Intravascular large B-cell lymphoma (IVBCL) represents a rare subtype of extranodal diffuse large B-cell lymphoma characterized by selective growth of neoplastic cells within the lumen of small and medium-sized vessels. The clinical spectrum is heterogeneous dominated by neurological and skin manifestations. Intra-vitam diagnosis still remains challenging and usually requires brain biopsy since no pathognomonic neuroradiological findings do exist for IVBCL. We report on the case of a 65-year old male patient presenting with multifocal neurological signs and symptoms including cognitive deficits, aphasia and paraparesis as leading features. Imaging findings were suggestive for cerebral vasculitis prompting initiation of steroid treatment. After initial stabilization the patient deteriorated in spite of systemic steroid treatment, developed severe sepsis and finally died from multi-organ failure. Histopathological findings revealed CD20 positive lymphoma cells in small- and middle-sized vessels of the cerebrum, the lungs and the skin diagnostic for IVBCL. This case should raise the alertness for consideration of IVBCL as a differential diagnosis resulting in early brain biopsy, if no etiology for CNS vasculitis is evident.

Objective: To analyze the association between patient age and good functional outcome after ischemic stroke with special focus on young patients who were numerically underrepresented in previous evaluations. Methods: Of 43,163 ischemic stroke patients prospectively enrolled in the Austrian Stroke Unit Registry, 6,084 (14.1%) were ≤55 years old. Functional outcome was available in a representative subsample of 14,256 patients free of prestroke disability, 2,223 of whom were 55 years or younger. Herein we analyzed the effects of age on good functional outcome 3 months after stroke (modified Rankin Scale score ≤2). Results: Good outcome was achieved in 88.2% (unadjusted probability) of young stroke patients (≤55 years). In multivariable analysis, age emerged as a significant predictor of outcome independent of stroke severity, etiology, performance of thrombolysis, sex, risk factors, and stroke complications. When the age stratum 56–65 years was used as a reference, odds ratios (95% confidence interval [95%CI]) of good outcome were 3.4 (1.9–6.4), 2.2 (1.6–3.2), and 1.5 (1.2–1.9) for patients aged 18–35, 36–45, and 46–55 years and 0.70 (0.60–0.81), 0.32 (0.28–0.37), and 0.18 (0.14–0.22) for those aged 66–75, 76–85, and >85 years (p < 0.001). In absolute terms, the regression-adjusted probability of good outcome was highest in the age group 18–35 years and gradually declined by 3.1%–4.2% per decade until age 75 with a steep drop thereafter. Findings applied equally to sexes and patients with and without IV thrombolysis or diabetes. Conclusions: Age emerged as a highly significant inverse predictor of good functional outcome after ischemic stroke independent of stroke severity, characteristics, and complications with the age-outcome association exhibiting a nonlinear scale and extending to young stroke patients. Neurology® 2012;78:279–285

Background: Current knowledge on primary or isolated basilar artery dissection (IBAD) is limited to case vignettes and small patient series. Objective: To delineate the frequency and clinical presentations of IBAD along with short-term outcome, specific prognosis and targeted management. Methods: Data were derived from a series of 12 consecutive patients and a review of 88 cases reported in the literature. In all the cases, the dissection was confined to the basilar artery. Results: Disease incidence was estimated at 0.25 per 100,000 person-years. IBAD accounted for roughly 1.0% of all subarachnoid hemorrhage events and for no less than 10.5 and 4.5% of posterior circulation and brain-supplying artery dissections, respectively. The main clinical presentations were subarachnoid hemorrhage (46%) and posterior circulation brain ischemia (42%). Subarachnoid hemorrhage typically manifested at a higher age than brain ischemia (mean age, 48.9 vs. 41.4 years) and was more prevalent among women. Rebleedings related to pseudoaneurysm formation in patients with subarachnoid hemorrhage and recurrent ischemia in stroke patients were common in the acute phase (26.1 and 33.3%, respectively) but were rare in the long term. The outcome was generally favorable in stroke patients but variable in subarachnoid hemorrhage (case fatality rate, 21.7%). The mainstay of therapy for subarachnoid hemorrhage related to IBAD was endovascular occlusion of the aneurysm pouch whereas stroke patients were usually put on anticoagulants. Conclusions: IBAD is probably an underrecognized disease with heterogeneous clinical presentation and prognosis. It should be considered as a differential diagnosis in peritruncal subarachnoid hemorrhage, classic subarachnoid hemorrhage and posterior circulation stroke, especially in young individuals. Case management is challenging and has to be tailored to each patient.