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Neurogenic Arthropathy

Charcot%27s Joint

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].

Gaucher Disease
  • New cases include discal cyst, polymyalgia rheumatica, Gaucher disease, pigmented villonodular synovitis, ventriculus terminalis cyst, and much more.[books.google.com]
Anhidrosis
  • Both patients have significant axial and appendicular bone abnormalities, Charcot-type arthopathy, heat intolerance and also anhidrosis. They have normal intelligence.[ncbi.nlm.nih.gov]
  • Anhidrosis (inability to sweat) is one of those problems. These children can’t tolerate high temperatures. And this heat intolerance is made worse by the fact that they live in a hot, dry desert climate.[eorthopod.com]
  • Online Mendelian Inheritance in Man (OMIM) Insensitivity to Pain, Congenital, with Anhidrosis; CIPA -256800 a b Brodsky MC (2016). Pediatric Neuro-Ophthalmology. Springer. p. 741. ISBN 978-1493933846. Retrieved April 13, 2017.[en.wikipedia.org]
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]
  • Differential diagnosis includes tumor and septic arthritis. We report a case of neurogenic hip arthropathy in a 30-year-old diabetic woman, and illustrate the clinical history and imaging findings.[link.springer.com]
  • Track disease progression and treat patients more effectively with the information on genetic findings, imaging outcomes, cell and biologic therapies, rheumatoid arthritis, and SLE.[books.google.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]
Joint Effusion
  • Hemorrhagic joint effusions and multiple small fractures can occur, accelerating disease progression.[merckmanuals.com]
  • Imaging Radiography indication for all patients findings can be normal in early Charcot joint acute bony consolidation with fractures, joint effusion, or bone destruction chronic bony deformity, new bone formation, and sclerosis resorption of bone Magnetic[medbullets.com]
  • Progressive joint effusion, fracture, fragmentation, and subluxation should raise the suspicion of neuroarthropathy.[emedicine.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]
Joint Swelling
  • ) 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]
  • ) Swelling After an injury, the synovial fluid that leaks out of the joint capsule may produce swelling.[drfoot.co.uk]
  • […] a neutrally medicated persistent hyperaemia. 1,7 A further theory proposes that joint changes result from damage to central nervous system ‘trophic centres’ which control bone and joint nutrition. 1 The classical clinical presentation of NA involves joint[nzma.org.nz]

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

  • 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]
  • To date, there are no reports in the orthopaedic literature regarding this disorder, particularly the clinical manifestations and treatment considerations.[ncbi.nlm.nih.gov]
  • 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]
  • Surgical indications for the various joints affected are discussed, and the results obtained after treatment are reported.[ncbi.nlm.nih.gov]
  • Organized around disease clusters, this comprehensive, clear and concise guide to disease information, ranging from causes, signs and symptoms, and diagnosis through treatment and special considerations, has been updated with the latest in original research[books.google.com]

Prognosis

  • Anatomic classification systems are the most commonly used and have the added benefit of predicting outcome and prognosis.[emedicine.com]

Etiology

  • PMID: 1297567 [Indexed for MEDLINE] MeSH terms MeSH terms Adult Arthrography Arthropathy, Neurogenic/diagnostic imaging* Arthropathy, Neurogenic/etiology Arthropathy, Neurogenic/surgery Humans Joints/surgery Middle Aged[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]
  • 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]
  • Location Sensorimotor and autonomic neuropathies of various etiologies are the primary predisposing factor.[radiopaedia.org]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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.com]

Prevention

  • 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]
  • This 10th edition features improved sections focused on health promotion and disease prevention— topics that have been receiving added emphasis in health care circles in recent years – as well as updates on many conditions for which a variety of clinical[books.google.com]
  • Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets[books.google.com]
  • Part of the encyclopedia is a disease dictionary including not only recommendation how to prevent each disease but also its initial symptoms, causes and useful advices about treatment for given illness.[beltina.org]

References

Article

  1. 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.
  2. 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.
  3. 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.
  4. Callaghan BC, Cheng H, Stables CL, Smith AL, Feldman EL. Diabetic neuropathy: Clinical manifestations and current treatments. Lancet Neurol. 2012;11(6):521-534.
  5. Botek G, Anderson MA, Taylor R.. Charcot neuroarthropathy: an often overlooked complication of diabetes. Cleve Clin J Med. 2010;77(9):593–599.
  6. Cullen AB, Ofluoglu O, Donthineni R. Neuropathic Arthropathy of the Shoulder (Charcot Shoulder). MedGenMed. 2005;7(1):29.
  7. 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.

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Last updated: 2019-07-11 20:50