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 Physicians may be unsure of all diagnostic testing available, and may order outdated tests or prematurely request a brain biopsy when the diagnostic workup is incomplete.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.

Endoscopic exposure of the infratemporal fossa is feasible. Using the combination of the endonasal and Caldwell-Luc approaches for direct transmaxillary access significantly extended exposure, allowing safe and effective resection of infratemporal fossa lesions.

OBJECTIVE The clinical significance of the Simpson system for grading the extent of meningioma resection and its role as a predictor of the recurrence of World Health Organization (WHO) Grade I meningiomas have been questioned in the past, echoing changes in meningioma surgery over the years. The authors reviewed their experience in resecting WHO Grade I meningiomas and assessed the association between extent of resection, as evaluated using the Simpson classification, and recurrence-free survival (RFS) of patients after meningioma surgery. METHODS Clinical and radiological information for patients with WHO Grade I meningiomas who had undergone resective surgery over the past 20 years was retrospectively reviewed. Simpson and Shinshu grading scales were used to evaluate the extent of resection. Statistical analysis was conducted using Kaplan-Meier curves and Cox proportional-hazards regression. RESULTS Four hundred fifty-eight patients were eligible for analysis. Overall tumor recurrence rates for Simpson resection Grades I, II, III, and IV were 5%, 22%, 31%, and 35%, respectively. After Cox regression analysis, Simpson Grade I (extensive resection) was revealed as a significant predictor of RFS (p = 0.003). Patients undergoing Simpson Grade I and II resections showed significant improvement in RFS compared with patients undergoing Grade III and IV resections (p = 0.005). Extent of resection had a significant effect on recurrence rates for both skull base (p = 0.047) and convexity (p = 0.012) meningiomas. Female sex and a Karnofsky Performance Scale score > 70 were also identified as independent predictors of RFS after resection of WHO Grade I meningioma. CONCLUSIONS In this patient cohort, a significant association was noted between extent of resection and rates of tumor recurrence. In the authors' experience the Simpson grading system maintains its relevance and prognostic value and can serve an important role for patient education. Even though complete tumor resection is the goal, surgery should be tailored to each patient according to the risks and surgical morbidity.