General Anesthesia Plus Muscle Relaxant in a Patient with Kugelberg Welander Disease: A Case Report

  • Ebrahim Espahbodi Department of Anesthesiology and Critical Care Medicine, Farabi Hospital, Tehran University of Medical Sciences, Tehran, Iran.
  • Amir Abbas Yaghooti Department of Anesthesiology and Critical Care Medicine, Farabi Hospital, Tehran University of Medical Sciences, Tehran, Iran.
  • Hossein Sadrossadat Department of Anesthesiology and Critical Care Medicine, Farabi Hospital, Tehran University of Medical Sciences, Tehran, Iran.
  • Mehrdad Shoroughi Department of Anesthesiology and Critical Care Medicine, Farabi Hospital, Tehran University of Medical Sciences, Tehran, Iran.
  • Alireza Ebrahim Soltani Department of Anesthesiology and Critical Care Medicine, Farabi Hospital, Tehran University of Medical Sciences, Tehran, Iran.
  • Mehrdad Goudarzi Department of Anesthesiology and Critical Care Medicine, Farabi Hospital, Tehran University of Medical Sciences, Tehran, Iran.
  • Mehdi Sanatkar Department of Anesthesiology and Critical Care Medicine, Farabi Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Keywords: spinal muscular atrophy, squint surgery, muscle relaxant

Abstract

Spinal muscular atrophies (SMAs) represent a rare group of inherited disorders that cause progressive degeneration of the anterior horn cells of the spinal cord. The exact cause of the degeneration is unknown. Loss of these cells results in a progressive lower motor neuron disease that has no sensory involvement and that is manifested as hypotonia, weakness, and progressive paralysis. Kugelberg Welander spinal muscular atrophy (also known as Wohlfart-Kugelberg-Welander syndrome or mild SMA) is a milder form of SMA, with symptoms typically presenting after age 18 months. Here in we report a case of anesthetic management of a patient with Kugelberg Welander disease who was refered for squint surgery and also we reviewed some other cases of SMA patients receiving different types of anesthesia.

References

Bradley WG, ed. Neurology in Clinical Practice. 5th ed. Philadelphia, Pa: Butterworth-Heinemann/Elsevier; 2008.

Herring JA, ed. Tachdjian's Pediatric Orthopaedics. 4th ed. Philadelphia, Pa: Saunders/Elsevier; 2008.

Wang CH, Finkel RS, Bertini ES, Schroth M, Simonds A, Wong B, et al. Consensus statement for standard of care in spinal muscular atrophy. J Child Neurol. 2007; 22(8):1027-49.

Prior TW, Russman BS. Spinal muscular atrophy. GeneReviews. www.ncbi.nlm.nih.gov/books/NBK1352/ (Accessed on November 03, 2014).

Thomas NH, Dubowitz V. The natural history of type I (severe) spinal muscular atrophy. Neuromuscul Disord. 1994; 4(5-6):497-502.

Farrar MA, Vucic S, Johnston HM, du Sart D, Kiernan MC. Pathophysiological insights derived by natural history and motor function of spinal muscular atrophy. J Pediatr. 2013; 162(1):155-9.

Lefebvre S, Bürglen L, Frézal J, Munnich A, Melki J. The role of the SMN gene in proximal spinal muscular atrophy. Hum Mol Genet. 1998; 7(10):1531-6.

Kaufmann P, McDermott MP, Darras BT, Finkel RS, Sproule DM, Kang PB, et al. Prospective cohort study of spinal muscular atrophy types 2 and 3. Neurology. 2012; 79(18):1889-97.

Kroksmark AK, Beckung E, Tulinius M. Muscle strength and motor function in children and adolescents with spinal muscular atrophy II and III. Eur J Paediatr Neurol. 2001; 5(5):191-8.

Rudnik-Schöneborn S, Hausmanowa-Petrusewicz I, Borkowska J, Zerres K. The predictive value of achieved motor milestones assessed in 441 patients with infantile spinal muscular atrophy types II and III. Eur Neurol. 2001; 45(3):174-81.

Brahe C, Servidei S, Zappata S, Ricci E, Tonali P, Neri G. Genetic homogeneity between childhood-onset and adult-onset autosomal recessive spinal muscular atrophy. Lancet. 1995; 346(8977):741-2.

Clermont O, Burlet P, Lefebvre S, Bürglen L, Munnich A, Melki J. SMN gene deletions in adult-onset spinal muscular atrophy. Lancet. 1995; 346 (8991-8992):1712-3.

Zerres K, Rudnik-Schöneborn S, Forkert R, Wirth B. Genetic basis of adult-onset spinal muscular atrophy. Lancet. 1995; 346 (8983):1162.

Veen A, Molenbuur B, Richardson FJ, Epidural anaesthesia in a child with possible spinal muscular atrophy. Paediatr Anaesth. 2002; 12(6):556-8.

Watts JC. Total intravenous anaesthesia without muscle relaxant for eye surgery in a patient with Kugelberg-Welander Syndrome. Anaesthesia, 2003; 58(1):96.

Habib AS, Helsley SE, Millar S, Deballi P 3rd, Muir HA. Anesthesia for cesarean section in a patient with spinal muscular atrophy. J Clin Anesth. 2004; 16(3):217-9.

Kitson R, Williams V, Howell C. Caesarean section ina parturient with type III spinal muscular atrophy and pre-eclampsia. Anaesthesia, 2004; 59(1):94-5.

McLoughlin L, Bhagvat P. Anaesthesia for caesarean section in spinalmuscular atrophy type III. Int J Obstet Anesth. 2004; 13(3):192-5.

Stucke AG, Stuth EA. Use of rapacuronium in a child with spinal muscular atrophy. Paediatr Anaesth. 2001; 11(6):725-8.

Buettner AU. Anaesthesia for caesarean section in a patient with spinal muscular atrophy. Anaesth Intensive Care. 2003; 31(1):92-4.

Harris SJ, Moaz K. Caesarean section conducted under subarachnoid block in two sisters with spinal muscular atrophy. Int J Obstet Anesth. 2002; 11(2):125-7.

Weston LA, DiFazio CA. Labor analgesia and snesthesia in a patient with spinal muscular atrophy and vocal cord paralysis a rare and unusual case report. Reg Anesth. 1996; 21(4):350-4.

Iwashita K, Sugi Y, Higa K, Katori K, Nitahara K. Anesthetic management of a patient with spinal muscular atrophy type III. Masui. 2008; 57(3):358-9.

Arima H, Sobue K, Tanaka S, Morishima T, Ando H, Katsuya H. Difficult airway in a child with spinal muscular atrophy type I. Paediatr Anaesth. 2003; 13(4):342-4.

Published
2015-10-03
How to Cite
1.
Espahbodi E, Yaghooti AA, Sadrossadat H, Shoroughi M, Ebrahim Soltani A, Goudarzi M, Sanatkar M. General Anesthesia Plus Muscle Relaxant in a Patient with Kugelberg Welander Disease: A Case Report. AACC. 1(1):34-6.
Section
Case Report(s)