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Marc Manix, Piyush Kalakoti, Miriam Henry, Jai Thakur, Richard Menger, Bharat Guthikonda and Anil Nanda

Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative condition with a rapid disease course and a mortality rate of 100%. Several forms of the disease have been described, and the most common is the sporadic type. The most challenging aspect of this disease is its diagnosis—the gold standard for definitive diagnosis is considered to be histopatho-logical confirmation—but newer tests are providing means for an antemortem diagnosis in ways less invasive than brain biopsy. Imaging studies, electroencephalography, and biomarkers are used in conjunction with the clinical picture to try to make the diagnosis of CJD without brain tissue samples, and all of these are reviewed in this article. The current diagnostic criteria are limited; test sensitivity and specificity varies with the genetics of the disease as well as the clinical stage. Physicians may be unsure of all diagnostic testing available, and may order outdated tests or prematurely request a brain biopsy when the diagnostic workup is incomplete. The authors review CJD, discuss the role of brain biopsy in this patient population, provide a diagnostic pathway for the patient presenting with rapidly progressive dementia, and propose newer diagnostic criteria.

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Piyush Kalakoti, Symeon Missios, Richard Menger, Sunil Kukreja, Subhas Konar and Anil Nanda


Because of the limited data available regarding the associations between risk factors and the effect of hospital case volume on outcomes after resection of intradural spine tumors, the authors attempted to identify these associations by using a large population-based database.


Using the National Inpatient Sample database, the authors performed a retrospective cohort study that involved patients who underwent surgery for an intradural spinal tumor between 2002 and 2011. Using national estimates, they identified associations of patient demographics, medical comorbidities, and hospital characteristics with inpatient postoperative outcomes. In addition, the effect of hospital volume on unfavorable outcomes was investigated. Hospitals that performed fewer than 14 resections in adult patients with an intradural spine tumor between 2002 and 2011 were labeled as low-volume centers, whereas those that performed 14 or more operations in that period were classified as high-volume centers (HVCs). These cutoffs were based on the median number of resections performed by hospitals registered in the National Inpatient Sample during the study period.


Overall, 18,297 patients across 774 hospitals in the United States underwent surgery for an intradural spine tumor. The mean age of the cohort was 56.53 ± 16.28 years, and 63% were female. The inpatient postoperative risks included mortality (0.3%), discharge to rehabilitation (28.8%), prolonged length of stay (> 75th percentile) (20.0%), high-end hospital charges (> 75th percentile) (24.9%), wound complications (1.2%), cardiac complications (0.6%), deep vein thrombosis (1.4%), pulmonary embolism (2.1%), and neurological complications, including durai tears (2.4%). Undergoing surgery at an HVC was significantly associated with a decreased chance of inpatient mortality (OR 0.39; 95% CI 0.16−0.98), unfavorable discharge (OR 0.86; 95% CI 0.76−0.98), prolonged length of stay (OR 0.69; 95% CI 0.62−0.77), high-end hospital charges (OR 0.67; 95% CI 0.60−0.74), neurological complications (OR 0.34; 95% CI 0.26−0.44), deep vein thrombosis (OR 0.65; 95% CI 0.45−0.94), wound complications (OR 0.59; 95% CI 0.41−0.86), and gastrointestinal complications (OR 0.65; 95% CI 0.46−0.92).


The results of this study provide individualized estimates of the risks of postoperative complications based on patient demographics and comorbidities and hospital characteristics and shows a decreased risk for most unfavorable outcomes for those who underwent surgery at an HVC. These findings could be used as a tool for risk stratification, directing presurgical evaluation, assisting with surgical decision making, and strengthening referral systems for complex cases.