No abstract available.
Neuromuscular Diseases*

The present study was designed to evaluate the diagnostic efficacy and the clinical usefulness of quantitative analysis of interf erence pattern in differentiating the neuromuscular diseases. To obtain the normal control value, the automatic turns/amplitude analysis of interference pattern in biceps brachii and tibialis anterior muscle was done in 30 healthy normal subjects. The same procedure was performed in 16 patients with myopathy and 14 neuropathy to determine the diagnostic sensitivity of this method. Original pairs of turns/amplitude data obtained from controls converted into the logarithmic scale and linear regression equation, were calculated. Boundary of normal cloud was defined by the + 2 S. D. lines, maximum turn and amplitude. The result was considered abnormal if two or more turns/amplitude values were outside the zone normal cloud. The values of myopathic patients shifted toward the right lower quadrant meaning high turns and small amplitudes, but those of neuropathic patients shifted toward the left upper quadrant meaning few turns and large amplitudes. In all subjects of each patient group, at least two turns/amplitude values were outside the zone of normal cloud. In addition, the sequential changes of mean turns/amplitude ratio were well correlated with the disease activity. These results suggest that this method of automatic turns/amplitude analysis could be used not only as a supplementary diagnostic tool in differentiating the neuromuscular disorders, but also a parameter of monitoring the disease activity.
Humans ; Linear Models ; Muscular Diseases ; Neuromuscular Diseases*

No abstract available.
Humans ; Neuromuscular Diseases* ; Noninvasive Ventilation* ; Weaning*

Thenar motor unit number estimation(MUNE) was performed in 49 normal subjects without known neuromuscular diseases using statistical method. The purposes of this study were to compare MUNE parameters between the dominant and non-dominant hands and to evaluate the changes according to age. Reproducibility was assessed in 20 subjects also. The results showed that thenar motor units number, maximum negative peak compound muscle action potential (CMAP) area and mean area of single motor unit potentials(SMUP) were not different between dominant and non-dominant sides, but maximum negative peak CMAP amplitude was larger in dominant than non-dominant side. With aging, the motor unit number, maximum negative peak CMAP amplitude and area decreased and the mean area of single motor unit potentials increased. There was a high reproducibility between the test and the retest values for motor unit number, maximum negative peak CMAP amplitude and area as well as mean area of single motor unit potentials. The statistical estimate of the motor unit number is a reliable method and easily available in clinical settings. The results of this study are expected to be used as a baseline data for the future.
Action Potentials ; Aging ; Hand ; Muscles* ; Neuromuscular Diseases

OBJECTIVE: To establish turns-amplitude, activity-envelope amplitude and activity-number of small segements (NSS) clouds of normal early teens using interference pattern. METHOD: Forty four healthy early adolescence from 11 to 15 years old participated in this study. The interference patterns were analyzed using quantitative electromyography of the biceps brachi and tibialis anterior muscles. The interference patterns were measured at 3 to 5 different force levels ranging from minimum to maximum and turns, mean amplitude, activity, envelope amplitude, NSS were analyzed. By turns/amplitude, activity/envelope amplitude and activity/ NSS ratio, normal clouds of gender related each parameters were obtained. RESULTS: The turns-amplitude, activity-envelope amplitude and activity-NSS ratio of the biceps brachii and tibialis anterior muscles were obtained. Normal clouds of gender related turns-amplitude, activity-envelope amplitude and activity-NSS for each muslces were established. CONCLUSION: By using normal cloud patterns of turns- amplitude, activity-envelope amplitude and activity-NSS, automatic interference pattern analysis may contribute to diagnose neuromuscular disease in early adolescent patients.
Adolescent* ; Electromyography* ; Humans ; Muscles ; Neuromuscular Diseases

Analysis of insertional activity is a routine part of the clinical electromyogrphic examination. It provides an information of muscle excitability but it's clinical significance has not perfectively accepted yet. This study was designed to evaluate clinical usefulness of insertional activity through quantitative analysis in the diagnostic field of pathology. Monopolar needle electrode was inserted briefly in the biceps brachii, paralumbar spinal and tibialis anterior muscles of the normal and denervated muscles. Total duration and spike duration of the insertional activity were measured 10 times in each muscle and averaged. Within spike duration we measured turns, mean amplitude, turns/amplitude, RMS, mean frequency and median frequency. The measured parameters of insertional activities were not significantly different according to the muscle in normal controls. In denervated muscles, the turns, mean amplitude, RMS, mean frequency and median frequency were decreased but turns/amplitude was increased compared to those of normal controls. But there were no difference in total duration and spike duration between normal and denervated muscles. In denervated muscles the muscle power was positively correlated with turns, mean amplitude, RMS, mean frequency and median frequency, and the grade of abnormal spontaneous activities was inversely correlated with turns, mean amplitude, RMS, mean frequency and median frequency. Therefore quantitative analysis of insertional activity could be a useful method for the diagnosis of neuromuscular disease.
Diagnosis ; Electrodes ; Muscles ; Needles ; Neuromuscular Diseases ; Pathology

Myotonic dystrophy, a multi-systemic disease with cardiac involvement, is the most common inherited neuromuscular disease. Here, we report the results of radiofrequency catheter ablation of persistent atrial fibrillation in a patient with myotonic dystrophy and achalasia-like esophageal dilatation.
Atrial Fibrillation* ; Catheter Ablation* ; Dilatation* ; Humans ; Myotonic Dystrophy* ; Neuromuscular Diseases

This is a case report of oculocraniosomatic neuromuscular disease with "ragged-red fibers". The patients is 36 years old female who is revealing slowly progressive ptosis, external ophthalmoplegia, general weakness and mild right hemiparesis and hemianesthesia. The EMG findings are suggestive of diffuse myopathic process and the frozen muscle specimen from the left deltoid muscle shows the characteristic "ragged-red fibers" with classical myopathic changes.
Adult ; Deltoid Muscle ; Female ; Humans ; Neuromuscular Diseases* ; Ophthalmoplegia ; Paresis

This is a case report of oculocraniosomatic neuromuscular disease with "ragged-red fibers". The patients is 36 years old female who is revealing slowly progressive ptosis, external ophthalmoplegia, general weakness and mild right hemiparesis and hemianesthesia. The EMG findings are suggestive of diffuse myopathic process and the frozen muscle specimen from the left deltoid muscle shows the characteristic "ragged-red fibers" with classical myopathic changes.
Adult ; Deltoid Muscle ; Female ; Humans ; Neuromuscular Diseases* ; Ophthalmoplegia ; Paresis

PURPOSE: To assess the ability of a mechanical in-exsufflator (MI-E), either alone or in combination with manual thrust, to augment cough in patients with neuromuscular disease (NMD) and respiratory muscle dysfunction. MATERIALS AND METHODS: For this randomized crossover single-center controlled trial, patients with noninvasive ventilator-dependent NMD were recruited. The primary outcome was peak cough flow (PCF), which was measured in each patient after a cough that was unassisted, manually assisted following a maximum insufflation capacity (MIC) maneuver, assisted by MI-E, or assisted by manual thrust plus MI-E. The cough augmentation techniques were provided in random order. PCF was measured using a new device, the Cough Aid. RESULTS: All 40 enrolled participants (37 males, three females; average age, 20.9±7.2 years) completed the study. The mean (standard deviation) PCFs in the unassisted, manually assisted following an MIC maneuver, MI-E-assisted, and manual thrust plus MI-E-assisted conditions were 95.7 (40.5), 155.9 (53.1), 177.2 (33.9), and 202.4 (46.6) L/min, respectively. All three interventions significantly improved PCF. However, manual assistance following an MIC maneuver was significantly less effective than MI-E alone. Manual thrust plus MI-E was significantly more effective than both of these interventions. CONCLUSION: In patients with NMD and respiratory muscle dysfunction, MI-E alone was more effective than manual assistance following an MIC maneuver. However, MI-E used in conjunction with manual thrust improved PCF even further.
Cough* ; Female ; Humans ; Insufflation ; Male ; Neuromuscular Diseases ; Respiratory Muscles*