Predictors of prognosis in type 1 myotonic dystrophy (DM1): longitudinal 18-years experience from a single center.

The aim of the study was to identify possible predictors of neurological worsening and need of non-invasive ventilation (NIV) in individuals affected by myotonic dystrophy type 1 (DM1), the most common form of adult-onset muscular dystrophy. METHODS: A retrospective observational cohort study was undertaken. Thirty-three patients with genetic diagnosis of DM1 were followed at our Neuromuscular unit in Modena. Abnormal trinucleotide repeat (CTG) expansion of dystrophy protein kinase gene (MDPK) on chromosome 19q 13.3 was the prerequisite for inclusion. The number of CTG repeats was determined. All the participants were older than 14 at the time of enrolment, therefore they could be included into the juvenile or adult form of the disease. Participants were neurologically evaluated every 6-8 months up to 18 years. Neurological impairment was assessed by Muscular Impairment Rating (MIRS), Medical Research Council (MRC), and modified Rankin (mRS) scales. The independent variables considered for prognosis were age at first evaluation, duration of the disease, CTG repeat number, gender, and presence of cardiac and vascular morbidities.Male patients were 51.5% and female patients 48.5%. Sixteen patients were younger than the mean age of 30.1 years, while the remaining 17 were up to 65. Twelve subjects (36.4%) underwent NIV before the end of follow-up. Muscle force and disability scores showed statistically significant deterioration (p < 0.001) during follow-up. The worsening was significantly higher among patients carrying higher number of CTG repeats and of younger age. The presence of cardio-vascular involvement has significant impact on neurological and respiratory progression.Neurological worsening is predicted by CTG expansion size, young age and presence of cardio-vascular morbidities.

Neuromuscular monitoring during modified rapid sequence induction: A comparison of TOF-Cuff® and TOF-Scan®.

BACKGROUND: Acceleromyometry is the clinical standard for quantitative neuromuscular monitoring, mostly using the stimulation pattern train-of-four (TOF). TOF-Cuff®, a recently introduced neuromuscular monitor with stimulating electrodes integrated within a blood pressure cuff, assesses the muscular response in the upper arm. METHODS: The time from administration of a neuromuscular blocking agent to TOF-ratio 0% during modified rapid sequence induction was compared between TOF-Cuff® and acceleromyometry (TOF-Scan®). Included were 26 adults with body mass index <35 kg/m2. TOF-Scan® and TOF-Cuff® were simultaneously fitted on patients' opposite arms. The mean difference to TOF-ratio 0% was compared using the one sample t-test (p < 0.05) and Bland-Altman plots. RESULTS: After anesthesia induction, atracurium 0.9 mg/kg (±0.08) i.v. was administered. The mean time to TOF ratio 0% for TOF-Scan® was 140.4 s (±34.3), and 132.7 s (±32.5) for TOF-Cuff®, with a mean difference of 5.4 (95% CI: -9.9 to 20.7, p = 0.472). The maximum difference between the two modalities was 135 s when the TOF-Cuff® was faster and 60 s when the TOF-Scan® was faster. CONCLUSIONS: No statistically significant systematic difference was found between TOF-Scan® and TOF-Cuff®. However, there was high variability and wide limits of agreement. The two devices cannot be used interchangeably.

Neuromuscular Performance and Hormonal Profile During Military Training and Subsequent Recovery Period.

INTRODUCTION: Military training loads may induce different physiological responses in garrison and field training and only a little is known about how short-time recovery, lasting a few days, affects neuromuscular fitness and hormonal profile. This study aimed to investigate the effects of garrison and field military service on neuromuscular performance and hormonal profile and to evaluate the effects of a 3-day recovery on those factors. METHODS: Twenty healthy male soldiers (20 ± 1 years) participated in the study, which consisted of 4 days of garrison training [days (D) 1-4] and 7 days of military field training (Days 5-12) followed by a 3-day recovery period (Day 15). Serum hormone concentrations [testosterone (TES), cortisol (COR), sex-hormone binding globulin (SHBG), free thyroxine (T4)] were assessed at D1, D5, D8-12, and D15. Handgrip strength was measured in 10 participants at D1, D5, D8, D12, and D15. Maximal isometric force, electromyography, and rate of force development (RFD) of the knee extensors and arm flexors were also measured at D5, D12, and D15. RESULTS: The maximal force of both the arm flexors and knee extensors was not affected by the garrison or field training, whereas the RFD of the knee extensors was decreased during the field training (D5: 383 ± 130 vs. D12: 321 ± 120 N/s, p < 0.05). In addition, handgrip strength was mostly no affected, although a significant difference was observed between D8 and D12 (531 ± 53 vs. 507 ± 43 N, p < 0.05) during the field training. TES decreased already during the garrison training (D1: 18.2 ± 3.9 vs. D5: 16.2 ± 4.0 nmol/L, p < 0.05) and decreased further during the field training compared to baseline (D8: 10.2 ± 3.6 - D11: 11.4 ± 5.4 nmol/L, p < 0.05) exceeding the lowest concentration in the end of the field training (D12: 7.1 ± 4.1 nmol/L, p < 0.05). Similar changes were observed in free TES (D1: 72.2 ± 31.4 vs. D12: 35.1 ± 21.5 nmol/L, p < 0.001). The TES concentration recovered back to the baseline level and free TES increased after the recovery period compared with the baseline values (D15: 19.9 ± 5.3 nmol/L, D15: 99.7 ± 41.1 nmol/L, respectively). No changes were observed in the COR or SHBG concentrations during the garrison period. COR was decreased in the end of the field training (D12: 388 ± 109 nmol/L) compared with baseline (D1: 536 ± 113 nmol/L) (p < 0.05-0.001) but recovered back to the baseline levels after the recovery period (D15: 495 ± 58 nmol/L), whereas SHBG linearly increased towards the end of the field training (p < 0.05-0.001). CONCLUSIONS: The present findings demonstrate that neuromuscular performance can be relatively well maintained during short-term garrison and field training even when a clear decrease in hormonal profile is evident. In addition, hormonal responses during field training seem to be greater compared to garrison training, however, the recovery of 3-day in free-living conditions seems to be sufficient for hormonal recovery. Therefore, a short-term recovery period lasting few days after the military field training may be required to maintain operational readiness after the field training.

Comparison of the TOFscan and the TOF-Watch SX during Recovery of Neuromuscular Function.

WHAT WE ALREADY KNOW ABOUT THIS TOPIC: WHAT THIS ARTICLE TELLS US THAT IS NEW: BACKGROUND:: Quantitative neuromuscular monitoring is required to ensure neuromuscular function has recovered completely at the time of tracheal extubation. The TOFscan (Drager Technologies, Canada) is a new three-dimensional acceleromyography device that measures movement of the thumb in multiple planes. The aim of this observational investigation was to assess the agreement between nonnormalized and normalized train-of-four values obtained with the TOF-Watch SX (Organon, Ireland) and those obtained with the TOFscan during recovery from neuromuscular blockade. METHODS: Twenty-five patients were administered rocuronium, and spontaneous recovery of neuromuscular blockade was allowed to occur. The TOFscan and TOF-Watch SX devices were applied to opposite arms. A preload was applied to the TOF-Watch SX, and calibration was performed before rocuronium administration. Both devices were activated, and train-of-four values were obtained every 15 s. Modified Bland-Altman analyses were conducted to compare train-of-four ratios measured with the TOFscan to those measured with the TOF-Watch SX (when train-of-four thresholds of 0.2 to 1.0 were achieved). RESULTS: Bias and 95% limits of agreement between the TOF-Watch SX and the TOFscan at nonnormalized train-of-four ratios between 0.2 and 1.0 were 0.021 and -0.100 to 0.141, respectively. When train-of-four measures with the TOF-Watch SX were normalized, bias and 95% limits of agreement between the TOF-Watch SX and the TOFscan at ratios between 0.2 and 1.0 were 0.015 and -0.097 to 0.126, respectively. CONCLUSIONS: Good agreement between the TOF-Watch SX with calibration and preload application and the uncalibrated TOFscan was observed throughout all stages of neuromuscular recovery.

Objective neuromuscular monitoring of neuromuscular blockade in Denmark: an online-based survey of current practice.

BACKGROUND: Neuromuscular blocking agents are commonly used during general anaesthesia but can lead to postoperative residual neuromuscular blockade and associated morbidity. With appropriate objective neuromuscular monitoring (objNMM) residual blockade can be avoided. In this survey, we investigated the use of objNMM in Denmark. METHODS: We conducted an anonymous Internet-based survey distributed through e-mails to Danish public anaesthesia departments. The survey consisted of 15-17 short questions regarding the use of objNMM. RESULTS: A total of 653 (27%) anaesthetists from 90% of the hospitals answered the questionnaire. ObjNMM was always used by 58% of the anaesthetists and 86% used objNMM at least 75% of the times. Despite the frequent use, 75% of the anaesthetists experienced difficulties with objNMM in at least 25% of the cases. The likelihood of using objNMM was higher among nurse anaesthetists vs. anaesthesiologists (odds ratio (OR) 2.24 [95% confidence interval (CI): 1.62-3.08]), if the department had an employee with special interest in objNMM (OR 1.66 [95% CI: 1.12-2.47]), if the anaesthetist had < 5 years of experience (OR 1.88 [95% CI: 1.29-2.73]), or if experiencing difficulties with objNMM < 25% of the cases (OR 1.60 [95% CI: 1.00-2.57]). CONCLUSION: In this survey, Danish anaesthetists frequently, in an international perspective, use objNMM, but the use is often associated with technical difficulties.

A national survey of neurological monitoring practice after obstetric regional anaesthesia in the UK.

Neuraxial anaesthesia is widely used in obstetrics and neurological complications are rare. However, when they occur, subsequent investigation and management are time-critical and correlate with the extent of neurological recovery. The Third National Audit Project recommended the implementation of guidelines in obstetric epidural management, including advice on monitoring for early signs of problems and acting upon concerns. However, no national guideline exists for postoperative management in the obstetric population. We conducted a national survey of monitoring after obstetric neuraxial blockade and the management of an abnormally prolonged block. We received responses from 112/189 (59.3%) obstetric anaesthetic leads invited to participate. We determined that post-neuraxial blockade monitoring in the UK is highly variable: only 63/112 (56.3%) respondents' units had a monitoring policy in place, although most of these did not undertake formal neurological monitoring, and a range of different monitoring methods and schedules were employed. In 12/63 (19%) local policies, the first review of neurology was performed at the standard postoperative visit the following day, and 66/112 (58.9%) units had no protocol in place to address emergency management of abnormally prolonged neuraxial blockade. Where a policy was in place, the initial recommended action and the type of imaging used were variable.

Sugammadex and neostigmine dose-finding study for reversal of residual neuromuscular block at a train-of-four ratio of 0.2 (SUNDRO20)†,.

BACKGROUND: The aim of this dose-finding study was to evaluate the dose-response relationship of sugammadex and neostigmine to reverse a commonly observed level of incomplete recovery from rocuronium-induced neuromuscular block, that is, a train-of-four ratio (TOFR) ≥0.2. METHODS: Ninety-nine anaesthetized patients received rocuronium 0.6 mg kg(-1) i.v. for tracheal intubation and, if necessary, incremental doses of 0.1-0.2 mg kg(-1). Neuromuscular monitoring was performed by calibrated electromyography. Once the TOFR recovered to 0.2, patients were randomized to receive sugammadex (0.25, 0.5, 0.75, 1.0, or 1.25 mg kg(-1) i.v.), neostigmine (10, 25, 40, 55, or 70 µg kg(-1) i.v.), or saline (n=9 per group). Primary and secondary end points were the doses necessary to restore neuromuscular function to a TOFR≥0.9 with an upper limit of 5 and 10 min for 95% of patients, respectively. RESULTS: Neostigmine was not able to fulfil the end points. Based on the best-fitting model, the sugammadex dose estimation for recovery to a TOFR≥0.9 for 95% of patients within 5 and 10 min was 0.49 and 0.26 mg kg(-1), respectively. CONCLUSION: A residual neuromuscular block of a TOFR of 0.2 cannot be reversed reliably with neostigmine within 10 min. In the conditions studied, substantially lower doses of sugammadex than the approved dose of 2.0 mg kg(-1) may be sufficient to reverse residual rocuronium-induced neuromuscular block at a recovery of TOFR≥0.2. CLINICAL TRIAL REGISTRATION: NCT01006720.

Severity of Myasthenia Gravis Influences the Relationship between Train-of-four Ratio and Twitch Tension and Run-down of Rat Endplate Potentials.

BACKGROUND: Train-of-four ratio (TOFR) is often used to evaluate muscle relaxation caused by neuromuscular-blocking agents (NMBAs). However, it is unknown whether TOFR reliably correlates with the first twitch tension (T1) in patients with myasthenia gravis (MG). By using rat models of experimental autoimmune MG (EAMG), the authors verified the hypothesis that the severity of MG influences the relationship between TOFR and T1. METHODS: EAMG rats were divided into sham, moderate MG, and severe MG groups. Isometric twitch tension of the hemidiaphragm was elicited by phrenic nerve stimulation with and without use of the NMBA rocuronium to measure TOFR and T1, and run-down of endplate potentials was estimated in the three groups. Changes around the neuromuscular junction in EAMG rats were investigated by observation of electron micrographs. RESULTS: With similar attenuation of T1, TOFR was significantly (n = 6) different among the three groups in the presence of 50% inhibitory concentrations of rocuronium (IC50). Run-down in the sham group was significantly (n = 8) greater with exposure to IC50, whereas that in the severe MG group was statistically insignificant. Width of the primary synaptic cleft in the severe MG group was significantly (n = 80) greater than that in the other groups. CONCLUSIONS: Severity of MG influences the relationship between TOFR and T1, together with changes in run-down of endplate potentials and those around the neuromuscular junction in rats. TOFR may, therefore, not be an accurate indicator of recovery from NMBAs in MG patients.

[Questionnaire on the anaesthesiology treatment of patients subjected to posterior fossa neurosurgery]. / Encuesta sobre el tratamiento anestesiológico de los pacientes sometidos a neurocirugía de fosa posterior.

OBJECTIVES: To find out, by means of a questionnaire, the procedures used by Spanish anaesthetists in peri-operative management of patients subjected to neurosurgery of the posterior cranial fossa. MATERIAL AND METHODS: A closed-question type questionnaire was sent to Anaesthesiology Departments with a Neurosurgery Department on the participation of anaesthetists in the peri-operative treatment of patients subjected posterior fossa surgery. RESULTS: The questionnaire was completed by 42 (57.5%) of the 73 national public hospitals with a Neurosurgery Department. The posterior fossa surgery was performed in the sitting position in 36 hospitals, although it was less frequently used than the lateral decubitus or prone decubitus position. There was little specific neurological monitoring, as well as little use of precordial and/or transcranial Doppler for detecting vascular air embolism. Nitrous oxide was used in less than 10% of the centres, and 15% avoided neuromuscular block when neurophysiological monitoring was used during the surgery. Cardiovascular problems were mentioned as being the most frequent in 29% of the centres, while in the post-operative period the most common complications were, cranial nerve déficit, airway oedema (23%), and post-operative vomiting (47%). CONCLUSIONS: The results obtained from the questionnaire showed that the sitting position was less used than the prone position in posterior fossa surgery, and that neurophysiological monitoring is during surgery is hardly used.