Cases reported "Muscular Atrophy, Spinal"

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1/5. Use of rapacuronium in a child with spinal muscular atrophy.

    We report the case of an 18-month-old girl with spinal muscular atrophy (SMA) that received 1 mg x kg(-1) rapacuronium for laryngospasm during induction of anaesthesia. Within 15 min, we observed some diaphragmatic recovery and, after emergence from anaesthesia, the child demonstrated adequate respiratory efforts. However, the child showed diminished strength of the upper extremity muscles. Since the preoperative workup had revealed bulbar symptoms and laryngeal function could not be easily assessed, the patient was kept intubated until upper extremity strength had returned to preoperative levels. Small doses of midazolam had been given to reduce the patient's anxiety but the patient was extubated within 5 h without any complications. Train of four (TOF) monitoring of the right adductor pollicis muscle, performed during anaesthetic recovery, was equivocal. In SMA, muscle groups are differentially affected so that TOF responses may be inconclusive and not reflect the state of the upper airway muscles. To our knowledge, this is the first report of use of a nondepolarizing neuromuscular blocking agent in a child with SMA.
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ranking = 1
keywords = anaesthesia
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2/5. Epidural anaesthesia in a child with possible spinal muscular atrophy.

    Spinal muscular atrophy (SMA) is a rare lower motor neurone disease in which anaesthetic management is often difficult as a result of muscle weakness and hypersensitivity to neuromuscular blocking agents. Neuraxial anaesthesia is controversial in these patients; however, some cases have been reported in which neuraxial anaesthesia has been used without neurological sequelae. We describe a 7-year-old patient with possible SMA scheduled for a Grice-arthrodesis. Because of previous prolonged postoperative drowsiness and poor oral intake, we decided to use an epidural technique with sevoflurane sedation and spontaneous ventilation to avoid the use of muscle relaxants and systemic opioids and consequently admission to the intensive care unit. After 3 days, the epidural analgesia was stopped and the patient regained her preoperative motor function within 5 h. Despite the controversy surrounding the use of neuraxial techniques in neuromuscular disease, we found no well-founded basis for this in patients with SMA in the literature.
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ranking = 3
keywords = anaesthesia
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3/5. Anaesthesia for caesarean section in a patient with spinal muscular atrophy.

    This report describes a 33-year-old primigravid woman with spinal muscular atrophy Type III (Kugelberg-Welander syndrome). Elective caesarean section was performed at 38 weeks gestation under spinal anaesthesia. The implications of spinal muscular atrophy for anaesthesia for caesarean section are described.
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ranking = 1
keywords = anaesthesia
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4/5. Use of epidural anaesthesia for surgery in a patient with Kennedy's disease.

    Use of neuraxial block in a patient with motor neuron disease is controversial. We describe the anaesthetic management by epidural anaesthesia of a patient with Kennedy's disease, a rare lower motor neuron disease characterized by progressive weakness and wasting of limbs and bulbar muscles. The perioperative course was uneventful, and there was no exacerbation of neurologic signs or symptoms. We suggest that a patient with Kennedy's disease may be successfully managed by epidural anaesthesia for surgical internal urethrotomy.
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ranking = 3
keywords = anaesthesia
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5/5. Anaesthesia for caesarean section in spinal muscular atrophy type III.

    We describe the conduct of general anaesthesia for a patient with spinal muscular atrophy Type III (Kugelberg-Welander disease) undergoing elective caesarean section. Apart from a delayed return of skeletal muscle power following non-depolarising neuromuscular blockade the procedure was uneventful. We found no previously published reports of general anaesthesia for caesarean section in this condition in the English language literature. We review the available literature and discuss the potential anaesthetic problems in the management of obstetric patients with this degenerative neuromuscular disorder.
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ranking = 1
keywords = anaesthesia
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