TL;DR: Using sensitive techniques, extraneural deposition of PrPSc is identified in spleen and muscle samples from approximately one third of patients who died with sporadic Creutzfeldt-Jakob disease, and appears to correlate with a long duration of disease.
Abstract: Background In patients with sporadic Creutzfeldt–Jakob disease, pathologic disease-associated prion protein (PrPSc) has been identified only in the central nervous system and olfactory-nerve tissue. Understanding the distribution of PrPSc in Creutzfeldt–Jakob disease is important for classification and diagnosis and perhaps even for prevention. Methods We used a highly sensitive method of detection — involving the concentration of PrPSc by differential precipitation with sodium phosphotungstic acid, which increased the sensitivity of Western blot analysis by up to three orders of magnitude — to search for PrPSc in extraneural organs of 36 patients with sporadic Creutzfeldt–Jakob disease who died between 1996 and 2002. Results PrPSc was present in the brain tissue of all patients. In addition, we found PrPSc in 10 of 28 spleen specimens and in 8 of 32 skeletal-muscle samples. Three patients had PrPSc in both spleen and muscle specimens. Patients with extraneural PrPSc had a significantly longer duration of...
TL;DR: It is clear that transdural access in continuity is not necessarily provided through the operative flap and that it may occur at sites removed from, or independent of, the site of operation, as demonstrated in two of the three cases described in this article.
TL;DR: Cerebrospinal fluid shunts, regardless of type, location, revision rate, or filter insertion, do not predispose pediatric patients with brain tumors to develop extraneural metastases, and a diagnosis of shunt-related metastases should be based on the development of intra-abdominal (VP shunt) or pulmonary (VA shunt).
Abstract: ✓ The authors reviewed the hospital charts of 415 pediatric patients treated for benign or malignant primary brain tumors over the past 20 years at the Children's Hospital Medical Center, Seattle. Patients' ages ranged from the neonatal period to 18 years. A shunt was placed in 152 patients (37%), 45 before and 94 after surgery. Confirmation of extraneural metastases was based on clinical and diagnostic examination. Factors analyzed as possibly influencing the occurrence of extraneural metastases were: 1) the shunt: type, valve, location, filter, and revisions; 2) extent of resection; 3) pathology; and 4) treatment regimen. Eight of the 415 patients developed extraneural metastases during life. All eight patients had a medulloblastoma (cerebellar primitive neuroectodermal tumor). These eight patients were separated into Group A (without a shunt) and Group B (with a shunt). In Group A (five patients), the mean interval from primary diagnosis to metastasis was 15 months. Two children had gross total resecti...
TL;DR: It is suggested that γ‐hydroxybutyrate may participate in the metabolism of many organs, and that GABA may not be the precursor in extraneural tissues.
Abstract: gamma-Hydroxybutyrate has been found to be widely distributed in both neural and extraneural tissues in the rat. The kidney and brown fat have more than 10 times higher concentrations of gamma-hydroxybutyrate than does the brain. This observation suggests that gamma-hydroxybutyrate may participate in the metabolism of many organs, and that GABA may not be the precursor in extraneural tissues.
TL;DR: It is shown that infection of the left sciatic nerve leads to direct spread of infection to brain (at a rate of approximately 1.0-2.0 mm/day), bypassing the need for extraneural replication and thus producing shorter incubation periods, and the efficiency of intraneural infection is low.