Journal of the neurological sciences
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Cerebral microangiopathies are responsible of a great number of strokes. In the recent years advances in molecular genetics identified several monogenic conditions involving cerebral small vessels and predisposing to ischemic and/or hemorrhagic stroke and diffuse white matter disease leading to vascular dementia. ⋯ Albeit with variable phenotypes and with different defective genes, all these disorders produce arteriopathy and microvascular disintegration with changes in brain functions. Specific diagnostic tools are recommended, genetic analysis being the gold standard for the diagnosis.
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Susac syndrome was named after J. O. Susac who first described the syndrome in 1979. ⋯ Patients are successfully treated with immunosuppression, however, the best regimen still needs to be defined. As a result of the rarity of the disease, controlled therapeutic trials are missing so far. In this review, we want to demonstrate the clinical features, natural history, treatment, and clinical course of Susac syndrome, illustrated by a typical case history.
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Recent epidemiological studies have indicated numerous associations between vascular and lifestyle related risk factors and incident dementia. However, evidence from randomised controlled trials (RCT) showing effectiveness of interventions aimed at these risk factors in preventing or postponing dementia onset is still lacking. Three large RCTs on multi-component interventions to prevent dementia (preDIVA, FINGER, MAPT) have been initiated in Europe to address these issues. ⋯ Separation of intervention and outcome assessment in time is a potential solution, but requires studies with very long follow-up. International collaboration of research groups with experience in dementia prevention studies and well-organised logistics for these major projects is pivotal to success for future large-scale dementia prevention studies. Founding of EDPI is an important first step in this direction.
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The direct impact of external carotid-internal carotid (EC-IC) bypass surgery on cognition of patients with severe steno-occlusive disease of internal carotid (ICA) or middle cerebral artery (MCA) is unknown. In this pilot study, we evaluated changes in cerebral hemodynamic and cognition in these patients. ⋯ EC-IC bypass surgery in carefully selected patients could improve cerebral hemodynamics and verbal memory and executive function.
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While evidence suggests that lobar intracerebral haemorrhage (ICH) is linked with dementia and cognitive impairment, the association between cognition and mortality risk from ICH is unclear. ⋯ A history of dementia or cognitive impairment is more common in lobar CAA-related ICH and it is a medium-term mortality predictor in lobar ICH but not in deep non-lobar ICH.