Risk of bias in included studies Figure 2 presents a summary of the 'Risk of bias' assessments of the included studies by the review authors. [cochranelibrary.com]

• Paresis

  Primary loss of the myelin sheath, called demyelination, leads to secondary axonal degeneration, resulting in muscular atrophy and paresis (Krajewski 2000; Martini 2001). [cochranelibrary.com]

• Limb Weakness

  The most prominent symptom in all forms of CMT1 is progressive distal wasting of peroneal and calf muscles resulting in distal lower limb weakness, beginning in the first or second decade of life (Vallat 2013). [cochranelibrary.com]

We assessed treatment effects by meta‐analysis of data using forest plots. We determined the overall treatment effect by the Z‐score. We considered a significance level of P < 0.05 as the threshold for statistically significant treatment effects. [cochranelibrary.com]

Congenital Myasthenic Syndromes: a Clinical and Treatment Approach. Current treatment options in neurology, 20(9), 36. https://doi.org/10.1007/s11940-018-0520-7 Rodríguez Cruz, P. M., Palace, J., & Beeson, D. (2018). [igenomix.es]

Arthrogryposis (multiple congenital contractures): Diagnostic approach to etiology, classification, genetics, and general principles. European Journal of Medical Genetics, 57(8), 464-472. doi:10.1016/j.ejmg.2014.03.008 Solicitar información [igenomix.es]

Pathophysiology The pathophysiological mechanisms that lead to the clinical phenotype are still unclear for many of the genes implicated in CMT. [cochranelibrary.com]