Tobias Bäumer1,2, Nina Bühring3, Thomas Schelle4, Alexander Münchau5, Nicole Muschol3. 1. Department of Paediatric and Adult Movement Disorders and Neuropsychiatry, Institute of Neurogenetics, University of Lübeck, Lübeck, Germany. tobias.baeumer@neuro.uni-luebeck.de. 2. Department of Neurology, University Medical Hospital Hamburg-Eppendorf, Hamburg, Germany. tobias.baeumer@neuro.uni-luebeck.de. 3. Department of Paediatrics, University Medical Hospital Hamburg-Eppendorf, Hamburg, Germany. 4. Department of Neurology, Städtisches Klinikum Dessau, Dessau Rosslau, Germany. 5. Department of Paediatric and Adult Movement Disorders and Neuropsychiatry, Institute of Neurogenetics, University of Lübeck, Lübeck, Germany.
Abstract
AIM: Mucopolysaccharidoses (MPS) are a group of diseases with an almost 100% lifetime incidence of carpal tunnel syndrome (CTS) in MPSsubtypes I, II, and VI. We compared nerve ultrasound with clinical signs and electrophysiology in a clinical setting to screen for CTSin MPS. METHOD: Twenty-four patients (13 male, 11 female, mean age of 7y 11mo [SD8y 5mo], range 6mo-29y) were screened for CTS. Eight of these patients were re-examined post-operatively. Clinical signs, distal motor latency, compound muscle action potential, sensory nerve action potential amplitude and velocity, as well as echogenicity and the cross-sectional area (CSA) of the median nerve at the wrist and forearm determined with ultrasound were assessed and the wrist to forearm ratio (WFR) calculated. Eighteen healthy participants formed a comparison group, who were also investigated with nerve ultrasound. RESULTS: In 26% of the patients' hands, clinical signs of CTSwere present; 77% fulfilled electrophysiological and 92% nerve ultrasound criteria for CTS. Post-operatively, electrophysiology improved significantly, whereas ultrasound results were unchanged. In the comparison group, age and height correlated with the CSA, but not with WFR. INTERPRETATION: Nerve ultrasound is a useful and painless primary screening tool for CTSin MPS.
AIM: Mucopolysaccharidoses (MPS) are a group of diseases with an almost 100% lifetime incidence of carpal tunnel syndrome (CTS) in MPSsubtypes I, II, and VI. We compared nerve ultrasound with clinical signs and electrophysiology in a clinical setting to screen for CTSin MPS. METHOD: Twenty-four patients (13 male, 11 female, mean age of 7y 11mo [SD8y 5mo], range 6mo-29y) were screened for CTS. Eight of these patients were re-examined post-operatively. Clinical signs, distal motor latency, compound muscle action potential, sensory nerve action potential amplitude and velocity, as well as echogenicity and the cross-sectional area (CSA) of the median nerve at the wrist and forearm determined with ultrasound were assessed and the wrist to forearm ratio (WFR) calculated. Eighteen healthy participants formed a comparison group, who were also investigated with nerve ultrasound. RESULTS: In 26% of the patients' hands, clinical signs of CTSwere present; 77% fulfilled electrophysiological and 92% nerve ultrasound criteria for CTS. Post-operatively, electrophysiology improved significantly, whereas ultrasound results were unchanged. In the comparison group, age and height correlated with the CSA, but not with WFR. INTERPRETATION: Nerve ultrasound is a useful and painless primary screening tool for CTSin MPS.
Authors: Pablo Martín-Juste; Isabel Parada-Avendaño; Victoria E Gómez-Palacio; Jorge Gil-Albarova Journal: Childs Nerv Syst Date: 2022-03-18 Impact factor: 1.532