Intrathecal Baclofen Facilitated Postanesthetic Tracheal Extubation in a Dystonic Patient Associated with Neurodegeneration of Brain Iron Accumulation (Hallervorden-Spatz Disease)

Abstract

Patients with Hallervorden-Spatz disease may be confronted by invasive procedure, like gastrostomy and thalamotomy for care of the status of extreme dystonia and rigidity. This rare disorder possesses potential perioperative risks, such as difficult airway management, aspiration pneumonia, hyperpyrexia, dehydration, acute renal failure, and postoperative pulmonary insufficiency. As patients were usually in the state of uncontrollable dystonia and rigidity, delayed endotracheal extubation had been suggested by several previous case reports. We report the anesthetic management of a 34-year-old Hallervorden-Spatz disease woman scheduled for intrathecal baclofen pump implantation under general anesthesia. Preoperatively she had suffered from severe dystonia, confusion and repeated attacks of respiratory insufficiency and pneumonia requiring mechanical ventilation and critical care. Anesthesia was induced uneventfully and the catheter of the pump reached the CSF via T2 interspace. Intraoperative intrathecal baclofen injection mildly relieved her severe dystonia and rigidity. Hemodynamics and arterial blood gas were improved than preoperative. She was extubated after consciousness was regained. Postanesthetic visit demonstrated a fair outcome. Whether this treatment applies to every case confronted by general anesthesia needs to be elucidated. Nevertheless, we suggest intrathecal baclofen may help postanesthetic pulmonary care by attenuating dystonia and rigidity and can be an adjuvant for anesthesia care in patients with Hallervorden-Spatz disease.

Share and Cite:

C. Lee, Y. Chu, C. Chuang, C. Chen, M. Tsou and K. Chan, "Intrathecal Baclofen Facilitated Postanesthetic Tracheal Extubation in a Dystonic Patient Associated with Neurodegeneration of Brain Iron Accumulation (Hallervorden-Spatz Disease)," Neuroscience and Medicine, Vol. 2 No. 4, 2011, pp. 351-354. doi: 10.4236/nm.2011.24046.

Conflicts of Interest

The authors declare no conflicts of interest.

References

[1] K. F. Swaiman, “Hallervorden-Spatz Syndrome,” Pediatric Neurology, Vol. 25, No. 2, 2001, pp. 102-108. doi:10.1016/S0887-8994(01)00253-3
[2] M. Kyriagis, P. Grattan-Smith, A. Scheinberg, C. Teo, N. Nakaji and M. Waugh, “Status Dystonicus and Hallervorden-Spatz Disease: Treatment with Intrathecal Baclofen and Pallidotomy,” Journal of Paediatrics Child Health, Vol. 40, No. 5-6, 2004, pp. 322-325. doi:10.1111/j.1440-1754.2004.00374.x
[3] J. Hinkelbeim, A. Kalenka and M. Alb, “Anesthesia for Patients with Pantothenate-Kinase-Associated Neurodegeneration (Hallervorden-Spatz Disease)—A Literature Review,” Acta Neuropsychiatrica, Vol. 18, No. 3-4, 2006, pp. 168-172. doi:10.1111/j.1601-5215.2006.00144.x
[4] R. C. Roy, S. McLain, A. Wise and L. D. Shaffner, “Anesthetic Management of a Patient with Hallervorden-Spatz Disease,” Anesthesiology, Vol. 58, 1983, pp. 382-384. doi:10.1097/00000542-198304000-00017
[5] M. T. Keegan, R. P. Flick, J. Y. Matsumoto, D. H. Davis and W. L. Lanier, “Anesthetic Management for Two-Stage Computer-Assisted, Stereotactic Thalamotomy in a Child with Hallervorden-Spatz Disease,” Journal of Neurosurgical Anesthesiology, Vol. 12, No. 2, 2000, pp. 107-111. doi:10.1097/00008506-200004000-00006
[6] W. Halliday, “The Nosology of Hallervorden-Spatz disease,” Journal of the Neurological Sciences, Vol. 134, Supplement, 1995, pp. 84-91. doi:10.1016/0022-510X(95)00212-K
[7] S. J. Hayflick, S. K. Westaway, B. Levinson, B. Zhou, M. A. Johnson, K. H. Ching and J. Gitschier, “Genetic, Clinical, and Radiographic Delineation of Hallervorden-Spatz Syndrome,” The New England Journal of Medicne, Vol. 348, 2003, pp. 33-40. doi:10.1056/NEJMoa020817
[8] S. Kapoor, K. Hortnagel, S. Gogia, R. Paul, V. Malhotra and A. Prakash, “Pantothenate Kinase Associated Neuro-degeneration (Hallervorden-Spatz Syndrome),” Indian Journal of Pediatrics, Vol. 72, 2005, pp. 261-263.
[9] R. P. Guillerman, “The Eye-of-the-Tiger Sign,” Radiology, Vol. 217, 2000, pp. 895-896.
[10] K. D. Sethi, R. J. Adams, D. W. Loring and T. El Gammal, “Hallervorden-Spatz Syndrome: Clinical and Magnetic Resonance Imaging Correlations,” Annals of Neurology, Vol. 24, No. 5, 1988, pp. 692-694. doi:10.1002/ana.410240519
[11] P. Mariotti, A. Fasano, M. F. Contarino, G. D. Marca, M. Piastra, O. Genovese, S. Pulitanò, A. Chiaretti and A. R. Bentivoglio, “Management of Status Dystonicus: Our Experience and Review of the Literature,” Movement Disorders, Vol. 22, No. 7, 2007, pp. 963-968. doi:10.1002/mds.21471
[12] E. C. Sheehy, P. Longhurst, D. Pool and M. Dandekar, “Self-Inflicted Injury in a Case of Hallervorden-Spatz Disease,” International Journal of Paediatric Dentistry, Vol. 9, No. 4, 1999, pp. 299-302. doi:10.1111/j.1365-263X.1999.00149.x
[13] I. Balas, N. Kovacs and K. Hollody, “Staged Bilateral Stereotactic Pallidothalamotomy for Life-Threatening Dystonia in a Child with Hallervorden-Spatz Disease,” Movement Disorders, Vol. 21, No. 1, 2006, pp. 82-85. doi:10.1002/mds.20655
[14] P. Hurtado, L. Salvador, E. Carrero, J. Rumia and N. Fabregas, “Anesthesia Considerations for Deep-Brain Stimulation in a Patient with Type-2 Pantothenate Kinase Deficiency (Hallervorden-Spatz Disease),” Revista Espanola de Anestesiología y Reanimación, Vol. 56, No. 3, 2009, pp. 180-184.

Copyright © 2024 by authors and Scientific Research Publishing Inc.

Creative Commons License

This work and the related PDF file are licensed under a Creative Commons Attribution 4.0 International License.