Résumé :
|
Introduction: The motor sensory neuropathies Charcot Marie Tooth (CMT) can be classify genetically as type 1, 2 and x link chromosome, later can be divided in subtypes A, B and C based on the genotype. CMT is the most common hereditary neuropathy,CMT type 2 is electrophysiologically characterized with normal nerve conduction velocities and low amplitude. On this type of patients Motor Unit Number Estimation (MUNE) is a good study to get information about distribution and severity of the pathology, mostly in diseases that have loss of motor units or axons, so it is a good study to establish prognosis or to stratify the disease by phases of evolution. Objective: Evaluate electrophysiological changes by the MUNE and F waves at patients with CMT type 2 evaluated at Rehabilitation National Institute (INR) with 1 year of evolution.Method: 10 patients were included; 7 males; 3 females with CMT 2 proven clinically, electrophysiologically and histopathologically. The median age was of 13.5 years old, and median evolution time of 8 years. Motor Nerve Conduction (MNC) studies and F wave at median and peroneal nerves were done, registering: latency, persistence and cronodispersion; MUNE was done using statistical method, it was used a Viking Select IV, Nicolet Biomedical Inc. hardware. Results: Significant correlations were obtained between the MUNE done in the study of 1 year ago at the right median and peroneal nerves (p<0.05). Also for F wave chonodispersion at the right peroneal nerve (p<0.05).Spearman correlation was done, but there was no significant correlations between the studied variables.Conclusions: It is required to increase the patients sample, so it can be stratify the disease by age and time of evolution, also the time of monitoring of the disease at patients might be increased as well to evaluate the electrophysiological changes in relation with the time of evolution of this pathology that is slowly progressive. Key words: Charcot Marie Tooth (CMT); Motor Unit Number Estimate (MUNE); Motor Nerve Conduction (MNC); Rehabilitation National Institute (INR).
|