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Repeat anesthetic management of a patient with Huntington's chorea

Chandigarh, India

To the Editor:

We present different approaches to the anesthetic management of a patient with Huntington's chorea, for which experience is limited.^1–6

A 57-yr-old female with Huntington's chorea had elective surgery on three successive occasions. She had gross choreiform movements, ataxia and received haloperidol and lorazepam po. Preoperative laboratory investigations were unremarkable. She was premedicated with ranitidine, lorazepam and haloperidol. On the first occasion, anesthesia was induced with morphine and midazolam iv. On the second occasion, anesthesia was induced with morphine, propofol iv and atracurium was used to facilitate intubation. On the third occasion, anesthesia was induced with fentanyl, propofol iv and atracurium was used to facilitate intubation. Anesthesia was maintained with 66% N₂O in O₂ with 1.5% isoflurane on the first and second occasions and 1.5% sevoflurane on the third. The patient was allowed to breathe spontaneously by face mask. Cognitive functions returned five minutes, two minutes, and immediately after reversal respectively.

The best anesthetic technique is yet to be established for these patients. The unpredictability of thiopentone¹ can be circumvented by using induction agents like midazolam² and propofol.³ Prolonged apnea following suxamethonium⁴ can be averted by using rocuronium when rapid sequence induction becomes necessary. Further, the incidence of postoperative shivering can be minimized by use of isoflurane and sevoflurane⁵ in place of halothane. The low solubility coefficients of these agents allow early emergence, intact cognitive function and early return of protective airway reflexes; thus the risk of pulmonary aspiration is minimized. For lower abdominal and perineal surgery spinal anesthesia⁶ is a tangible alternative although achieving proper position and avoidance of trauma remains difficult in these patients.

References

1 Davies DD. Abnormal response to anaesthesia in a case of Huntington's chorea. A case report. Br J Anaesth 1966; 38: 490–1.[Abstract/Free Full Text]

2 Gualandi W, Bonfati G. A case of prolonged apnea in Huntington's chorea. Acta Anaesthesiol (Padova) 1968; 19: 235–8.

3 Rodrigo MRC. Huntington's chorea: midazolam, a suitable induction agent? Br J Anaesth 1987; 59: 388–9.[Free Full Text]

4 Kaufman MA, Erb T. Propofol for patients with Huntington's chorea? Anaesthesia 1990; 45: 889–90.

5 Nagele P, Hammerle AF. Sevoflurane and mivacurium in a patient with Huntington's chorea. Br J Anaesth 2000; 85: 320–1.[Abstract/Free Full Text]

6 Fernandez IG, Sanchez MP, Ugalde AJ, Hernandez CM. Spinal anaesthesia in a patient with Huntington's chorea. Anaesthesia 1997; 52: 391.

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