ANALYSIS OF ELECTRONEUROMYOGRAPHIC PARAMETERS IN PATIENTS WITH HEREDITARY MOTOR AND SENSORY NEUROPATHY TYPE 1A
DOI:
https://doi.org/10.11603/1811-2471.2023.v.i1.13652Keywords:
hereditary motor sensory neuropathy, electroneuromyographyAbstract
SUMMARY. The study of the correlation between neurophysiological parameters, clinical features, and neurological characteristics in patients with hereditary motor sensory neuropathy (HMSN) type 1A is crucial for comprehending the pathophysiology of this condition and identifying factors that contribute to disease severity and progression.
The aim – to assess changes in electroneuromyographic parameters in patients with HMSN type 1A based on age and duration of the period after the manifestation of the disease.
Material and Methods. We conducted stimulation electroneuromyography (ENMG) to evaluate the motor and sensory nerve conduction velocity (NCV), M-response parameters, and sensory responses of the ulnar, tibial, superficial peroneal, musculocutaneous, and femoral nerves on both sides of patients with HMSN type 1A . We analyzed ENMG data from 97 patients withHMSN type 1A at different times and categorized them by age. We only included ENMG studies with a complete description of the parameters of NCV and M-responses in digital terms. We used a Neuro-MVP-8 electroneuromyograph (DX-Systems, Ukraine) with computer registration for the study.
Results. We found that the greatest decrease in M-response amplitude occurred in patients aged 36–60 and 61–75 years with HMSN type 1A. Patients with a duration of more than 10 years after the manifestation of the disease had lower M-response amplitudes than those with a duration of less than 10 years. NCV remained stable in 88.7 % of cases during the observation period, whereas the M-response amplitude decreased in 85.4 % of cases as the disease progressed. We observed a high negative correlation between M-response amplitude and the duration of the disease.
Conclusions. The SPI did not correlate with age or onset of the disease in patients with HMSN type 1A but was positively associated with the duration of the period after the manifestation of the disease. A low NCV and M-response amplitude in the early stages of the disease may indicate a faster rate of progression of HMSN type 1A and serve as a predictor.
References
Carter, G.T., England, J.D., & Chance, P.F. (2004). Charcot-Marie-Tooth disease: electrophysiology, molecular genetics and clinical management. IDrugs : the investigational drugs journal, 7(2), 151-159.
Tavee, J. (2019). Nerve conduction studies: Basic concepts. Handbook of clinical neurology, 160, 217-224. DOI: 10.1016/B978-0-444-64032-1.00014-X.
Rubin, D.I. (2019). Needle electromyography: Basic concepts. Handbook of clinical neurology, 160, 243-256. DOI: 10.1016/B978-0-444-64032-1.00016-3.
Gans, B.M., & Kraft, G.H. (1981). M-response quantification: a technique. Archives of Physical Medicine and Rehabilitation, 62(8), 376-380.
Dyussenbayev, A. (2017). Age Periods Of Human Life. Advances in Social Sciences Research Journal, 4(6), 258-263. DOI: 10.14738/assrj.46.2924.
Morena, J., Gupta, A., & Hoyle, J.C. (2019). Charcot-Marie-Tooth: From Molecules to Therapy. International Journal of Molecular Sciences, 20(14), 3419. DOI: 10.3390/ijms20143419.
