Novel measurement for automated interference pattern analysis of the diaphragm.

The automated interference pattern analysis for limb muscles was modified to take into account the unique features of the needle EMG of the diaphragm. The modification was successful in recording more accurately the number of small and large segments and the activity levels with inspiratory effort. "Clouds" were generated in ten healthy subjects. The techniques may prove useful in electrophysiological investigations.

Repetitive phrenic nerve stimulation study in normal subjects.

In patients with myasthenia gravis (MG), it may be difficult to determine by clinical methods if respiratory insufficiency is due to a defect in neuromuscular transmission. We therefore studied the technique of repetitive electrical stimulation of the phrenic nerve in 6 healthy subjects. It was easily performed and quite reproducible. Responses at 3-Hz stimulation were recorded from surface electrodes of the ipsilateral hemidiaphragm, before and after exercise. We analyzed the percent decrement of the negative peak (NP) amplitude, area, and duration of the diaphragmatic compound muscle action potential (CMAP) between the first and the fifth or sixth potentials. The mean percentage change of the area was -2.2% (+/-4.3), and in all tests the change was <10.6%, yielding a normal range of <11%. The change in the NP amplitude was 12.1% (+/-8.3); in duration, the change was -8.7% (+/-9.6). Producing diaphragm fatigue did not change these results. The increase in amplitude and decrease in duration with little change in area, termed pseudofacilitation, may be due to shifts in the position of the diaphragm affecting volume conduction. The technique is a promising tool in the diagnosis of respiratory involvement from neuromuscular transmission disorders.

Subacute uremic and diabetic polyneuropathy.

We present 4 patients who had a subacute, predominantly motor polyneuropathy associated with diabetes mellitus and end-stage renal disease. Electrophysiological studies and muscle biopsy indicated a primary axonal degeneration of nerve with secondary segmental demyelination, and mild to moderate, acute and chronic denervation of muscle. A relative absence of denervation potentials on needle electromyography was an unusual feature. Three of our patients improved with a switch from conventional to high-flux hemodialysis. We speculate on possible mechanisms.

Distinctive electrophysiological features of denervated muscle in uremic patients.

Abnormal spontaneous activity is a hallmark of acute or subacute denervation of skeletal muscle, particularly in patients with uremic neuropathy. We report 5 patients in whom such activity was unexpectedly minimal or absent.

Central and peripheral respiratory electrophysiological studies in myotonic dystrophy.

Acute and chronic respiratory failure is a common and potentially life-threatening feature in patients with myotonic dystrophy (MD). The causes may be varied, and can involve both the central and peripheral nervous system. To evaluate the incidence of respiratory muscle involvement and the function of the central motor inspiratory pathway to phrenic motor neurons we performed magnetic stimulation of the cortex and cervical spinal cord, phrenic nerve conduction studies and needle EMG of diaphragm and intercostal muscles in 25 patients with MD. The results were compared with those from 35 healthy subjects. In addition, pulmonary function tests, blood gas analyses and static mouth pressures were evaluated. Abnormalities in response to magnetic stimulation, including a reduced compound muscle action potential (CMAP) from the diaphragm and increased excitability threshold, indicated impaired central inspiratory drive in 20% of cases. Phrenic nerve conduction showed a reduced diaphragmatic CMAP amplitude in 20%, and a delayed negative peak onset latency in 4% of cases. Abnormalities in diaphragm and intercostal muscle needle EMG were found in 76% of cases, these were mainly myotonic discharges (68%) and a decrease in the number of active motor units (36%). Patients with abnormal respiratory electrophysiological parameters had a significantly lower functional vital capacity (FVC; P = 0.005). The duration of the disease correlated negatively with diaphragmatic CMAP amplitude to phrenic nerve, but not magnetic, stimulation. Our results demonstrate that the involvement of the central inspiratory pathway is common in MD patients. Central and peripheral electrophysiological studies of the diaphragm should be considered in the diagnosis and management of patients with MD and dyspnoea.

Lambert-Eaton myasthenic syndrome presenting with severe respiratory failure.

Two cases of Lambert-Eaton myasthenic syndrome (LEMS) who presented with primary respiratory failure are reported. In each case, although not initially suspected clinically, the electrophysiological findings, which included reduced compound muscle action potential amplitudes, decrement to 3-Hz stimulation, and potentiation after 40-Hz stimulation, led to the diagnosis in the critical care unit. Electrophysiological studies of the respiratory system, including repetitive nerve stimulation of the phrenic nerve, were extremely valuable in management. As shown by these cases, the severe respiratory failure in LEMS is reversible with treatment. Thus, LEMS should be considered in cases of unexplained respiratory failure, other clinical features of the disorder sought, and the electrophysiological hallmarks looked for including studies of the respiratory system.

The cortical representation of somatosensory evoked potentials of the phrenic nerve.

Respiratory electrophysiological studies are of essential value in diagnosing and managing patients with respiratory failure, but assessment of the sensory phrenic nerve fibres has been neglected. We recorded phrenic nerve somatosensory evoked potentials (SSEPs) by combining neurophysiological and neuroimaging techniques in three healthy subjects. Evoked potentials of the phrenic nerve showed the highest amplitude at CP3, determined by the modified 10-20 EEG system, and occurred at a constant latency, PI at 12.0 +/- 0.6 ms, and NI at 17.3 +/- 0.8 ms. Single photon emission computer tomography (SPECT) performed during phrenic nerve stimulation revealed focal neuronal activation in the somatosensory pathways. Intravenously administered Tc-99m Ethyl Cysteinate Dimer (ECD) was used as a blood flow tracer to obtain baseline and activated images. After image registration, baseline images were compared voxel-by-voxel with the activation images. The mean inter-subject summation image of the activated state was compared with that of the baseline state using ten normal subjects. The extent of the total voxel volume increase on the mean images of the 3 activated SPECT images was 0.7%, and a mean signal increase of 22%. For further anatomic localization of regional increases in signal, the magnetic resonance image (MRI) scan of each subject was registered and superimposed on the activated stage SPECT image. This method may be used clinically to study the pathophysiology of impaired central respiratory drive.

Transcortical and cervical magnetic stimulation with recording of the diaphragm.

Transcortical and cervical magnetic stimulation is a potential method of examining the central inspiratory pathway to phrenic motor neurons. The reliability and accuracy of this technique were studied. We performed magnetic stimulations of the cortex and cervical spinal cord with recording from both hemidiaphragms in 35 normal subjects using two different stimulation coils (90-mm circular coil and 70-mm figure-of-eight coil). Needle electrode recordings and ultrasound real-time documentation in 2 subjects excluded volume-conducted contaminations from adjacent chest wall and abdominal muscles. The effect of diaphragmatic facilitation (stimulation at the end of a deep breath) on latency, and amplitude were compared to the effect of hypothenar muscle facilitation. Normal ranges were established for: latency; central motor conduction time; amplitude; amplitude ratio between between peripheral and both cortical and cervical amplitude; and excitability threshold. The latencies were similar for both coils. The amplitudes were significantly higher, and excitability thresholds significantly lower for the 90-mm circular coil, indicating that this coil is preferable for transcortical diaphragmatic stimulations. The effect of facilitation was greater for hypothenar than diaphragmatic recordings. There was excellent right-left agreement for all measurements. Transcortical and cervical magnetic stimulation with recording from the diaphragm can be used routinely to diagnose and monitor patients with impaired central respiratory drive.

Needle EMG of the human diaphragm: power spectral analysis in normal subjects.

Needle EMG of the diaphragm was performed in 43 diaphragms in 23 healthy volunteers. The mean +/- standard deviation for the median frequency (MF) of the power spectrum was 233.3 +/- 58.1 Hz. The MF increased with age and showed a negative correlation with the forced vital capacity (FVC), but there was no correlation with other anthropometric measures or the results of phrenic nerve conduction study. The higher MF in older subjects may be due to early recruitment of larger units. The negative correlation between MF and FVC is likely secondary to a lower level of contraction required for normal breathing in subjects with high FVC. The integrated EMG (iEMG) of each inspiration strongly correlated with the tidal volume and the duration of inspiration. Power spectral analysis of diaphragmatic EMG is feasible and reliable. It can be used to assess respiratory muscle fatigue and may help in the diagnosis of neuromuscular disorders affecting the diaphragm.

Respiratory electrophysiological studies in Guillain-Barré syndrome.

Respiratory failure is a common and potentially life threatening complication in patients with Guillain-Barré syndrome. The incidence of phrenic nerve involvement and the predictive value of phrenic nerve conduction and diaphragmatic needle EMG were studied in 40 patients with Guillain-Barré syndrome within the first three days of admission to hospital. The negative peak onset latency of the diaphragmatic compound muscle action potential (CMAP), and its amplitude, duration, and area were abnormal in 83%. The need for ventilation was correlated with diaphragmatic CMAP amplitude (P = 0.005), and area (P = 0.001), but not with latency or duration. Abnormalities in diaphragmatic needle EMG were found in 45%, mainly a decreased number of motor unit potentials. The abnormalities correlated with the need for ventilation (P = 0.013). Of the 40% who required ventilation, all had either abnormal phrenic conduction, abnormal diaphragmatic needle EMG, or both. Eighty one per cent of the ventilated patients had abnormal forced vital capacity on the day of the electrophysiological examination. The results indicate that phrenic nerve conduction studies and diaphragmatic EMG are useful in detecting respiratory involvement in patients with Guillain-Barré syndrome and in identifying those at risk of respiratory failure.

Neuromuscular disorders associated with failure to wean from the ventilator.

OBJECTIVE: To determine, by retrospective chart analysis, the frequency, type and significance of neuromuscular disorders in patients whose clinical features suggested a neuromuscular cause of failure to wean. BACKGROUND: Failure to wean is a common and difficult problem in critical care units. While a neuromuscular cause may be suspected in some patients, the frequency and type has not been determined utilizing comprehensive electrophysiological studies of limbs and the respiratory system. Such knowledge may aid in patient management and prognosis. METHODS: The clinical setting was a critical care/trauma centre that admits 1500 patients per year, approximately 500 being on ventilators for longer than five days. We analyzed the hospital charts of 40 patients admitted to the unit during three years, whose respiratory assessment suggested a neuromuscular cause for failure to wean from the ventilator. To investigate this possibility, we performed electrophysiological studies of the limbs and also of the respiratory system by phrenic nerve conduction and needle electromyography of the chest wall and diaphragm. The results were compared to 25 healthy controls. RESULTS: 38 of 40 patients (95%) had a neuromuscular disorder: 25--critical illness polyneuropathy, 2--Guillain-Barré syndrome, 4--diabetic and critical illness polyneuropathy, 2--uremic and critical illness polyneuropathy, 10--an abnormality of central drive, 5--unilateral phrenic nerve palsy, 3--a neuromuscular transmission defect, and 5--a primary myopathy. Fifteen (38%) had a combination of disorders. Patients with more severe polyneuropathy took longer to wean, a mean of 136 versus 52 days (p = 0.007). The severity of the polyneuropathy had no effect on mortality. CONCLUSIONS: Electrophysiological studies of limbs and the respiratory system are together valuable in confirming the presence, and identifying the specific type of neuromuscular cause for difficulty in weaning from the ventilator. This information is important in patient management and prognosis.

Electrophysiological study of diaphragmatic myoclonus.

This is the first reported detailed electrophysiological study of diaphragmatic myoclonus. An 86 year old woman had rapid, intermittent epigastric pulsations. Neurological examination and imaging studies of the brain and spinal cord were normal. Needle EMG showed rhythmic contractions of the diaphragm and external intercostal muscles at 4 to 5 Hz. These contractions were often associated with suppression of normal breathing and were capable of maintaining adequate ventilation. Both diaphragms were involved but showed considerable variability in their relative latencies. Automated interference pattern analysis suggested a change in recruitment order, with selective activation of large phrenic motoneurons. The supraspinal mechanisms mediating diaphragmatic myoclonus are different from that of voluntary and involuntary rhythmic breathing, and seem to be unrelated to palatal myoclonus. The generator source is likely related to respiratory centres in the rostral medulla.

Phrenic nerve conduction study in normal subjects.

Phrenic nerve conduction studies were performed in 50 phrenic nerves from 25 normal subjects using a technique modified from previously described methods. The normal ranges for latency, amplitude, negative peak area, and duration were established. The latency correlates with age and the amplitude increases with chest circumference. With our method, the amplitude increases and the duration decreases with lung volume. We found good right-left agreement and reproducibility. Therefore, the unaffected side can be used as a reference in unilateral phrenic nerve lesions and previous studies can be used for comparison in serial studies. We recommend that phrenic nerve conduction studies be used routinely to diagnose and monitor patients with respiratory involvement from neuromuscular diseases.