Progeria is a rare condition characterized by premature aging beginning in very early life and invariably ending in premature death. Progeria is caused by a mutation of the LMNA gene on chromosome1q. Based on clinical descriptions and, more recently, genetic analyses, several progeroid syndromes have been defined in addition to progeria itself. The manifestations of these conditions vary, but each has some clinical features that resemble physiologic aging and a variable risk of stroke. Most children with progeria develop premature severe vascular disease. Coronary artery disease and myocardial infarction are common, and heart disease is the leading cause of death. The Wiedemann-Rautenstrauch syndrome, sometimes called neonatal progeria, typically manifests from birth and features delayed development, poor growth, alopecia, and lipoatrophy. Given the severity of large artery pathology in patients with progeroid syndromes, it is probably reasonable to use antiplatelet agents.

This chapter investigates sarcoidosis-associated stroke syndromes. Sarcoidosis is often referred to as a "disease of exclusion". The definitive diagnosis of sarcoidosis requires histopathologic demonstration of noncaseating epithelioid granulomas that are not due to infection or malignancy. Patients can be classified as having possible, probable, or definite neurosarcoidosis based on the certainty of the diagnosis of multisystem sarcoidosis, the pattern of neurological disease, and the response to therapy. Corticosteroids are the mainstay of treatment for patients with symptomatic neurosarcoidosis; however, the severity and chronicity of this disease in some patients often leads to reluctance in subjecting patients to the long-term sequelae of corticosteroid treatment. Nonetheless, it is reasonable to decrease sarcoidosis-associated inflammation to decrease stroke risk. Anecdotal evidence suggests that concurrent use of corticosteroids and adjunctive agents is a reasonable approach. Antiplatelet agents has been used as a stroke-preventive strategy but have avoided thrombolytic interventions.

Patients with carotid stenosis are at high risk of stroke and acute ischemic events in other vascular territories and require intensive medical treatment. This chapter reviews the evidence for specific medical treatments in patients with carotid stenosis. Antiplatelet agents, cholesterol-lowering drugs and blood-pressure-lowering drugs should be considered in all patients with carotid stenosis. The use of drug combinations has the theoretical advantage of inhibiting platelet activity through more than one pharmacological mechanism, hence potentially conferring a greater antiplatelet effect. Loss of the normal autoregulatory capacity of the cerebral circulation such that cerebral blood flow is directly dependent on perfusion pressure, is common and there has been concern that blood pressure lowering may reduce cerebral perfusion and increase the risk of stroke. Intima-media thickness (IMT) is correlated with the presence of cardiovascular risk factors and possibly with the future risk of vascular events including stroke and myocardial infarction.