piperidines and Neuromuscular-Diseases

Aspiration is a significant cause of anesthetic morbidity, occurring most commonly during the induction of anesthesia. For patients with a high likelihood of aspiration, rapid sequence intubation (RSI) techniques may minimize this risk by reducing the time between the loss of protective airway reflexes and the placement of a cuffed endotracheal tube. Although RSI frequently involves the administration of a neuromuscular-blocking agent (NMBA) such as succinylcholine or rocuronium, there are times when the administration of an NMBA is contraindicated or undesirable. We present an 11-year-old boy who presented with vomiting, papilledema, and a history concerning for an undiagnosed neuromuscular disorder. Deep sedation or anesthesia was required during an emergent lumbar puncture to evaluate his symptoms. Rapid sequence intubation was successfully performed with propofol and remifentanil without the use of an NMBA. We highlight the anesthetic considerations in such a clinical scenario and review the literature regarding the combination of remifentanil and propofol for RSI.

Topics: Anesthesia, Inhalation; Child; Contraindications; Deep Sedation; Emergencies; Headache; Humans; Hypnotics and Sedatives; Intracranial Hypertension; Intubation, Intratracheal; Laryngoscopy; Male; Methyl Ethers; Muscle Weakness; Neuromuscular Depolarizing Agents; Neuromuscular Diseases; Piperidines; Propofol; Remifentanil; Respiratory Aspiration of Gastric Contents; Risk; Sevoflurane; Spinal Puncture; Succinylcholine; Vision Disorders; Vomiting

Remifentanil belongs to opioid drugs, and its pharmacokinetic characteristics make it unique in this class of drugs and appropriate for use during intubation without neuromuscular blockage. This up-to-date review aims to summarize the findings of recent studies regarding remifentanil and intubation. Remifentanil combined either with propofol or with inhaled anesthetic agents has been proved to provide acceptable intubating conditions. Regarding children patients, remifentanil can be used safely, and as far as intubating conditions are concerned, its effectiveness is as excellent as with neuromuscular blockage. Strong evidence exists that illuminates the usefulness of the drug in cases of difficult airway as well as in neuromuscular diseases. Beyond all these favorable characteristics, anesthesiologists must be conscious with the use of remifentanil.

Topics: Airway Management; Analgesics, Opioid; Anesthetics, Inhalation; Child; Drug Therapy, Combination; Humans; Hypnotics and Sedatives; Intubation, Intratracheal; Neuromuscular Blocking Agents; Neuromuscular Diseases; Piperidines; Propofol; Remifentanil

Open muscle biopsies in children are generally performed under general anesthesia. Alternatively, deep sedation and analgesia may be required.. The aim of our study was to compare the Bispectral Index (BIS) and Comfort Score (CS) with respect to their clinical significance for sedation/analgesia in children undergoing open muscle biopsy.. Thirty pediatric patients subjected to open muscle biopsy for diagnosis of their underlying disease were prospectively enrolled. Sedation/analgesia was performed in all patients using remifentanil and propofol. The patients were simultaneously monitored using the CS and BIS.. All sedations and muscle biopsies were performed uneventfully. The CS and BIS were significantly correlated (R = 0.589; P < 0.01). Receiver operating characteristic (ROC) analysis revealed an area under the curve (AUC) of 0.918 with a maximum cut-off point of BIS 70.5 (sensitivity 0.9; specificity 0.785) for adequate sedation. Sensitivity of 100% was achieved at BIS 60. Accordingly, all patients with BIS ≤60 had CS within the target range of 10-14. The BIS showed substantial intra- and interindividual variability (30 points and 58 points, respectively) during sedation, whereas CS varied only within close ranges during sedation. In 25 patients, sedatives were reduced according to low BIS values (<60). No unintended anesthesia awareness was noted during the study period.. Bispectral Index provides an additional helpful tool to guide sedation/analgesia in minor surgical procedures in children. BIS values ≤60 correlated with sufficient depth of sedation and prevented unintended awareness. Additionally, BIS measurement allowed for distinct regulation of depth of sedation without prolonged sedation/analgesia due to unintended overdose.

Topics: Adolescent; Analgesia; Anesthetics, Intravenous; Area Under Curve; Biopsy; Child; Child, Preschool; Consciousness Monitors; Deep Sedation; Female; Humans; Infant; Intraoperative Awareness; Male; Monitoring, Physiologic; Muscle, Skeletal; Neuromuscular Diseases; Piperidines; Propofol; Prospective Studies; Remifentanil; ROC Curve

Evidence indicates that neuroinflammation contributes to motor neuron degeneration in amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disease leading to progressive muscular paralysis. However, it remains elusive whether inflammatory cells can interact with degenerating distal motor axons, influencing the progressive denervation of neuromuscular junctions (NMJs). By analyzing the muscle extensor digitorum longus (EDL) following paralysis onset in the SOD1G93A rat model, we have observed a massive infiltration and degranulation of mast cells, starting after paralysis onset and correlating with progressive NMJ denervation. Remarkably, mast cells accumulated around degenerating motor axons and NMJs, and were also associated with macrophages. Mast cell accumulation and degranulation in paralytic EDL muscle was prevented by systemic treatment over 15 days with masitinib, a tyrosine kinase inhibitor currently in clinical trials for ALS exhibiting pharmacological activity affecting mast cells and microglia. Masitinib-induced mast cell reduction resulted in a 35% decrease in NMJ denervation and reduced motor deficits as compared with vehicle-treated rats. Masitinib also normalized macrophage infiltration, as well as regressive changes in Schwann cells and capillary networks observed in advanced paralysis. These findings provide evidence for mast cell contribution to distal axonopathy and paralysis progression in ALS, a mechanism that can be therapeutically targeted by masitinib.

Topics: Amyotrophic Lateral Sclerosis; Animals; Axons; Benzamides; Disease Models, Animal; Male; Mast Cells; Muscle, Skeletal; Neuromuscular Diseases; Neuromuscular Junction; Piperidines; Pyridines; Rats; Thiazoles

The present study attempts to assess the comparative effects of Bacopa monniera, (40 mg/kg body weight) and donepezil (2.5 mg/kg b. wt) on aluminum (100 mg / kg b. wt. of AlCl3) mediated oxidative damage in the cerebellum of aged rats (24 months) along with the associated dysfunctioning of neuromuscular coordination and motor activity. A significant decrease in the activities of antioxidant enzymes and increased total reacting oxygen species, lipid and protein peroxidation products observed in aluminum exposed rats. We observed that treatment with B. monniera extract restored the altered antioxidant enzyme activities more, when compared with donepezil. However, acetylcholinesterase showed similar effect both in donepezil and B. monniera treated groups. The content of aluminum was increased in all experimental groups, however, iron content was found increased in all groups except the B. monniera treated groups. Moreover, aluminum treated groups of rats exhibited significant changes in behavioral profiles but these changes were in both B. monniera and donepezil treated groups. The light microscopic and ultrastructural studies revealed damaged Purkinje's neurons and altered granular cell layer along with the increased accumulation of lipofuscin granules in aluminum treated animals. These changes were quite less pronounced in B. monniera group than that of donepezil and this may be due to the reduction of excess iron content by B. monniera. On the basis of our results it may be concluded that Al may be linked with cerebellar degeneration and neuromuscular disorders while Bacopa monniera extract helps in reversing these changes.

Topics: Aging; Aluminum; Animals; Bacopa; Cerebellum; Donepezil; Indans; Male; Motor Activity; Neuromuscular Diseases; Nootropic Agents; Oxidative Stress; Piperidines; Plant Extracts; Rats; Rats, Wistar

Intraoperative monitoring of the spinal cord via cortical somatosensory-evoked potentials (SSEP) is a routine during spinal surgery. However, especially in neuromuscular scoliosis, the reliability of cortical SSEP has been questioned. Therefore, we compared the feasibility of cortical SSEP in idiopathic and neuromuscular scoliosis using anaesthetics known to have only minimal effect on SSEP recordings.. Total intravenous anaesthesia with propofol and remifentanil as continuous infusion was standardized for all the patients. Median and tibial nerve cortical SSEP were monitored in 54 patients who underwent surgery for spinal deformity. Twenty-seven had idiopathic scoliosis and 27 had neuromuscular scoliosis. The portion of reproducible results and intraoperative changes were compared between the groups.. In both groups, cortical SSEP could be monitored with sufficient reliability. Only in two patients with idiopathic and four patients with neuromuscular scoliosis no reproducible traces could be obtained. The amplitudes in patients with neuromuscular scoliosis were lower than in those with idiopathic scoliosis, but not statistically significant. There were no postoperative neurological deficits. The number of false positive and true positive did not differ between the groups.. Assessment of cortical SSEP during spine surgery was equally effective and reliable in patients with neuromuscular scoliosis and in patients with idiopathic scoliosis, possibly as a result of propofol-remifentanil anaesthesia.

Topics: Adolescent; Adult; Anesthetics, Combined; Anesthetics, Intravenous; Child; Child, Preschool; Diagnostic Errors; Evoked Potentials, Somatosensory; Feasibility Studies; Female; Humans; Male; Monitoring, Intraoperative; Neuromuscular Diseases; Piperidines; Propofol; Prospective Studies; Remifentanil; Scoliosis; Spinal Cord

Perhexiline maleate is an amphiphilic molecule. Along with many other drugs it is responsible for experimental and, in some instances, clinical lipidoses. Sphingomyelinase deficiency has been evidenced in cell cultures incubated with perhexiline maleate. We describe the occurrence of a similar defect in a patient. The disturbances in the phospholipid turnover which are responsible for the thesaurismosis may originate in the sphingomyelinase deficiency.

Topics: Cerebellar Ataxia; Female; Humans; Lipidoses; Middle Aged; Neuromuscular Diseases; Perhexiline; Peripheral Nerves; Peripheral Nervous System Diseases; Piperidines; Schwann Cells

Topics: Angina Pectoris; Body Weight; Humans; Liver Diseases; Neuromuscular Diseases; Perhexiline; Piperidines

Topics: Aged; Diabetic Angiopathies; Female; Follow-Up Studies; Humans; Male; Muscles; Neuromuscular Diseases; Perhexiline; Peripheral Nerves; Piperidines

Topics: Bronchial Neoplasms; Diagnostic Errors; Humans; Male; Middle Aged; Neuromuscular Diseases; Perhexiline; Piperidines