4.5 Review

NERVE SONOGRAPHY IN CHARCOT-MARIE-TOOTH DISEASE: A SYSTEMATIC REVIEW AND META-ANALYSIS OF 6061 MEASURED NERVES

Journal

ULTRASOUND IN MEDICINE AND BIOLOGY
Volume 48, Issue 8, Pages 1397-1409

Publisher

ELSEVIER SCIENCE INC
DOI: 10.1016/j.ultrasmedbio.2022.04.220

Keywords

Neuromuscular ultrasound; Sonography; Nerve cross-sectional area; Hereditary neuropathy; Charcot-Marie-Tooth disease

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Due to its insidious character and variations in symptoms, diagnosing Charcot-Marie-Tooth (CMT) disease in children is challenging. This study conducted a systematic review and meta-analysis to evaluate the effectiveness of ultrasound in investigating nerve cross-sectional area (CSA) in CMT patients. The results showed the potential of ultrasound as a diagnostic tool for CMT.
Because of the insidious character and variations in presenting symptoms, Charcot-Marie-Tooth (CMT) disease is challenging to diagnose in children. Diagnosis is based on clinical and nerve conduction studies, as well as genetic examination. Therefore, competent nerve imaging techniques and non-invasive alternatives to nerve conduction studies are a necessity, especially in children. We performed a systematic review and meta-analysis to evaluate the current evidence and effectiveness of ultrasound in investigating nerve cross-sectional area (CSA) in those with CMT compared with healthy controls and to pool the CSA measurements. We included studies published in international peer-reviewed journals that measured nerve CSA by ultrasound in patients with CMT. We implemented double-arm meta-analyses to compare the mean CSA of nerves between patients with CMT and healthy controls by calculating the pooled mean difference in CSA. Moreover, we performed sub-group analyses by stratifying the studies according to the site of CSA measurement and examined the difference in nerve CSA between CMT1A and other CMT types. The included studies provide measurements of 12 nerve roots and nerves (vagus, C3, C4, C5, C6, greater auricular, phrenic, median, ulnar, fibular, tibial and sural nerves) in 628 patients with CMT and 586 healthy controls with a total of 6061 measured nerves. Meta-analyses of sonographic nerve CSA are provided to express nerve ultrasonography in the diagnosis of CMT patient. (C) 2022 World Federation for Ultrasound in Medicine & Biology. All rights reserved.

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