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Primary age-related tauopathy (PART) is a neuropathological designation introduced in 2014 to describe the neurofibrillary tangles (NFT) that are commonly observed in the brains of normally aged and cognitively impaired individuals that can occur independently of the amyloid plaques of Alzheimer's disease (AD).
AL amyloidosis is caused by the deposition of abnormal antibody free light chains. The abnormal light chains are produced by monoclonal plasma cells, and, although AL amyloidosis can occur without diagnosis of another disorder, it is often associated with other plasma cell disorders, such as multiple myeloma and Waldenström's macroglobulinemia. [6]
The familial amyloid neuropathies (or familial amyloidotic neuropathies, neuropathic heredofamilial amyloidosis, familial amyloid polyneuropathy) are a rare group of autosomal dominant diseases wherein the autonomic nervous system and/or other nerves are compromised by protein aggregation and/or amyloid fibril formation. [1] [2] [3]
Diagnosis of amyloidosis generally requires tissue biopsy. [2] The biopsy is assessed for evidence of characteristic amyloid deposits. The tissue is treated with various stains. The most useful stain in the diagnosis of amyloid is Congo red, which, combined with polarized light, makes the amyloid proteins appear apple-green on microscopy.
The Boston criteria version 2.0 [1] is a set of guidelines designed to diagnose cerebral amyloid angiopathy (CAA), a disease that affects small blood vessels in the brain, particularly those in the cortex and leptomeninges. Although the gold standard for diagnosis is histopathological examination, the Boston criteria provide clinicians with a ...
CAA is associated with brain hemorrhages, particularly microhemorrhages.The accumulation of amyloid beta peptide deposits in the blood vessel walls results in damage of the blood vessels and hindrance of normal blood flow, making blood vessels more prone to bleeding [10] Since CAA can be caused by the same amyloid protein that is associated with Alzheimer's dementia, brain bleeds [11] are more ...
Peripheral neuropathy and mononeuropathy are common neurological disorders, with a diverse range of variables and causes to conclude a diagnosis. [2] Neuropathy has three sub-classifications; mononeuropathy is a result of an entrapped or traumatised nerve or nerve area, Mononeuropathy multiplex is linked to chronic diseases like leprosy, and ...
In May 2019, the FDA approved two tafamidis preparations for the treatment of transthyretin-mediated cardiomyopathy, but has not approved it for the treatment of transthyretin familial amyloid polyneuropathy. [15] In August 2018, the FDA approved patisiran, an siRNA-based treatment, at an expected cost of up to $450,000 per year. [16]