Muscle velocity recovery cycles in myopathy.

OBJECTIVE: To understand the pathophysiology of myopathies by using muscle velocity recovery cycles (MVRC) and frequency ramp (RAMP) methodologies. METHODS: 42 patients with quantitative electromyography (qEMG) and biopsy or genetic verified myopathy and 42 healthy controls were examined with qEMG, MVRC and RAMP, all recorded from the anterior tibial muscle. RESULTS: There were significant differences in the motor unit potential (MUP) duration, the early and late supernormalities of the MVRC and the RAMP latencies in myopathy patients compared to controls (p < 0.05 apart from muscle relatively refractory period (MRRP)). When dividing into subgroups, the above-mentioned changes in MVRC and RAMP parameters were increased for the patients with non-inflammatory myopathy, while there were no significant changes in the group of patients with inflammatory myopathy. CONCLUSIONS: The MVRC and RAMP parameters can discriminate between healthy controls and myopathy patients, more significantly for non-inflammatory myopathy. MVRC differences with normal MRRP in myopathy differs from other conditions with membrane depolarisation. SIGNIFICANCE: MVCR and RAMP may have a potential in understanding disease pathophysiology in myopathies. The pathogenesis in non-inflammatory myopathy does not seem to be caused by a depolarisation of the resting membrane potential but rather by the change in sodium channels of the muscle membrane.

MScanFit motor unit number estimation (MScan) and muscle velocity recovery cycle recordings in amyotrophic lateral sclerosis patients.

OBJECTIVE: Motor Unit Number Estimation (MUNE) methods, such as the recently developed MScanFit MUNE (MScan), may be valuable in tracking motor unit loss in ALS. Muscle Velocity Recovery Cycles (MVRCs) provide information about muscle membrane properties and can reveal disease-related changes. This study was undertaken to test the applicability of MScan to the anterior tibial muscle (TA) and to test whether the MVRCs could improve understanding of ALS pathophysiology. METHODS: Twenty-six ALS patients and 25 healthy controls were evaluated by quantitative electromyography, nerve conduction study and the two novel methods: MScan and MVRC; all in the TA and peroneal nerve. RESULTS: The estimated number of motor units for ALS patients (Median: 45, interquartile range: 28.5-76.5) was significantly lower than for the controls (117, 96.0-121.0) (P = 2.19 × 10-7). Unit size was increased only when amplitudes were expressed as percentage of CMAP. Of MVRC measurements, only relative refractory period was significantly abnormal in patients. CONCLUSION: MScanFit MUNE gives a sensitive and quantitative measure of loss of TA motor units in ALS. Muscle fiber membrane properties are mostly unaffected, despite substantial denervation, presumably due to collateral reinnervation. SIGNIFICANCE: MScan is suitable for detecting motor unit loss in TA. MVRCs do not provide new insights in ALS.

Fasciculations in nerve and muscle disorders - A prospective study of muscle ultrasound compared to electromyography.

OBJECTIVES: We examined the clinical utility of muscle ultrasound (MUS) in detecting fasciculations in patients with nerve and muscle disorders (NMD) and investigated the impact on diagnostic sensitivity when combining electromyography (EMG) and MUS. METHODS: We included 58 consecutive patients suspected to have NMD and 38 healthy subjects (HS). Patients and HS underwent MUS in 14 skeletal and two bulbar muscles and the video recordings of the MUS were anonymised. Only patients underwent EMG. RESULTS: The follow-up diagnoses were: 15 Amyotrophic lateral sclerosis (ALS), 15 polyneuropathy, 14 patients had other diagnoses (disease-control group) and 14 patients had no pathological findings. MUS detected more muscles with fasciculations among ALS patients compared to all other groups. In ALS patients, the dominating pattern of fasciculations was continuous (45%). More proximal muscles showed fasciculations among ALS patients compared to all other patient groups. MUS was more sensitive than EMG in detecting fasciculations (58% vs. 48%). When combining the two methods, the sensitivity in detecting fasciculations increased to 65%. Fasciculations in nine muscles could predict the ALS diagnosis with high sensitivity and specificity. CONCLUSIONS: MUS is a sensitive tool in detecting fasciculations in patients with NMD and performs well compared to EMG in diagnosing ALS. SIGNIFICANCE: MUS may add valuable information in the clinic, especially in diagnosing ALS.

Neurophysiological localisation of ulnar neuropathy at the elbow: Validation of diagnostic criteria developed by a taskforce of the Danish Society of clinical neurophysiology.

OBJECTIVE: This study validates consensus criteria for localisation of ulnar neuropathy at elbow (UNE) developed by a taskforce of the Danish Society of Clinical Neurophysiology and compares them to the existing criteria from the American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM). The Danish criteria are based on combinations of conduction slowing in the segments of the elbow and forearm expressed in Z-scores, and difference between the segments in m/s. Examining fibres to several muscles and sensory fibres can increase the certainty of the localisation. METHODS: Diagnostic accuracy for UNE was evaluated on 181 neurophysiological studies of the ulnar nerve from 171 peer-reviewed patients from a mixed patient-group. The diagnostic reference standard was the consensus diagnosis based on all available clinical, laboratory, and electrodiagnostic information reached by a group of experienced Danish neurophysiologists. RESULTS: The Danish criteria had high specificity (98.4%) and positive predictive value (PPV) (95.2%) and fair sensitivity (76.9%). Compared to the AANEM criteria, the Danish criteria had higher specificity (p<0.001) and lower sensitivity (p=0.02). CONCLUSIONS: The Danish consensus criteria for UNE are very specific and have high PPV. SIGNIFICANCE: The Danish criteria for UNE are reliable and well suited for use in different centres as they are based on Z-scores.

Salzburg Consensus Criteria for Non-Convulsive Status Epilepticus--approach to clinical application.

BACKGROUND: Salzburg Consensus Criteria for diagnosis of Non-Convulsive Status Epilepticus (SCNC) were proposed at the 4th London-Innsbruck Colloquium on status epilepticus in Salzburg (2013). METHODS: We retrospectively analyzed the EEGs of 50 consecutive nonhypoxic patients with diagnoses of nonconvulsive status epilepticus (NCSE) at discharge and 50 consecutive controls with abnormal EEGs in a large university hospital in Austria. We implemented the American Clinical Neurophysiology Society's Standardized Critical Care EEG Terminology, 2012 version (ACNS criteria) to increase the test performance of SCNC. In patients without preexisting epileptic encephalopathy, the following criteria were applied: (1) more than 25 epileptiform discharges (ED) per 10-second epoch, i.e., >2.5/s and (2) patients with EDs ≤ 2.5/s or rhythmic delta/theta activity (RDT) exceeding 0.5/s AND at least one of the additional criteria: (2a) clinical and EEG improvements from antiepileptic drugs (AEDs), (2b) subtle clinical phenomena, or (2c) typical spatiotemporal evolution. In case of fluctuation without evolution or EEG improvement without clinical improvement, "possible NCSE" was diagnosed. For identification of RDT, the following criteria were compared: (test condition A) continuous delta-theta activity without further rules, (B) ACNS criterion for rhythmic delta activity (RDA), and (C) ACNS criteria for RDA and fluctuation. RESULTS: False positive rate in controls dropped from 28% (condition A) to 2% (B) (p = 0.00039) and finally to 0% (C) (p = 0.000042). Application of test condition C in the group with NCSE gives one false negative (2%). Various EEG patterns were found in patients with NCSE: (1) 8.2%, (2a) 2%, (2b) 12.2%, and (2c) 32.7%. Possible NCSE was diagnosed based on fluctuations in 57.1% and EEG improvement without clinical improvement in 14.2%. CONCLUSION: The modified SCNC with refined definitions including the ACNS terminology leads to clinically relevant and statistically significant reduction of false positive diagnoses of NCSE and to minimal loss in sensitivity. This article is part of a Special Issue entitled "Status Epilepticus".

Modulation of the muscle and nerve compound muscle action potential by evoked pain.

Background and aims To our knowledge there are no studies that have examined the effects of the experimental pain on muscle fibre excitability as measured by the amplitudes of the potentials evoked by direct muscle stimulation (DMS) in a muscle at rest. We hypothesized that evoked pain can modulate the muscle compound action potential (CMAP) obtained by DMS possibly due to changes in muscle fibre excitability. Methods Pain was evoked by intramuscular infusion of hypertonic saline in 50 men. Ten control subjects were infused with isotonic saline. The infusions were given distal to the motor end plate region of the dominant brachial biceps muscle (BBM) in a double-blind manner. The nerve CMAP was obtained by stimulating the musculocutaneous nerve and recording from the BBM using surface-electrodes. Muscle CMAPs were obtained by direct muscle stimulation with subdermal electrodes placed subcutaneously in the distal third of the muscle. A stimuli-response curve of the amplitudes from muscle CMAP was obtained by stimulating from 10 to 90 mA. Results There was a decrease of the nerve CMAP amplitudes after infusion of isotonic saline (from 13.78mV to 12.16 mV), p-value 0.0007 and of hypertonic saline (from 13.35 mV to 10.85 mV), p-value 0.0000. The percent decrease from before to after infusion was larger in the hypertonic saline group (19.37%) compared to the isotonic saline group (12.18%), p-value 0.025. There was a decrease of the amplitudes of the muscle CMAP after infusion of both isotonic (at 90 mA from 13.84mV to 10.32 mV, p value 0.001) and of hypertonic saline (at 90 mA from 14.01 mV to 8.19 mV, p value 0.000). The percent decrease was larger in the hypertonic saline group compared to the isotonic saline group for all the stimulations intensities. At 90 mA we saw a 42% decrease in the hypertonic saline group and 24.5% in the isotonic saline group, p value 0.005. There were no changes in conduction velocity. Conclusion We found a larger amplitude decrease of the muscle and nerve potentials following hypertonic saline infusion compared with that of isotonic saline. We suggest that this deferential outcome of hypertonic saline on muscle CMAP may be linked to the nociceptive effect on muscle fibre membrane excitability. Implications The study supplies with some evidence of the peripheral effect of muscle pain. However, further trials with other nociceptive substances such as capsaicin should be performed.

Clinical assessment of prognostic factors for long-term pain and handicap after whiplash injury: a 1-year prospective study.

BACKGROUND AND PURPOSE: Physical mechanisms are the possible factors involved in the development and maintenance of long-term handicaps after acute whiplash injury. This study prospectively examined the role of active neck mobility, cervical and extra-cervical pains, as well as non-painful complaints after a whiplash injury as predictors for subsequent handicap. METHODS: Consecutive acute whiplash patients (n = 688) were interviewed and examined by a study nurse after the median of 5 days after injury, and divided into a high- or a low-risk group by an algorithm based on pain intensity, number of non-painful complaints and active neck mobility [active cervical range of motion (CROM)]. All 458 high-risk patients and 230 low-risk patients received mailed questionnaires after 3, 6 and 12 months. Two examiners examined all high-risk patients (n = 458) and 41 consecutive low-risk patients at median 11, 109, 380 days after injury. The main outcome measures were: handicaps, severe headaches, neck pain and neck disability. RESULTS: The relative risk for a 1-year disability increased by 3.5 with initial intense neck pain and headaches, by 4.6 times with reduced CROM and by four times with multiple non-painful complaints. CONCLUSION: Reduced active neck mobility, immediate intense neck pain and headaches and the presence of multiple non-painful complaints are the important prognostic factors for a 1-year handicap after acute whiplash.

A double-blind, controlled study of botulinum toxin A in chronic myofascial pain.

BACKGROUND: Recent studies have reported a potential analgesic effect of botulinum toxin A (BTXA) in musculoskeletal pain. The present double-blind, randomized, placebo-controlled, parallel clinical trial studied the effect of BTXA on pain from muscle trigger points and on EMG activity at rest and during voluntary contraction. METHODS: Thirty patients with trigger points in the infraspinatus muscles received either 50 units/0.25 mL of BTXA or 0.25 mL of isotonic saline. Baseline measures were determined during a run-in period of 1 week. Outcome measures including local and referred spontaneous pain, pain detection and tolerance thresholds to mechanical pressure, and shoulder movement were assessed at 3 and 28 days after injection. The interference pattern of the EMG during maximal voluntary effort of infraspinatus muscle was recorded and a standardized search for spontaneous electrical motor endplate activity at the trigger points was performed before and 28 days after BTXA or saline injection. RESULTS: BTXA reduced motor endplate activity and the interference pattern of EMG significantly but had no effect on either pain (spontaneous or referred) or pain thresholds compared with isotonic saline. CONCLUSIONS: The results do not support a specific antinociceptive and analgesic effect of botulinum toxin A.