Successful Spinal Anesthesia in a Patient with Huntington’s Chorea

Abstract

To the Editor: Huntington’s chorea is a rare, autosomal, dominant hereditary disorder of the nervous system. Symptoms occur in the third and fourth decade of life and manifest as involuntary choreiform movements, ataxia, and progressive mental deterioration (1). Only a few case reports have been published describing the anesthetic management of patients with Huntington’s chorea (2–9). Patients are at greater risk for some intraoperative complications (e.g., regurgitation and pulmonary aspiration), poor respiratory function, prolonged response to succinylcholine and thiopental, increased sensitivity to midazolam, and postoperative shivering leading to rigid spasms (2–9). We present our successful experience using spinal anesthesia in a 44-yr-old woman with Huntington’s chorea. A 44-yr-old woman with a 15-yr history of Huntington’s chorea, pes planus, and hallux valgus was scheduled for elective surgery to correct the hallux valgus pathology. Results of a preoperative physical examination and standard laboratory tests were unremarkable, except for mild dysarthria, mild ataxia, and gross choreiform movements in all 4 extremities. We orally administered famotidine (40 mg) and diazepam (5 mg) 1 h before surgery. After routine ASA monitoring, we sedated the patient with IV midazolam (2.5 mg). Choreiform movements decreased slightly after sedation. We used a 25-gauge Quincke needle with 3.5 mL hyperbaric bupivacaine (0.5%) to perform spinal anesthesia with the patient in the lateral decubitus position. Anesthesia was successful on the first attempt. After 4 min, we turned the patient to a supine position. Spinal block reached the level of T7 at 10 min. While mild choreiform movements in the upper extremities remained throughout surgery, those of the lower extremities ceased totally for the duration of the spinal anesthetic. Optimal surgical conditions were provided by the spinal anesthetic for the 90-min duration of surgery. The patient’s perioperative course was uneventful. Approximately 2 h after surgery, her motor and sensory functioning returned to normal. The patient was discharged from the hospital on the third postoperative day. Although different anesthetic techniques have been recommended for patients with Huntington’s chorea, the best anesthetic technique remains elusive (2–9). Some practitioners recommend a completely IV anesthesia technique, one that avoids having the patient inhale potent drugs that can bring about postoperative shivering and precipitate generalized tonic spasms (9). Others have used inhaled anesthetics, such as halothane, isoflurane, and sevoflurane, without problems (6). Only one case of successful spinal anesthesia has been reported (7). Although proper positioning and prevention from trauma may be a problem during spinal anesthesia in patients with Huntington’s chorea, in appropriate surgical procedures spinal anesthesia may be effective, comfortable, and preferable to general anesthesia, yielding frequent success rates while preventing the possible disadvantages of general anesthesia. Asım Esen Pelin Karaaslan Rahmi Can Akgün Gülnaz Arslan Baskent University Ankara, Turkey [email protected]