention in high-risk PLDN - to prevent the diabetic foot- is certainly much more efficient. Preventing the first or recurrent ulcers[ 86 ] should be no question. [wjgnet.com]
References
- Kopec K, Kusz D, Cielinski L, Wojciechowski P, Hajduk G. Bilateral neurogenic hip arthropathy. A case report. Neuro Endocrinol Lett. 2009;30(6):709-714.
- Kucera T, Shaikh HH, Sponer P. Charcot Neuropathic Arthropathy of the Foot: A Literature Review and Single-Center Experience. J Diabetes Res. 2016;2016:3207043.
- Nather A, Keng Lin W, Aziz Z, HJ Ong C, MC Feng B, B Lin C. Assessment of sensory neuropathy in patients with diabetic foot problems. Diabet Foot Ankle. 2011;2:10.3402/dfa.v2i0.6367.
- Callaghan BC, Cheng H, Stables CL, Smith AL, Feldman EL. Diabetic neuropathy: Clinical manifestations and current treatments. Lancet Neurol. 2012;11(6):521-534.
- Botek G, Anderson MA, Taylor R.. Charcot neuroarthropathy: an often overlooked complication of diabetes. Cleve Clin J Med. 2010;77(9):593–599.
- Cullen AB, Ofluoglu O, Donthineni R. Neuropathic Arthropathy of the Shoulder (Charcot Shoulder). MedGenMed. 2005;7(1):29.
- Chakraborty PP, Datta S, Ray S, Bhattacharjee R, Chowdhury S. Unilateral neuropathic arthropathy of the shoulder secondary to syringomyelia: Diagnostic challenges. World J Clin Cases. 2015;3(12):1017-1020.