Upper Extremity Entrapment Neuropathies in Adults With Arthrogryposis Multiplex Congenita: A National Database Study.

Abstract

Adults with arthrogryposis multiplex congenita (AMC) experience lifelong disability from joint contractures, yet susceptibility to upper extremity compressive neuropathy remains unevaluated. Using the TriNetX US Collaborative Network, we identified 2772 adults with AMC and 100,903,909 controls. Prevalence of upper extremity entrapment neuropathies was compared using prevalence ratios (PR) and 95% confidence intervals, with age-stratified analyses across three strata (18-39, 40-59, and 60-69 years). Adults with AMC demonstrated elevated entrapment neuropathy rates across all distributions. Carpal tunnel syndrome was nearly twice as prevalent (PR 1.84, 95% CI 1.49-2.27), cubital tunnel syndrome nearly four times as prevalent (PR 3.76, 95% CI 2.87-4.91), and proximal neuropathies four- to sixfold more common (PR range 4.77-5.44, all p < 0.001). Electrodiagnostic testing was proportionally less elevated (PR 2.43), arguing against detection bias. Carpal tunnel syndrome comprised a smaller share of diagnoses in AMC versus controls (44% vs. 65%), reflecting pan-upper-extremity involvement. Age-stratified rate ratios increased progressively, reaching 16.43-fold for carpal tunnel syndrome and 74.70-fold for cubital tunnel syndrome in the 60-69 stratum. AMC confers elevated, age-progressive risk for compressive neuropathy across multiple upper extremity distributions, warranting proactive surveillance to prevent further functional impairment and chronic pain.