Lower limb manual muscle testing in the early stages of Charcot-Marie-Tooth disease type 1A.

摘要: Charcot-Marie-Tooth disease (CMT) is a genetically and clinically heterogeneous disorder that affects approximately one in 2,500 individuals. CMT 1A, which due to duplication the area containing PMP-22 gene on chromosome 17, most frequent subtype. To date, there no consensus among authors about muscles are weakened early stages of CMT, even though this knowledge would be crucial for deciding appropriate interventions restore balance between prevent development deformities. The aim study was evaluate strength several lower limb 1A. In series 45 patients (age 10-72 years; 21 males, 24 females) affected by we evaluated 83 non-operated limbs corresponded two milder five-level functional classification. foot muscles, seven leg thigh three pelvic girdle graded using manual muscle testing techniques Daniels Worthingham; power triceps surae graded, prone position, 4-level scale ability raise heel from floor. Muscle determined basis interobserver agreement estimated kappa statistics observers. flexor hallucis brevis lumbricals were very weak all limbs; strong more than 90% limbs, except peronei (strong 83.13%); test, but 16.87% standing test; proximal strong. large majority intrinsic