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1/4. Epidural analgesia for labour in a patient with charcot-marie-tooth disease.

    PURPOSE: We report a case of a parturient with peroneal muscular atrophy-charcot-marie-tooth disease type 1 (CMT)-who received epidural analgesia for labour. The effects of pregnancy and labour on the course of CMT are reviewed, together with the current literature on the provision of epidural and general anaesthesia in this disease. CLINICAL FEATURES: A 26-yr-old primipara with CMT was provided with epidural analgesia for labour. She experienced good pain control and suffered no untoward neurological sequelae. CONCLUSION: Epidural analgesia, after thorough discussion with the patient, may be offered to parturients with CMT.
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ranking = 1
keywords = anaesthesia
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2/4. Response to atracurium and mivacurium in a patient with charcot-marie-tooth disease.

    PURPOSE: We studied the neuromuscular effects of both atracurium and mivacurium in a patient with charcot-marie-tooth disease (CMTD) during nitrous oxide-oxygen-alfentanil-propofol anaesthesia. neuromuscular blockade was monitored electromyographically. Train-of-four stimulation (2 Hz @ 20 sec intervals) was delivered to the ulnar nerve throughout the period of observation. CLINICAL FEATURES: A 17-yr-old man with the diagnosis of CMTD was presented twice for two different orthopaedic surgical procedures. The CMTD had been diagnosed since childhood. Neurological examination revealed distal wasting of the upper and lower limbs, generalised absence of reflexes and decreased sensation in a stocking distribution. In both anaesthetics, induction was carried out with alfentanil and propofol, and anaesthesia was maintained with nitrous oxide in oxygen, alfentanil and propofol infusion. The patient demonstrated a normal response to both atracurium and mivacurium. Onset time and the maximum block attained after atracurium and mivacurium were 240 and 210 sec, and 97% and 99% inhibition of T1 (the first twitch of TOF stimulation), respectively. Recovery of T1 to 10% of the control value occurred 30 and 11.5 min after the administration of atracurium and mivacurium, respectively. The patient made uneventful recoveries after both anaesthetics. CONCLUSION: There was no evidence of prolonged response to atracurium and mivacurium in our patient with CMTD.
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ranking = 2
keywords = anaesthesia
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3/4. Anaesthesia for caesarean section in a patient with charcot-marie-tooth disease.

    We describe the management of a 25-year-old primigravida with severe respiratory insufficiency secondary to charcot-marie-tooth disease type I scheduled for Caesarean section. Incremental subarachnoid anaesthesia via a microcatheter was utilised. Mother and baby made an uneventful recovery and were discharged home on the tenth postoperative day.
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ranking = 1
keywords = anaesthesia
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4/4. Total intravenous anaesthesia in charcot-marie-tooth disease. Case report.

    A Total Intravenous Anaesthesia (TIVA) protocol in a case of hereditary motor-sensory neuropathy charcot-marie-tooth disease in three consecutive orthopaedic surgical procedures is described. history, physical examination and sural nerve fascicular biopsy findings are reported. Problems related to the perioperative anaesthesiological management in peripheral neuropathies are discussed. propofol and fentanyl without muscle relaxants, proved to be a safe technique, fast at reaching the level of surgical anaesthesia required, manageable for maintenance and rapidly reversible.
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ranking = 5
keywords = anaesthesia
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