The human prion diseases, or transmissible spongiform encephalopathies, have captivated our imaginations since their discovery in the Fore linguistic group in Papua New Guinea in the 1950s. The mysterious and poorly understood “infectious protein” has become somewhat of a household name in many regions across the globe. From bovine spongiform encephalopathy (BSE), commonly identified as mad cow disease, to endocannibalism, media outlets have capitalized on these devastatingly fatal neurological conditions. Interestingly, since their discovery, there have been more than 492 incidents of iatrogenic transmission of prion diseases, largely resulting from prion-contaminated growth hormone and dura mater grafts. Although fewer than 9 cases of probable iatrogenic neurosurgical cases of Creutzfeldt-Jakob disease (CJD) have been reported worldwide, the likelihood of some missed cases and the potential for prion transmission by neurosurgery create considerable concern. Laboratory studies indicate that standard decontamination and sterilization procedures may be insufficient to completely remove infectivity from prion-contaminated instruments. In this unfortunate event, the instruments may transmit the prion disease to others. Much caution therefore should be taken in the absence of strong evidence against the presence of a prion disease in a neurosurgical patient. While the Centers for Disease Control and Prevention (CDC) and World Health Organization (WHO) have devised risk assessment and decontamination protocols for the prevention of iatrogenic transmission of the prion diseases, incidents of possible exposure to prions have unfortunately occurred in the United States. In this article, the authors outline the historical discoveries that led from kuru to the identification and isolation of the pathological prion proteins in addition to providing a brief description of human prion diseases and iatrogenic forms of CJD, a brief history of prion disease nosocomial transmission, and a summary of the CDC and WHO guidelines for prevention of prion disease transmission and decontamination of prion-contaminated neurosurgical instruments.

Despite the increasing prevalence of spinal infections, the subcategory of holospinal epidural abscesses (HEAs) is extremely infrequent and requires unique management. Panspinal imaging (preferably MRI), modern aggressive antibiotic therapy, and prompt surgical intervention remain the standard of care for all spinal axis infections including HEAs; however, the surgical decision making on timing and extent of the procedure still remain ill defined for HEAs. Decompression including skip laminectomies or laminoplasties is described, with varied clinical outcomes. In this review the authors present the illustrative cases of 2 patients with HEAs who were treated using skip laminectomies and epidural catheter irrigation techniques. The discussion highlights different management strategies including the role of conservative (nonsurgical) management in these lesions, especially with an already identified pathogen and the absence of mass effect on MRI or significant neurological defects. Among fewer than 25 case reports of HEA published in the past 25 years, the most important aspect in deciding a role for surgery is the neurological examination. Nearly 20% were treated successfully with medical therapy alone if neurologically intact. None of the reported cases had an associated cranial infection with HEA, because the dural adhesion around the foramen magnum prevented rostral spread of infection. Traditionally a posterior approach to the epidural space with irrigation is performed, unless an extensive focal ventral collection is causing cord compression. Surgical intervention for HEA should be an adjuvant treatment strategy for all acutely deteriorating patients, whereas aspiration of other infected sites like a psoas abscess can determine an infective pathogen, and appropriate antibiotic treatment may avoid surgical intervention in the neurologically intact patient.

Background: Autologous bone continues to represent the first choice for reconstruction of calvarial defects. However, unanswered questions remain on the natural history of the graft and the influence of patient-related risk factors. This study investigated the outcomes of skull reconstruction with split calvarial bone graft, examining the natural history and stratifying the risk of unfavorable results. Methods: Patients who underwent cranioplasty with split calvarial bone graft between 1982 and 2016 at the Cleveland Clinic were analyzed, recording demographics, comorbidities, indications, size and location of defect, and outcomes including complications and reoperations. Changes in graft thickness were analyzed using computed tomographic and magnetic resonance imaging scans. Results: Forty-one patients with an average age of 33.2 years, cranial defect size of 68 cm2, and mean follow-up of 28 months were included. The majority of patients (85 percent) had significant risk factors, with 43 percent suffering prior infection. Seventy-three percent of patients experienced successful restoration. A major complication was recorded in 26.8 percent; resorption occurred in 19.5 percent of patients (in 75 percent only at the recipient site), with 9.8 percent requiring reoperation. Patients with one or more risk factors or a smoking history were more likely to experience a complication. The mean ratio of the graft to the bicortical donor bone thickness was 0.48 ± 0.17 for the recipient site and 0.57 ± 0.10 for the donor site at an average radiographic follow-up of 11.9 ± 10.9 years. Conclusions: This study demonstrated a 73.2 percent first-attempt success rate in a high-risk population. The grafts maintain thickness over time, with no evidence of bone hypertrophy. Defect characteristics and patient systemic factors appear to be important variables influencing success. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.