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Sentinel Node Biopsy and Lumpectomy in a Patient with Machado-Joseph Disease.
Case Rep Anesthesiol. 2019; 2019:2309598.CR

Abstract

Spinocerebellar ataxia 3 (SCA3), also known as Machado-Joseph disease (MJD) is an autosomal dominant, progressive neurodegenerative disorder. Patients present with cerebellar ataxia, dystonia, rigidity, and neuropathy that worsen with time. On a molecular level, it occurs due to a CAG trinucleotide repeat expansion in the ATXN3 gene. Due to the risk of pulmonary aspiration, hypoventilation, autonomic and thermoregulatory dysfunction, vocal cord paralysis, progressive paraplegia, parkinsonian symptoms, and chronic pain, it has significant anesthesia implications. Rarely, case reports occur in the literature describing regional anesthetic management of patients with SCA3, but none that describe general anesthesia specifically with MJD. We therefore describe a case of a patient with SCA3 who successfully underwent general anesthesia and considerations for perioperative management of this patient population.

Authors+Show Affiliations

Anesthesiology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA.Anesthesiology/Pain Medicine, H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA.Neurology, Barnes Jewish Hospital, St. Louis, MO, USA.Anesthesiology/Pain Medicine, H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA.

Pub Type(s)

Case Reports

Language

eng

PubMed ID

31934455

Citation

Aldawoodi, N N., et al. "Sentinel Node Biopsy and Lumpectomy in a Patient With Machado-Joseph Disease." Case Reports in Anesthesiology, vol. 2019, 2019, p. 2309598.
Aldawoodi NN, Escher AR, Padalia A, et al. Sentinel Node Biopsy and Lumpectomy in a Patient with Machado-Joseph Disease. Case Rep Anesthesiol. 2019;2019:2309598.
Aldawoodi, N. N., Escher, A. R., Padalia, A., & Padalia, D. (2019). Sentinel Node Biopsy and Lumpectomy in a Patient with Machado-Joseph Disease. Case Reports in Anesthesiology, 2019, 2309598. https://doi.org/10.1155/2019/2309598
Aldawoodi NN, et al. Sentinel Node Biopsy and Lumpectomy in a Patient With Machado-Joseph Disease. Case Rep Anesthesiol. 2019;2019:2309598. PubMed PMID: 31934455.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Sentinel Node Biopsy and Lumpectomy in a Patient with Machado-Joseph Disease. AU - Aldawoodi,N N, AU - Escher,A R,Jr AU - Padalia,A, AU - Padalia,D, Y1 - 2019/12/12/ PY - 2019/09/30/received PY - 2019/11/25/accepted PY - 2020/1/15/entrez PY - 2020/1/15/pubmed PY - 2020/1/15/medline SP - 2309598 EP - 2309598 JF - Case reports in anesthesiology JO - Case Rep Anesthesiol VL - 2019 N2 - Spinocerebellar ataxia 3 (SCA3), also known as Machado-Joseph disease (MJD) is an autosomal dominant, progressive neurodegenerative disorder. Patients present with cerebellar ataxia, dystonia, rigidity, and neuropathy that worsen with time. On a molecular level, it occurs due to a CAG trinucleotide repeat expansion in the ATXN3 gene. Due to the risk of pulmonary aspiration, hypoventilation, autonomic and thermoregulatory dysfunction, vocal cord paralysis, progressive paraplegia, parkinsonian symptoms, and chronic pain, it has significant anesthesia implications. Rarely, case reports occur in the literature describing regional anesthetic management of patients with SCA3, but none that describe general anesthesia specifically with MJD. We therefore describe a case of a patient with SCA3 who successfully underwent general anesthesia and considerations for perioperative management of this patient population. SN - 2090-6382 UR - https://www.unboundmedicine.com/medline/citation/31934455/Sentinel_Node_Biopsy_and_Lumpectomy_in_a_Patient_with_Machado-Joseph_Disease L2 - https://dx.doi.org/10.1155/2019/2309598 DB - PRIME DP - Unbound Medicine ER -
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