Anaesthesia and juvenile Huntington's disease

Juvenile Huntington's Disease (JHD) is an involuntary movement disorder tha t 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 aspirat ion, possible associated autonomic neuropathy, poor respiratory function an d the avoidance of precipitating convulsions and clonic spasms. We describe the management of a 12-year-old girl with JHD scheduled for gastroscopy un der general anaesthesia necessitating the use of suxamethonium. We suggest an alternative mechanism for the delayed recovery seen in our patient and i n other adult case reports.