Polyarticular Charcot: A rare case report and a literature review in Indian context

Introductıon 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.

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.
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, 1 st 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.

Discussion
Neuropathic arthropathy is a minimally painful, destructive and progressive, arthropathy caused by a neurologic deficit. The diagnosis of neuropathic joint is uncommon in day-today clinical practice, except in diabetes Charcot arthropathy.
Although the data on neuropathic joints from India is  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 1 Conducting neurological examination is important for upper limb asymmetric arthritis.
2 Mono or oligo-articular presentation is a common in syringomyelia. But this case shows that polyarticular involvement may also occur.

3
MRI of spine is helpful (not the affected joint) in proven case of syrinx.

4
Ulnar nerve may be thickened in syringomyelia, with or without neuropathic elbow joint, may mimic leprosy. But in leprosy, lower limb joints are usually affected, and not the upper limbs joints. Ulnar neurolysis is helpful to relieve symptoms due to nerve compression.

5
Syringomyelia associated neuropathic joints may mimic rheumatoid arthritis, psoriatic arthritis, tubercular arthritis, and articular manifestations of leprosy.

No. Publication
Year of publication Joints affected Etiology

Patient declaration statement
The authors certify that the patient had given her consent for images and other clinical information to be reported in the journal. The patient understood that her names and initials will not be published and due efforts will be made to conceal her identity.

Citation
Patel JH. Polyarticular charcot: a rare case report and a literature review in Indian context. IJIR. 2020; (8)