Innovative quantitative testing of hand function in Charcot‐Marie‐Tooth neuropathy

Abstract

To describe a new test to quantitatively evaluate hand function in patients affected by Charcot-Marie-Tooth neuropathy (CMT). The sensor-engineered glove test (SEGT) was applied to CMT patients (N: 26) and compared with a cohort of healthy controls (HC, N: 26). CMT patients were further divided into subjects with clinically normal (group 1) or impaired hand (group 2) function. The SEGT parameters evaluated were touch duration, inter-tapping interval, and movement rate parameters of two different sequences: finger tapping (FT) and index-medium-ring-little (IMRL) performed at self-paced mode (SPM) and maximum velocity (MV). Hand function and strength were assessed by the 9-hole peg test (9HPT) and dynamometry. Disability of patients was measured by the CMT neuropathy score. CMT patients had significantly worst performances at SEGT than controls regarding the rate of execution of both FT (at MV) and IMRL sequences (at SPM and MV). The rate parameter at MV in IMRL sequence showed a significant trend of decreasing in its average between HC (n: 26, rate = 3.08 ± 0.52 Hz), group 1 (n: 9, rate = 2.64 ± 0.66 Hz) and group 2 (n: 17, rate = 2.19 ± 0.45 Hz) (p for trend <0.001). No correlations were found with either 9HPT, dynamometry, electrophysiology, and the CMT neuropathy score. The SEGT test is sensitive to show hand dysfunction in CMT patients, with and without clinically impaired hands.

Testing overwork weakness in Charcot‐Marie‐tooth disease: Is it true or false?

“…Based on previous observations, we considered the FT and IMRL exercises at the maximum velocity with eyes closed paradigm. 17 In both exercises, normal subjects have a statistically significant better performance in the DH, proving the superiority of the DH 25 even in the dexterity.…”

“…Data were processed with customized software from Glove Analyzer System which permits selection to acquisition and experimental protocol. The following parameters were measured: touch duration (TD) or contact time between thumb and another finger (in ms); inter‐tapping interval (ITI) or time between the end of the contact of the thumb and another finger and the beginning of successive contact (in ms); movement rate (MR, 1/[TD + ITI]) or frequency of complete motor task (in Hz) …”

“…The SEGT was used according to a previously published protocol. 17 Briefly, finger opposition movements were evaluated using motor sequences in a quantitative spatial-temporal way. An "eyes-closed para- finger and the beginning of successive contact (in ms); movement rate (MR, 1/[TD + ITI]) or frequency of complete motor task (in Hz).…”

“…An "eyes-closed para- finger and the beginning of successive contact (in ms); movement rate (MR, 1/[TD + ITI]) or frequency of complete motor task (in Hz). 17…”

“…1 Finally, we tested the dexterity of the subjects with an innovative quantitative operator-independent method, the SEGT. 17 Although the effect of the hand dominance in the dexterity in normal subjects is unknown, we expect that DH performs better than NDH. In CMT patients, there are few works that affirms to evaluate the dexterity with the 9 hole peg test, and even if the results reported seem to be accurate and responsive, they refer to a small population.…”