Corpus Callosum In Patient Research Articles

Anaesthesia for a biopsy of corpus callosum in patient with a recent intra-operative anaphylaxis to an unknown anaesthetic allergen: a case report

BackgroundAnaphylaxis in general anaesthesia occurs with a frequency of 1:5000-1:20000. This clinical summary reports on the use of an effective risk management strategy employing second line anaesthesia agents and alternative endotracheal intubation tools in a patient with a recent history of an intra-operative anaphylaxis to an unknown anaesthetic agent.Case presentationA 71-year-old male presented for a repeat biopsy of corpus callosum 4 days following the cancellation of the procedure for a presumed anaphylactic reaction to an unknown anaesthetic agent.During the repeat care episode, the decision was made to proceed based on the urgent need for tissue diagnosis to facilitate further treatment and lack of feasibility for more definitive identification of the causative agent(s). A consideration was made of the optimum ways to manage and mitigate risk in this setting.The airway was managed using flexible endoscopic intubation in a spontaneously ventilating awake patient. Continuous remifentanil infusion was maintained throughout the case. Anaesthesia was maintained with sevoflurane at less than one MAC, with an uneventful completion of the biopsy of corpus callosum. All of the anaesthetic agents used during the prior care episode, with the exception of remifentanil, were avoided.ConclusionIn cases of an anaphylaxis to an unknown anaesthetic allergen, anaesthetic strategy consists of careful risk mitigation and deployment of second agent approaches. Awake flexible endoscopic intubation and remifentanil infusion are viable alternatives to standard induction techniques.

Early Myoclonic Encephalopathy and Nonketotic Hyperglycinemia

Early myoclonic encephalopathy is an epileptic syndrome with different etiologies. Nonketotic hyperglycinemia is one cause. We describe two cases of early myoclonic encephalopathy, secondary to nonketotic hyperglycinemia, with fatal evolution in the neonatal period. These two cases may better clarify clinical findings that can be associated with impairment of glycine metabolism. Distinguishing features include agenesis of the corpus callosum in patient 1, and weight loss exceeding 10%, associated with metabolic acidosis, in patient 2. The burst-suppression electroencephalography pattern is relatively common in neonatal encephalopathies, and is frequently associated with seizures. Nonketotic hyperglycinemia is an inborn error of metabolism caused by mutations in genes encoding protein in the mitochondrial glycine cleavage system. The neonatal form is a severe, frequently lethal neurologic disease. When associated with electro-clinical features, progressive lethargy and hypotonia occur in the first days of life, progressing to apnea and often death. Prospective treatment with oral sodium benzoate, the N-methyl-d-aspartate receptor antagonist ketamine, and dextromethorphan can favorably modify the early neonatal course of severe nonketotic hyperglycinemia, but does not prevent poor long-term outcomes.