Non-Alzheimers Amyloidoses of the Neurological System: Cerebral Amyloid Angiopathy and Familial Amyloid Polyneuropathy

Abstract

Amyloid deposition plays a significant, albeit overlooked, role in neurologic disorders. Deposition of amyloid proteins in both the central and peripheral nervous systems lead to debilitating, and often deadly, organ dysfunction, including cerebral amyloid angiopathy, familial amyloid neuropathy, and Alzheimer’s disease (AD). Alzheimer’s disease is discussed in some detail in a separate chapter within this book, and therefore will not be discussed in detail in this chapter. In this chapter, we present the pathological mechanisms, disease manifestations, diagnostic approach, and treatment modalities for diseases of the nervous system caused by amyloid deposition. While significant strides have been made over the years in identifying key underlying pathologic mechanisms, the medical community’s understanding of these rare conditions remain limited. The primary goal of this chapter is to provide additional resources and information for clinicians to help identify these disorders early in their course before they cause irreparable damage to their patients.