Objective:To determine if severe perivascular space (PVS) dilation is associated with longitudinal cognitive decline and incident dementia over four and eight years respectively, we analyzed data from a prospective cohort study.Methods:414 community dwelling older adults aged 72-92 were assessed at baseline and biennially for up to eight years, with cognitive assessments, consensus dementia diagnoses and 3T MRI imaging. The numbers of PVS in two representative slices in the basal ganglia (BG) and centrum semiovale (CSO) were counted and severe PVS pathology defined as the top quartile. The effects of severe PVS pathology in i) either region; ii) both regions; and those with iii) severe BG PVS and iv) severe CSO PVS were examined. White matter hyperintensity volume, cerebral microbleed number and lacune number were calculated.Results:Participants with severe PVS pathology in both regions or in the CSO alone had greater decline in global cognition over four years, even after adjustment for the presence of other small vessel disease neuroimaging markers. The presence of severe PVS pathology in both regions was an independent predictor of dementia across eight years (OR 2.91, 95%CI 1.43–5.95, p= 0.003). Further, the presence of severe PVS pathology in all groups examined was associated with greater dementia risk at either year four or six.Conclusions:Severe PVS pathology is a marker for increased risk of cognitive decline and dementia, independent of other small vessel disease markers. The differential cognitive associations for BG and CSO PVS may represent differences in their underlying pathology.

We present the first quantitative analysis of atypical teratoid rhabdoid tumors (ATRT) in adults, including two patients from our own institutions. These are of interest as one occurred during pregnancy and one is a long-term survivor. Our review of pathological findings of 50 reported cases of adult ATRT leads us to propose a solely ectodermal origin for the tumor and that epithelial–mesenchymal transition (EMT) is a defining feature. Thus, the term ATRT may be misleading. Our review of clinical findings shows that ATRT tends to originate in mid-line structures adjacent to the CSF, leading to a high rate of leptomeningeal dissemination. Thus, we hypothesize that residual undifferentiated ectoderm in the circumventricular organs, particularly the pituitary and pineal glands, is the most common origin for these tumors. We note that if growth is not arrested soon after diagnosis, or after the first relapse/progression, death is almost universal. While typically rapidly fatal (as in our first case), long-term remission is possible (as in our second). Significant predictors of prognosis were the extent of resection and the use of chemotherapy. Glial differentiation (GFAP staining) was strongly associated with leptomeningeal metastases (chi-squared p = 0.02) and both predicted markedly worse outcomes. Clinical trials including adults are rare. ATRT is primarily a disease of infancy and radiotherapy is generally avoided in those aged less than 3 years old. Treatment options in adults differ from infants in that cranio-spinal irradiation is a viable adjunct to systemic chemotherapy in the adult population. Given the grave prognosis, this combined approach appears reasonable. As effective chemotherapy is likely to cause myelosuppression, we recommend that stem-cell rescue be available locally.

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We describe the use of computed tomography (CT) with multiplanar reconstruction (MPR) in positive diagnosis of a case of enteric intussusception in an adult. To our knowledge, there have been no previous reports of the use of MPR in this setting. Intussusception in adults is an uncommon cause of bowel obstruction that usually presents with non-specific symptoms. An underlying lesion is found in most cases. Multiplanar reconstruction should be used to confirm the diagnosis of intussusception when suspected on axial views, and should increase the yield of positive diagnoses when used in the assessment of a complex abdominal mass involving the bowel.