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Cortical superficial siderosis (cSS) is a magnetic resonance imaging marker of cerebral amyloid angiopathy (CAA) and can be its sole imaging sign. cSS has further been identified as a risk marker for future intracranial hemorrhage. Although uncommon in the general population, cSS may be much more prevalent in high risk populations for amyloid pathology. We aimed to determine the frequency of cSS in patients with cognitive impairment presenting to a memory clinic. We prospectively evaluated consecutive patients presenting to our memory clinic between April 2011 and April 2013. Subjects received neuropsychological testing using the Consortium to Establish a Registry for Alzheimer's Disease battery (CERAD-NP). Two hundred and twelve patients with documented cognitive impairment further underwent a standardized 3T-MR-imaging protocol with T2*-weighted gradient-echo sequences for detection of cSS. Thirteen of 212 patients (6.1 %) displayed cSS. In seven of them (54 %) cSS was the only imaging sign of CAA. Patients with cSS did not differ from patients without cSS with regard to medical history, age or cardiovascular risk profile. Subjects with cSS performed worse in the mini-mental state examination ( p = 0.001), showed more white matter hyperintensities ( p = 0.005) and more often had microbleeds ( p = 0.001) compared to those without cSS. cSS is common in patients with cognitive impairment. It is associated with lower cognitive scores, white matter hyperintensities and microbleeds and can be the only imaging sign for CAA in this patient group. [ABSTRACT FROM AUTHOR]

Deep cerebral venous system thrombosis (DCVST) is a rare variety of cerebral vein and sinus thrombosis (CVST), therefore clinical information regarding presentation, course and outcome are limited. In this two-center study including 32 patients, we tried to better define symptoms, neuroradiological findings, course, and outcome in DCVST. All consecutive patients with DCVST admitted to our two institutions over a period of more than 10 years were identified from prospective registries on CVST and stroke patients. Data from the registries were confirmed and complemented by retrospective analysis of patients’ charts and neuroradiological imaging. Only patients with an unequivocal diagnosis of DCVST confirmed by MRI and MRA were included. Information on long-term functional outcome (modified Rankin Scale, mRS; ability to return to work) was obtained by telephone interviews performed between 2006 and 2008. The clinical presentation was highly variable with headache (81%) and reduced consciousness (72%) as the most frequent symptoms. In nine patients (28%) thrombosis was confined to the deep venous system (isolated DCVST). In the remaining patients other sinuses and/or cortical veins were additionally affected (non-isolated DCVST). Diagnosis was made within one to 76 days (mean = 10.0 ± 14.1 days) but was significantly delayed in patients with isolated compared to non-isolated DCVST (19.1 ± 23.0 vs. 6.3 ± 6.5 days, P = 0.02). Thalamic edema was the most frequent parenchymal MRI finding present in 69% of patients, bilateral in 47%. d-dimer levels were normal in 13% of patients. Most patients (75%) stabilized and later improved on intravenous heparin or subcutaneous low molecular weight heparin. Eight (25%) patients deteriorated with progressing coma; six of them received local endovascular therapy but two died. After a mean follow-up of 3.8 years (range 3 months–13 years), 26 patients (81%) were functionally independent (mRS ≤ 2) including 24 patients (75%) with a mRS ≤ 1 of whom 23 (96%) returned to their previous job, activity or education. No patients were severely disabled (mRS 4–5). Extension of thrombosis beyond the deep venous system had no effect on outcome. Due to its variable clinical presentation the diagnosis of DCVST is often difficult and heparin treatment therefore established with substantial delay. While most patients stabilize and have a good recovery, progressing coma associated with poor outcome is seen in a subset of patients who may thus require other treatment options, such as endovascular therapy. [ABSTRACT FROM AUTHOR]