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In this chapter, the authors will discuss the epidemiology and clinical presentations of amyloidosis. The main body of this chapter will concentrate on treatment options, both FDA-approved and experimental, specific to the various forms of amyloidosis. Since this set of diseases can affect multiple organ systems, we tackle the therapeutic avenues and the current challenges in each system under clinical investigation, including neurological, psychiatric, gastrointestinal, cardiovascular, endocrine, renal and hematologic, in addition to options for palliative treatment for severe symptom management and improved quality of life. Several recent groundbreaking discoveries have opened up the potential for successful treatment of peripheral and central neurological amyloidoses making this an exciting and evolving field.

Lacunar syndromes are clinical manifestations of lacunar infarctions. Lacunar infarctions are defined as small subcortical lesions with a size of less than 15 mm in diameter caused by occlusion of a penetrating artery from a large cerebral artery, most commonly from the Circle of Willis. These penetrating arteries arise at sharp angles from major vessels and are thus, anatomically prone to constriction and occlusion. Other common sources of these penetrating arteries include the middle cerebral artery and the basilar artery. The term “lacune” was first described in the late 19th and early 20th century based on pathologic analysis and adapted after imaging technology confirmed the initial hypothesis in the 21st century. A lacune usually describes a small, chronic cavity that represents the healed phase of lacunar infarction. However, there have been cases reported in which a lacune results from a larger infarct or intracerebral hemorrhage. The anatomic distribution of lacunar syndromes and infarctions is most commonly the basal ganglia (globus pallidus, putamen, thalamus, and caudate), the pons, and the subcortical white matter structures (internal capsule and corona radiate). These anatomical sites correspond to lesions at the lenticulostriate arteries, the anterior choroidal artery, thalamoperforant arteries, paramedian branches of the basilar artery, and the recurrent artery of Heubner from the anterior cerebral artery. Lesions at these specific sites account for many of the symptoms of lacunar syndromes. There are over 20 lacunar syndromes that have been described, but the most common ones are pure motor hemiparesis, pure sensory stroke, ataxic hemiparesis, sensorimotor stroke, and dysarthria-clumsy hand syndrome., (Copyright © 2022, StatPearls Publishing LLC.)