These TFNS are classically described as recurrent, stereotyped, spreading paraesthesias lasting several minutes but there is a wide spectrum of presentations encompassing both positive (spreading paraesthesia or visual symptoms) and negative (paresis, [radiopaedia.org]

FBD is caused by a stop to Arg mutation in the BRI2 gene, generating de novo created amyloid molecule ABri which accumulates in FBD brains but is not present in the normal population. [ncbi.nlm.nih.gov]

A simplified overview is presented below. [amyloid.nl]

Amyloid and Related Disorders presents an overview of the most recent developments in this area including clinical presentation, etiology, pathogenesis, and differential diagnosis. [link.springer.com]

Presentation on theme: "Plasma cell dyscrasia and renal amyloidosis: What we can learn from basic science Per Westermark Rudbeck Laboratory Uppsala University, Sweden. [slideplayer.com]

• Night Blindness

  Clinical features include mild photophobia, progressive loss of central vision, night blindness, and hyperreflectivity of nerve and ganglion cell layers. {ECO:0000269 PubMed:24026677}. [genecards.org]

• Recurrent Headache

  headaches, ischemic lesions, hemorrhagic strokes, and progressive dementia (Revesz et al., 2003; pubmed: 14533778). [flybase.org]

However, the diagnosis requires a sample of an affected organ. [4] [7] Treatment The most effective treatment is autologous bone marrow transplants with stem cell rescues. [ipfs.io]

Effective treatment of the underlying inflammatory condition has reduced incidence in developed countries. [perthhaematology.com.au]

[…] standard of care includes treatments for the underlying inflammatory disease with anti-inflammatory drugs, immunosuppressive agents or biologics. [en.wikipedia.org]

TREATMENT The treatment of cardiac amyloidosis has two facets: on the one hand, the treatment of cardiac symptoms and, on the other hand, the treatment of the underlying disease which produces the amyloid protein. [revespcardiol.org]

The prognosis does not appear to be related to the degree of the proteinuria. Treatment by peritoneal dialysis or hemodialysis or kidney transplantation improves the prognosis considerably. Hypertension is rare, except in long-standing amyloidosis. [perthhaematology.com.au]

[ edit ] Prognosis varies with the type of amyloidosis. [en.wikipedia.org]

PROGNOSIS • Prognosis with generalized amyloidosis is poor. • those with AL amyloidosis have a median survival rate of 2 years after diagnosis. 60. [de.slideshare.net]

Cardiac involvement decides the prognosis. [revespcardiol.org]

[…] weak to tolerate this approach. [8] [9] Other treatments can involve application of chemotherapy similar to that used in multiple myeloma. [9] A combination of bortezomib and dexamethasone has been proposed, [10] as has melphalan and dexamethasone. [8] Prognosis [ipfs.io]

Amyloid and Related Disorders presents an overview of the most recent developments in this area including clinical presentation, etiology, pathogenesis, and differential diagnosis. [link.springer.com]

[…] appreciated 25 MRI: curvilinear regions of signal drop-out localized to one of more sulci best seen on T2* sequences (GRE, echo-planar, SWI) 9,25 ischemia ischemic leukoencephalopathy chronic lesions, indistinguishable from leukoaraiosis due to other etiologies [radiopaedia.org]

Noninvasive etiologic diagnosis of cardiac amyloidosis using 99m Tc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy. J Am Coll Cardiol. 2005;46:1076-84. Medline 66. [revespcardiol.org]

New chapters on glomerulonephritis associated with complement disorders, interventional treatments for hypertension, renal disease and cancer, and epidemiology and prognostic impact of acute kidney injury. [books.google.de]

"Systemic AA amyloidosis: epidemiology, diagnosis, and management". Clinical Epidemiology. 6 : 369–77. doi : 10.2147/CLEP.S39981. PMC 4218891. PMID 25378951. ^ a b "AA (Inflammatory) Amyloidosis". Medscape Reference. ^ "AA Amyloidosis". [en.wikipedia.org]

[…] bortezomib and dexamethasone has been proposed, [10] as has melphalan and dexamethasone. [8] Prognosis Median survival for patients diagnosed with AL amyloidosis was 13 months in the early 1990s, but had improved to c. 40 months a decade later. [11] Epidemiology [ipfs.io]

Epidemiologic characteristics of primary amyloidosis, that is, amyloidosis without overt immunoproliferative disease, and myeloma are not significantly different. [abdominalkey.com]

For example, apolipoprotein E is reportedly a common constituent of amyloid deposits, and epidemiologic studies have shown an increased risk of Alzheimer disease among white persons carrying the 4 allele of apolipoprotein E. [humpath.com]

"Amyloidosis and silicone synovitis: Updated classification, updated pathophysiology, and synovial articular abnormalities". Radiologic clinics of North America. 34 (2): 375–94, xi. [en.wikipedia.org]

[…] random distribution fat embolism syndrome 'starfield' pattern of distribution lesions also show restricted diffusion on DWI and are likely visible on other sequences radiation induced vasculopathy microhemorrhages have a very similar appearance (similar pathophysiology [radiopaedia.org]

The disease has no effective therapy and the mechanisms responsible for pathophysiology are not well understood. Mutations linked to familial ALS have been helpful in exploring the processes that cause the disease. [bitanlab.neurology.ucla.edu]

Despite their biochemical and clinical differences, the various amyloidoses share common pathophysiologic features: a precursor substance in appropriate concentration; appropriate host genetic background; abnormalities in proteolysis of fibril precursors [perthhaematology.com.au]

Several of these small-molecule inhibitors efficiently prevent TTR amyloid fibril formation in vitro, and are being tested for their in vivo activity. [nature.com]

Bri23 peptide prevents aggregation of APP amyloid-beta protein 42 into toxic oligomers. [uniprot.org]

The structures produced in these studies will guide in the development of inhibitors to prevent amyloid formation in Alzheimer's disease. [case.edu]

N-linked, glycosylation can prevent glycoprotein folding and full inhibition can be toxic to an individual cell. [wikivisually.com]

Colchicine has been shown to be effective in preventing acute attacks and amyloidosis in patients with FMF. The major causes of death are heart disease and renal failure. Sudden death, presumably due to arrhythmias, is common. [perthhaematology.com.au]