Abstract SNACC-63

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The perioperative management of suspected prion disease

Samuelson C, Chandler D, Benson K, Hamilton C, Fox C
LSU-Shreveport, Shreveport, LA, USA

Creutzfeldt-Jakob Disease (CJD) and its variants are a group of transmissible spongiform encephalopathies that lead to dementia, neurological decline and death in all infected patients. While the exact method of transmission remains a matter of debate, it is known that a prion plays a central role in the spread and progression of the disease. These infectious proteins are resistant to common disinfection procedures, and consequently have been linked to iatrogenic infection in three separate circumstances: after the use of insufficiently sterilized equipment; after the use of extracted pituitary hormones; and after the implantation of corneal and dural grafts. While some studies suggest the incidence of CJD to be as low as 1:1,000,000, a 2004 study of tonsillar tissue samples from asymptomatic UK patients reported between 4,000 and 30,000 carriers of the infectious protein. Due to the resistant nature of the prion protein and the devastating consequences of iatrogenic transmission among patients and healthcare workers, patients with suspected or confirmed CJD pose an interesting challenge to anesthesia providers.

We present the case of a previously healthy 46 year old African-American female admitted for acute onset of progressive neurologic decline. She was evaluated extensively by Neurology and was found to have EEG evidence of encephalitis, slightly elevated Protein 14-3-3 levels and hyperintensity of the caudate nucleus on MRI of the brain. As the goal was to rule out prion disease from other causes of treatable encephalopathy, Neurosurgery was asked to perform a brain biopsy. While the biopsy results were negative, this case highlights the need for health-care providers to be knowledgeable of the current recommendations that should be applied for any patient with suspected or confirmed prion disease.

We performed a review of the literature on the anesthetic management of patients with CJD. The accompanying tables summarize the recommendations on patient management based on operative staging as well as the inherent transmission risk of the surgery (Table 1; Table 2; Table 3).

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