Reviews
Polyarticular Charcot: A rare case report and a literature review in Indian context
Jeet Hemant Kumar Patel, Ved Chaturvedi, Gurbir Singh Bhandari, Mayank Gupta1
Author Affiliations
1Sir Ganga Ram Hospital, New Delhi, India
Correspondence: Dr. Jeet Hemantkumar Patel
IJRCI: 2020;(8)1:R1
Submitted: 27 December 2019, Accepted: 6 February 2020, Published: 16 March 2020
© IJRCI
Abstract
Charcot or neuropathic arthropathies are a progressive form of destructive, erosive and generally painless arthropathies. Prevalence of neuropathic joints has decreased globally with reduction in the cases of leprosy and syphilis. However, syringomyelia and DM have emerged as the major causes for upper limb and lower limb Charcot joints respectively. Literature evidence shows lack of Indian data pertaining to these arthropathies. The present study describes a case of polyarticular Charcot in a patient with syrinx and Chiari malformation. The patient history revealed a provisional diagnosis of rheumatoid arthritis and Koch’s elbow, and was treated with anti-tubercular treatment (ATT) and disease modifying anti-rheumatic drugs (DMARDS). Nervous system examination would have easily led to the diagnosis of syringomyelia. The present study also provides a review of Indian literature on neuropathic joints from 2001 to 2019. Diabetes mellitus (DM), syringomyelia, leprosy and syphilis are major etiologies for Charcot joints.
Keywords: Charcot, arthropathy, polyarticular, Syrinx, syringomyelia
Introduction
Neuropathic arthropathy is a destructive form of progressive articular disease. It is also called neuropathic osteoarthritis or Charcot arthropathy. Jean-Martin Charcot was the first to describe arthropathies associated with tabs dorsalis in 1868.1 Neuropathic joints have become far less common in India with the availability of more efficient treatment for DM, syphilis and leprosy.2, 3 Moreover, recent years have witnessed a change in the prevalence of etiologies of arthropathies. In recent years, syringomyelia and DM have emerged as the major etiologies for neuropathic joints in upper and lower limbs respectively. Literature evidence from India on neuropathic joints is scarce. The present study discusses a rare case of polyarticular Charcot, which was provisionally diagnosed as rheumatoid arthritis, and the patient had undergone treatment with DMARDs and anti-tubercular therapy. The study also reviews the available Indian publications on the same.
Case report
A 48-year-old female presented with insidious onset heaviness in left arm/forearm and pain in multiple joints for 2 years. She gradually developed progressive numbness and pinprick sensations over left shoulder area, left elbow area, which gradually progressed to left forearm and left hand. She also had numbness over right hypochondrium and lower part of right breast. She also complained of multiple joints pain (both the shoulder joints, left elbow, left wrist, and left small joints of hand). She had mechanical pain in both the shoulders, knee and ankle joints. She noticed swelling in left elbow, left wrist and left small joints of hand (2nd and 3rd MCP joints and 1st CMC joints).
The patient history revealed that she had been operated earlier for left elbow swelling (synovectomy + ulnar neurolysis) and was on ATT for 4 months. The patient was treated with DMARDs (methotrexate, hydroxychloroquine), corticosteroids and non-steroidal anti-inflammatory drugs (NSAIDS). The history also revealed a wrong provisional diagnosis of rheumatoid arthritis (RA), Koch’s elbow, and ulnar neuropathy.
Musculoskeletal examination revealed tenderness at bilateral shoulder joints with decreased range of motion (ROM) for overhead abduction. Swollen left elbow with decreased ROM, piano key movement at left wrist, swelling and radial deviation of 1st CMC joint left side, swollen 1st and 2nd left MCPs, and bilateral knee crepitus were other musculoskeletal findings (Fig. 1). Nervous system examination revealed hypotonia in both the upper limbs with atrophied thenar and hypothenar muscles. There was a mild reduction in the hand grip (left more than right) and limited touch and temperature sensation over left upper limb. Sensory level was present up to T8. Biceps, triceps and supinator jerks were absent on left side. Lower limbs reflexes were normal. Ulnar nerves were palpable and thickened bilaterally.
Fig. 1: (A) Left swollen elbow with a surgical mark of synovectomy, (B) Left hand showing radial deviation of 1st CMC joint
Hemogram and biochemical blood investigations were normal, and rheumatoid factor (RF) was absent. Nerve conduction velocity testing of both upper limbs revealed distal mildly asymmetrical, large fiber predominantly motor, demyelinating axonal polyneuropathy/ polyneuoronopathy in a bilateral C5 to T1 distribution (left more than right). A moderate degree of carpal tunnel syndrome on the left side was present. X-ray of left elbow showed decreased joint space, erosions and destructive articular changes, and that of left hand showed decrease joint space, sclerosis, osteophytes and degenerative changes at distal radioulnar, radiocarpal, 1st CMC, and MCP joints. X-ray of knee was suggestive of changes of osteoarthritis on both the sides (Fig. 2). MRI of left elbow revealed altered marrow signal intensities in humerus, radius, ulna, and attached soft tissue and significant elbow joint effusion (Fig. 3). MRI of cervical spine revealed syrinx involving cervical cord, extending to thoracic spine with longitudinally extended altered signal intensity changes with septae. There was also cerebellar tonsillar herniation up to C2 level. The MRI was suggestive of Chiari malformation with syrinx (Fig. 4). The patient was referred to undergo a corrective surgery for Chiari malformation.
Fig. 2: (A) X-ray of knees showing changes of osteoarthritis (B) X-ray of left hand showing irregular joint margin, erosions and sclerosis at 1st CMC and radio-carpal joints, (C) X-ray of left elbow showing destroyed joint
Fig. 3: Altered marrow signal intensities in humerus, radius, ulna, attached soft tissue and significantelbow joint effusion
Fig. 4: (A) T2 image, (B) T1 image S/O Chiari malformation with syringomyelia of cervical-dorsal spine cord
Table 1: Salient points that can be deduced from the case
Discussion
Neuropathic arthropathy is a minimally painful, destructive and progressive, arthropathy caused by a neurologic deficit. Common etiologies of neurologic deficit that can cause Charcot arthropathy are DM, syringomyelia, leprosy, and tabes dorsalis. Although William Musgrave, a British physician identified it for the first time in 1703, Jean-Martin Charcot, a French neurologist, first described the underlying pathology of this condition.1
The diagnosis of neuropathic joint is uncommon in day-to-day clinical practice, except in diabetes Charcot arthropathy. Although the data on neuropathic joints from India is scarce, clinical experience shows that there is a significant decrease in the prevalence of neuropathic joint associated with syphilis and leprosy. The present study has carried out a literature review in PubMed and non-PubMed databases since 2001 using the keywords Charcot arthropathy, neuropathic arthropathy, syringomyelia, and India. Most of the publications on neuropathic joints from India were on DM and syringomyelia. The search identified 19 publications on syringomyelia leading to neuropathic joints and 7 articles, including certain reviews, on DM leading to Charcot joints (Table 2).
Table 2: Literature on neuropathic arthropathy from India since 2001
Several reviews on neuropathic joints have reported clinical and radiological characteristics of neuropathic joints and its pathogenesis.16, 19, 32 The present study has tried to include all the published Indian literature on neuropathic joints. The study could not find any Indian review on syringomyelia-associated Charcot joints. One review was identified upon conducting a search for syringomyelia and neuropathic joints from countries other than India. Wang et al. reviewed 34 cases of syringomyelia with Charcot joints. The mean age noted was 45.21 years (ranging from 25 to 80 years) with increased female preponderance.35 Causes of syrinx formation identified are Chiari malformation, spinal cord injury (trauma or post-spinal anesthesia), infective vertebral damage, and idiopathic syrinx. Meningitis, arachnoiditis, tethered spinal cord syndrome, spina bifida occulta and spinal cord tumors are other causes responsible for syrinx formation, albeit the current study could not find these etiologies for Indian syringomyelia cases. The management should be aimed at treating the primary etiology for syrinx, and neuropathic joint. Although there are studies reporting the usefulness of bisphosphonates like zoledronic acid and alendronate in diabetic Charcot foot, their use for managing syringomyelia-related Charcot joints have not yet evaluated.20
Posterior cranial fossa decompression has been identified as one of the treatment options for syrinx with Chiari malformation, no treatment is available for idiopathic syrinx. Ramnarayan et al. showed the beneficial effects of posterior fossa decompression with duraplasty in most of the patients with Chiari type 1 malformations.36 Surgery is also useful for treating tethered cord syndrome, spinal tumors and post-spinal cord injury syrinx.
Syringomyelia was present in up to 25% of patients with Chiari malformation, particularly those with type 1 and Charcot arthropathy in 25% of syringomyelia cases.37,38 Agarwal et al. found 63 cases of Chiari malformations. Increased male preponderance was noted and >50% of the subjects were younger patients (<20 years of age). Out of 63 cases, 46 cases were of type I and 14 cases were of type II Chiari malformations. Syringomyelia was present in 30 cases (47.62%) and was mostly associated with type I Chiari malformations.39
Prevalence of Charcot arthropathy in DM was 0.1 to 0.5%, and it involved tarsal, tarsometatarsal, metatarsophalangeal joints.40 A direct association was noted between the prevalence and duration of disease. Shalini et al. reported that the prevalence rate of Charcot arthropathy in >50-year older diabetic patients with severe peripheral neuropathy was 9.8%. The mean age was 63 ± 8.36 years, and mean duration to develop neuropathic joints in DM was 18.01 ± 8.23 years.7 Many surgical techniques and medical management were evaluated for usefulness in DM Charcot joint patients in India.9, 16, 20 One Indian study by Durgia et al. reviewed bisphosphonates, calcitonin, and denosumab in management of acute Charcot arthropathies.41 A systemic review by Richard et al. showed the effectiveness of bisphosphates in the management of acute Charcot joints, but the evidence was not strong due to insufficient data.42 However, there is no study on the use of bisphosphonates for treating Charcot joint associated with non-diabetic causes like syringomyelia, leprosy etc.
Leprosy-associated Charcot joints are rare to find in daily clinical practice. Wakhlu et al. described 29 cases of rheumatological manifestations of leprosy and found only one patient with Charcot arthropathy. The current study could not find any other literature on leprosy-associated Charcot joints from India, but could identify one case of syphilis-associated disease.30
Conclusion
Neuropathic arthropathy is a rare cause of erosive joint disease. It may mimic Koch’s joint involvement, psoriatic arthritis, Hansen’s disease, rheumatoid arthritis and other forms of erosive arthritis, particularly in upper limb. Syringomyelia with or without Chiari malformations may present as upper limb asymmetric neuropathic arthropathy. Nervous system examination may assist in diagnosis, thereby to avoid unnecessary investigations, procedures and treatment. More data on the usefulness of medical agents like bisphosphonates, denosumab and calcitonin in syringomyelia Charcot arthropathy from Indian settings are needed.
Competing interests
The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
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