Huntington's chorea (HC) 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. Only a few case reports have been published describing the anesthetic management of patients with HC. 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. All these factors make an ideal anesthesiological management necessary for this group of patients to guarantee an absolute airway protection during all the anesthesia and a fast and safe recovery. To the best of our knowledge, epidural anesthesia in HC patients has not been reported in the literature. Herein, we present our successful experience using epidural anesthesia in a HC patient.
1. Bloch M, Adam S, Fuller A, Kremer B, Welch JP, Wiggins S, et
al. Diagnosis of Huntington disease: a model for the stages of
psychological response based on experience of a predictive
testing program. Am J Med Genet 1993;47(3):368-74
2. Rodrigo MR. Huntingtons chorea: midazolam, a suitable
induction agent? Br J Anaesth 1987;59(3):388-9
3. Kaufman MA, Erb T. Propofol for patients with Huntingtons
chorea? Anaesthesia 1990;45(10):889-90
4. Nagele P, Hammerle AF. Sevoflurane and mivacurium in a
patient with Huntingtons chorea. Br J Anaesth 2000;85(2):320-
1
5. Fernandez IG, Sanchez MP, Ugalde AJ, Hernandez CM. Spinal
anaesthesia in a patient with Huntingtons chorea. Anaesthesia
1997;52(4):391
6. Gaubatz CL, Wehner RJ. Anesthetic considerations for the
patient with Huntingtons disease. AANA J 1992;60(1):41-4
7. MacPherson P, Harper I, MacDonald I. Propofol and
remifentanil total intravenous anesthesia for a patient with
Huntington disease. J Clin Anesth 2004;16(7):537-8
8. Gilli E, Bartoloni A, Fiocca F, Dall'Antonia F, Carluccio S
Anaesthetic management in a case of Huntington's chorea
Minerva Anestesiol 2006;72(9):757-62
9. Stoelting RK, Dierdorf SF, Mccammon RL. Anesthesia and coexisting
disease. New York: Churchill Livingstone 1988:307
10. Qin ZH, Wang J, Gu ZL. Development of novel therapies for
Huntingtons disease: hope and challenge. Acta Pharmacol Sin
2005;26(2):129-42
Huntington koresi (HK) nadir görülen otozomal dominant geçiş gösteren sinir sistemi bozukluğudur. Semptomları hayatın 3. ve 4. dekatlarında istemsiz koreiform hareketleri, ataksi ve ilerleyici mental bozulma olarak gösterir. HK tanılı olgularda anestezi yönetimi ile ilgili sadece birkaç olgu sunumu yayınlanmıştır. Hastalar bazı intraoperatif komplikasyonlar (regürjitasyon ve pulmoner aspirasyon gibi), zayıf respiratuar fonksiyon, uzamış suksinilkolin ve tiyopental cevabı, midazolama karşı yükselmiş sensitivite, ve rijit spazmlara yol açan titremeler gibi postoperatif komplikasyonlar açısından yüksek risk altındadırlar.Tüm bunlar güvenli geri dönüş ve anestezi sırasında güvenli havayolu sağlamak için ideal anestezi yönetimi gerektirmektedir. Bildiğimiz kadarıyla, literatürlerde HKli hastalarda epidural anestezi uygulanması bulunmamaktadır. Burada, HK tanılı olgumuzda başarılı epidural anestezi deneyimimizi sunmayı amaçladık.
1. Bloch M, Adam S, Fuller A, Kremer B, Welch JP, Wiggins S, et
al. Diagnosis of Huntington disease: a model for the stages of
psychological response based on experience of a predictive
testing program. Am J Med Genet 1993;47(3):368-74
2. Rodrigo MR. Huntingtons chorea: midazolam, a suitable
induction agent? Br J Anaesth 1987;59(3):388-9
3. Kaufman MA, Erb T. Propofol for patients with Huntingtons
chorea? Anaesthesia 1990;45(10):889-90
4. Nagele P, Hammerle AF. Sevoflurane and mivacurium in a
patient with Huntingtons chorea. Br J Anaesth 2000;85(2):320-
1
5. Fernandez IG, Sanchez MP, Ugalde AJ, Hernandez CM. Spinal
anaesthesia in a patient with Huntingtons chorea. Anaesthesia
1997;52(4):391
6. Gaubatz CL, Wehner RJ. Anesthetic considerations for the
patient with Huntingtons disease. AANA J 1992;60(1):41-4
7. MacPherson P, Harper I, MacDonald I. Propofol and
remifentanil total intravenous anesthesia for a patient with
Huntington disease. J Clin Anesth 2004;16(7):537-8
8. Gilli E, Bartoloni A, Fiocca F, Dall'Antonia F, Carluccio S
Anaesthetic management in a case of Huntington's chorea
Minerva Anestesiol 2006;72(9):757-62
9. Stoelting RK, Dierdorf SF, Mccammon RL. Anesthesia and coexisting
disease. New York: Churchill Livingstone 1988:307
10. Qin ZH, Wang J, Gu ZL. Development of novel therapies for
Huntingtons disease: hope and challenge. Acta Pharmacol Sin
2005;26(2):129-42
Erbaş, M., Dost, B., & Karapolat, B. S. (2012). Epidural anesthesia in a patient with Huntingtons chorea. Gaziantep Medical Journal, 18(2), 113-114.
AMA
Erbaş M, Dost B, Karapolat BS. Epidural anesthesia in a patient with Huntingtons chorea. Gaziantep Medical Journal. March 2012;18(2):113-114.
Chicago
Erbaş, Mesut, Burhan Dost, and Bekir Sami Karapolat. “Epidural Anesthesia in a Patient With Huntingtons Chorea”. Gaziantep Medical Journal 18, no. 2 (March 2012): 113-14.
EndNote
Erbaş M, Dost B, Karapolat BS (March 1, 2012) Epidural anesthesia in a patient with Huntingtons chorea. Gaziantep Medical Journal 18 2 113–114.
IEEE
M. Erbaş, B. Dost, and B. S. Karapolat, “Epidural anesthesia in a patient with Huntingtons chorea”, Gaziantep Medical Journal, vol. 18, no. 2, pp. 113–114, 2012.
ISNAD
Erbaş, Mesut et al. “Epidural Anesthesia in a Patient With Huntingtons Chorea”. Gaziantep Medical Journal 18/2 (March 2012), 113-114.
JAMA
Erbaş M, Dost B, Karapolat BS. Epidural anesthesia in a patient with Huntingtons chorea. Gaziantep Medical Journal. 2012;18:113–114.
MLA
Erbaş, Mesut et al. “Epidural Anesthesia in a Patient With Huntingtons Chorea”. Gaziantep Medical Journal, vol. 18, no. 2, 2012, pp. 113-4.
Vancouver
Erbaş M, Dost B, Karapolat BS. Epidural anesthesia in a patient with Huntingtons chorea. Gaziantep Medical Journal. 2012;18(2):113-4.