Cases reported "Huntington Disease"

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1/2. Anaesthesia and juvenile Huntington's disease.

    Juvenile Huntington's disease (JHD) is an involuntary movement disorder that comprises both neurological and psychiatric symptoms. Whilst it has many similarities to Huntington's disease, it is regarded as a separate clinical entity. The anaesthetic plan should be based on careful assessment of the important issues, including the risk of regurgitation and pulmonary aspiration, possible associated autonomic neuropathy, poor respiratory function and the avoidance of precipitating convulsions and clonic spasms. We describe the management of a 12-year-old girl with JHD scheduled for gastroscopy under general anaesthesia necessitating the use of suxamethonium. We suggest an alternative mechanism for the delayed recovery seen in our patient and in other adult case reports.
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ranking = 1
keywords = anaesthesia
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2/2. Sevoflurane and mivacurium in a patient with Huntington's chorea.

    There is little experience of anaesthesia for patients with Huntington's chorea. These patients have an increased risk of intraoperative complications such as pulmonary aspiration. We present the successful anaesthetic management of a 17-yr-old patient suffering from Huntington's chorea requiring urgent appendectomy. After rapid-sequence induction with thiopental 400 mg and succinylcholine 100 mg, anaesthesia was maintained with sevoflurane. For maintenance of neuromuscular blockade mivacurium 10 mg was administered and repeated 15 min later. Except for a short episode of postoperative shivering, the perioperative course was uneventful. Sevoflurane and mivacurium were used safely and effectively in this patient.
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ranking = 2
keywords = anaesthesia
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