Neuropathic Arthropathy of the Shoulder

Abstract

Background: Neuropathic arthropathy of the shoulder is a chronic progressive process characterized by joint destruction in the presence of a neurosensory deficit. Causes include syringomyelia, syphilis, diabetes, chronic alcoholism, and leprosy, with syringomyelia accounting for the vast majority of upper-extremity Charcot joints. Early presentation of this rare condition includes nonspecific symptoms such as swelling, erythema, sensory symptoms, and decreased functionality, making diagnosis challenging. Methods: We systematically reviewed 32 case reports published between 1924 and 2016. A total of 59 shoulders from 56 patients are included in this analysis. Variables include patient demographic characteristics, presentation, etiology, diagnostic techniques, treatment, outcome, and follow-up of Charcot shoulder. Results: We compiled a total of 25 right shoulders (42%), 24 left shoulders (41%), and 10 shoulders (17%) with unspecified laterality. The mean patient age (and standard deviation) was 49 ± 11 years, and the median age was 47 years. There was a higher prevalence in men (37 shoulders [63%]) compared with women (22 shoulders [37%]). Presenting symptoms included reduced range of motion (53 shoulders [90%]), paresthesia or hypoesthesia (45 [76%]), swelling (44 [75%]), weakness (40 [68%]), pain (31 [53%]), and reduction in deep tendon reflexes (22 [37%]). Shoulder radiographs were made in all cases. The presence of a syrinx was detected in 45 shoulders (76%) with magnetic resonance imaging, myelography, or clinical diagnosis. Sixteen shoulders (27%) reported exposure to trauma, with a 69% decrease in time from presentation to diagnosis compared with non-traumatic cases. Treatment was categorized as solely nonoperative management (14 [24%]), operative management (13 [22%]), combined therapy (20 [34%]), and no treatment listed (10 [17%]). Two surgical cases (3%) were excluded from our treatment group analysis as they were treated for unrelated or misdiagnosed conditions. Conclusions: Our study increases awareness and understanding of this complex, progressive disease to reduce delay and misdiagnosis and to contribute to the standard-of-care recommendations. Level of Evidence: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Charcot Arthropathy of the Shoulder Associated with Syringomyelia: A Report of 2 Cases

“…In general, the affected shoulder presents a progressive degeneration of the humeral head and glenoid. 5 The pathophysiology of neuropathic arthropathy is not completely proven, and there are two commonly accepted theories: neurovascular and neurotraumatic. The neurotraumatic theory claims that joint destruction is caused by repeated microtrauma, as a consequence of loss of proprioceptive and peripheral sensitivity resulting in macroscopic injuries such as fractures, dislocations, and joint deformities.…”

“…4 About 5% of Charcot arthropathy cases affect the shoulder joint. 5 Shoulder involvement in Charcot neuroarthropathy is commonly misdiagnosed and often confused with infections, rotator cuff tendon rupture, fractures, or pathological conditions with a better prognosis. 6 The most common cause of shoulder Charcot arthropathy is syringomyelia.…”

Radiographic Absence of the Left Humeral Head

“…The diagnosis of Charcot shoulder should be made only after exclusion of other causes of joint destruction, including septic arthritis, osteolytic lesions from malignancy, rheumatoid arthritis, synovial chondromatosis, trauma, soft tissue sarcoma, and Milwaukee shoulder syndrome, a destructive arthropathy caused by deposition of hydroxyapatite crystals …”

“…Surgical resection of a syrinx in patients with Charcot shoulder due to syringomyelia may slow joint deterioration and improve neurologic symptoms . Treatments for Charcot shoulder include nonsteroidal anti-inflammatory medications, joint aspiration if an effusion is present, patient education about avoidance of mechanical trauma, physical therapy, and rehabiliation . Shoulder surgery is not typically recommended, but may be considered for a subset of patients with Charcot shoulder who do not improve with nonoperative management …”

“…Approximately 80% of people with Charcot shoulder have syringomyelia, characterized by a fluid-filled cavity (syrinx) in the spinal cord . Other conditions associated with Charcot shoulder include diabetes, syphilis, alcohol use disorder, Arnold-Chiari malformations, cervical spondylosis, amyloidosis, end-stage kidney disease, myelodysplasia, multiple sclerosis, peripheral neuropathy, intra-articular steroid injection, tuberculosis, leprosy (Hansen disease), and gigantism …”