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Condition: Amyloidosis

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Total 63 results found since Jan 2013.

Cerebral embolism secondary to cardiac amyloidosis: A case report and literature review.
Authors: Zhang XD, Liu YX, Yan XW, Fang LG, Fang Q, Zhao DC, Wang YN Abstract Cardiac amyloidosis (CA) describes a group of heterogeneous diseases that are characterized by the extracellular fibril deposition of amyloid protein in the myocardium. The abnormal protein is usually derived from light-chain amyloidosis, mutant transthyretin amyloidosis and wild-type transthyretin. Patients with ischemic strokes and amyloidosis have been sporadically reported, however, they are not well summarized. In the present study, a case of cerebral ischemic stroke, secondary to CA was described. This patient presented with dyspnea...
Source: Experimental and Therapeutic Medicine - December 20, 2017 Category: General Medicine Tags: Exp Ther Med Source Type: research

Inotropic myocardial reserve deficiency is the predominant feature of exercise haemodynamics in cardiac amyloidosis
ConclusionsCardiac amyloid deposits are associated with severely reduced inotropic myocardial reserve and increased left and right ventricular filling pressures during exercise. Furthermore, CA subjects have severely reduced PAC, which may contribute to right heart failure and reduced exercise capacity.
Source: European Journal of Heart Failure - August 24, 2017 Category: Cardiology Authors: Tor Skibsted Clemmensen, Henning M ølgaard, Jens Sörensen, Hans Eiskjaer, Niels Frost Andersen, Søren Mellemkjaer, Mads Jønsson Andersen, Lars Poulsen Tolbod, Hendrik J. Harms, Steen Hvitfeldt Poulsen Tags: Original Article Source Type: research

Unveiling transthyretin cardiac amyloidosis and its predictors among elderly patients with severe aortic stenosis undergoing transcatheter aortic valve replacement
ConclusionsTransthyretin cardiac amyloidosis is prevalent in 16% of patients with severe calcific AS undergoing TAVR and is associated with a severe AS phenotype of low-flow low-gradient with mildly reduced ejection fraction. Average tissue Doppler mitral annular S ’ of <  6 cm/s may be a sensitive measure that should prompt a confirmatory99mTc-PYP scan and subsequent testing for ATTR-CA. Prospective assessment of outcomes after TAVR is needed in patients with and without ATTR-CA.
Source: European Heart Journal - August 1, 2017 Category: Cardiology Source Type: research

Relationship between aetiology and left ventricular systolic dysfunction in hypertrophic cardiomyopathy
Conclusions In adults with HCM, LV systolic dysfunction is more frequent in those with rare phenocopies. When combined with age at presentation, it is a marker for specific aetiologies and is associated with poorer long-term survival.
Source: Heart - January 26, 2017 Category: Cardiology Authors: Rosmini, S., Biagini, E., O'Mahony, C., Bulluck, H., Ruozi, N., Lopes, L. R., Guttmann, O., Reant, P., Quarta, C. C., Pantazis, A., Tome-Esteban, M., Mckenna, W. J., Rapezzi, C., Elliott, P. M. Tags: Hypertrophic cardiomyopathy, Drugs: cardiovascular system, Echocardiography, Interventional cardiology, Clinical diagnostic tests, Epidemiology, Metabolic disorders Heart failure and cardiomyopathies Source Type: research

Long-term utility and complication profile of open craniotomy for biopsy in patients with idiopathic encephalitis
Publication date: Available online 12 December 2016 Source:Journal of Clinical Neuroscience Author(s): Kalil G. Abdullah, Yin Li, Prateek Agarwal, Nikhil R. Nayak, Jayesh P. Thawani, Ramani Balu, Timothy H. Lucas Neurosurgeons are often asked to perform open biopsy for diagnosis of encephalitis after medical investigations are non-diagnostic. These patients may be critically ill with multiple comorbidities. Patients and their families often request data regarding the success rates and complication profile of biopsy, but minimal literature exists in this area. Retrospective chart review of all patients undergoing open brai...
Source: Journal of Clinical Neuroscience - December 11, 2016 Category: Neuroscience Source Type: research

Early Magnetic Resonance Imaging and Cognitive Markers of Hereditary Cerebral Amyloid Angiopathy Brief Report
Background and Purpose—Early markers for cerebral amyloid angiopathy are largely unknown. We aimed to identify which magnetic resonance imaging (MRI) (performed at 7 and 3T) and cognitive markers are an early sign in (pre) symptomatic subjects with hereditary cerebral hemorrhage with amyloidosis-Dutch type.Methods—Twenty-seven DNA-proven Dutch-type mutation carriers (15 symptomatic and 12 presymptomatic) (mean age of 45.9 years) and 33 controls (mean age of 45.6 years) were included. 7T and 3T MRI was performed, cerebral amyloid angiopathy and small-vessel disease type MRI markers were estimated, and cognitive performa...
Source: Stroke - November 27, 2016 Category: Neurology Authors: Sanneke van Rooden, Anna M. van Opstal, Gerda Labadie, Gisela M. Terwindt, Marieke J.H. Wermer, Andrew G. Webb, Huub A.M. Middelkoop, Steven M. Greenberg, Jeroen van der Grond, Mark A. van Buchem Tags: Magnetic Resonance Imaging (MRI), Cerebrovascular Disease/Stroke, Cognitive Impairment Brief Reports Source Type: research

PiB-PET detects transthyretin-related cerebral amyloid angiopathy
Transthyretin (TTR) is an amyloidogenic protein synthesized primarily (>95%) by the liver and, to a lesser extent, by the choroid plexuses and retinal pigment epithelium. Hereditary TTR amyloidosis (ATTR) is a multisystem disorder that may manifest with 3 main clinical phenotypes: familial amyloid polyneuropathy (FAP), familial amyloid cardiomyopathy, and familial leptomeningeal amyloidosis.1 TTR-FAP is the most common presentation. More than 130 autosomal dominant mutations cause ATTR, with the Val30Met mutation being the most frequent variant worldwide: prevalence of up to 1:1,000 in endemic regions of Portugal, Swede...
Source: Neurology - August 21, 2016 Category: Neurology Authors: Cavallaro, T., Klunk, W. Tags: PET, Peripheral neuropathy, All Cerebrovascular disease/Stroke, All Genetics EDITORIALS Source Type: research

Cerebral amyloid angiopathy in posttransplant patients with hereditary ATTR amyloidosis
Conclusions: TFNE due to ATTR-type CAA occurred frequently in posttransplant patients with long disease durations. 11C-PiB-PET is a useful diagnostic tool for ATTR-type CAA. ATTR amyloid deposition in the CNS, as measured by PiB-PET, was detected approximately 10 years before onset of TFNE.
Source: Neurology - August 21, 2016 Category: Neurology Authors: Sekijima, Y., Yazaki, M., Oguchi, K., Ezawa, N., Yoshinaga, T., Yamada, M., Yahikozawa, H., Watanabe, M., Kametani, F., Ikeda, S.-i. Tags: PET, Peripheral neuropathy, All Cerebrovascular disease/Stroke, All Genetics ARTICLE Source Type: research

Selective astrocytic endothelin-1 overexpression contributes to dementia associated with ischemic stroke by exaggerating astrocyte-derived amyloid secretion
g & Sookja K Chung
Source: Journal of Cerebral Blood Flow and Metabolism - September 30, 2015 Category: Neuroscience Authors: Victor K L HungPatrick K K YeungAngela K W LaiMaggie C Y HoAmy C Y LoKevin C ChanEd X K WuStephen S M ChungChi W CheungSookja K Chung Tags: Alzheimer’s disease amyloidosis astrocytes endothelin-1 ischemic stroke Source Type: research

Prognostic Value of Late Gadolinium Enhancement Cardiovascular Magnetic Resonance in Cardiac Amyloidosis.
CONCLUSIONS: -There is a continuum of cardiac involvement in systemic AL and ATTR amyloidosis. Transmural LGE is determined reliably by PSIR and represents advanced cardiac amyloidosis. The PSIR technique provides incremental information on outcome even after adjusting for known prognostic factors. PMID: 26362631 [PubMed - as supplied by publisher]
Source: Circulation - September 11, 2015 Category: Cardiology Authors: Fontana M, Pica S, Reant P, Abdel-Gadir A, Treibel TA, Banypersad SM, Maestrini V, Barcella W, Rosmini S, Bulluck H, Sayed RH, Patel K, Mahmood S, Bucciarelli-Ducci C, Whelan CJ, Herrey AS, Lachmann HJ, Wechalekar AD, Manisty CH, Schelbert EB, Kellman P, Tags: Circulation Source Type: research

Selective astrocytic endothelin-1 overexpression contributes to dementia associated with ischemic stroke by exaggerating astrocyte-derived amyloid secretion
g & Sookja K Chung
Source: Journal of Cerebral Blood Flow - June 24, 2015 Category: Neurology Authors: Victor K L HungPatrick K K YeungAngela K W LaiMaggie C Y HoAmy C Y LoKevin C ChanEd X K WuStephen S M ChungChi W CheungSookja K Chung Tags: Alzheimer’s disease amyloidosis astrocytes endothelin-1 ischemic stroke Source Type: research

Brain Microbleeds 12 Years after Orthotopic Liver Transplantation in Val30Met Amyloidosis
Unexplained focal neurologic episodes (FNEs) can occur in patients with transthyretin-related familial amyloidotic polyneuropathy (TTR-FAP) after orthotopic liver transplantation (OLT). A patient with Val30Met FAP underwent OLT at age 34 years. Twelve years after transplantation, she presented with recurrent FNEs lasting from 10 minutes to 8 hours each, with nonuniform deficitary clinical features and variably associated with headache. Magnetic resonance imaging showed multiple brain microbleeds and diffuse contrast enhancement of the craniospinal leptomeninges consistent with amyloid deposits.
Source: Journal of Stroke and Cerebrovascular Diseases - March 20, 2015 Category: Neurology Authors: Fabrizio Salvi, Francesca Pastorelli, Rosaria Plasmati, Cristina Morelli, Claudio Rapezzi, Andrea Bianchi, Mario Mascalchi Tags: Case Report Source Type: research

Transthyretin-type cerebral amyloid angiopathy: a serious complication in post-transplant patients with familial amyloid polyneuropathy
Liver transplantation is a well-established treatment for transthyretin (TTR)-type familial amyloid polyneuropathy (TTR-FAP).1 According to data in the Familial Amyloidotic Polyneuropathy World Transplant Registry (http://www.fapwtr.org/ram_fap.htm), more than 2000 liver transplantations have been performed to date in 19 countries. Transplantation replaces the variant TTR gene with the wild-type gene in the liver, the main source of serum circulating TTR. The serum concentration of variant TTR decreases rapidly, reaching almost zero after the operation. The effects of liver transplantation on neuropathy are evident as its ...
Source: Journal of Neurology, Neurosurgery and Psychiatry - January 14, 2015 Category: Neurosurgery Authors: Sekijima, Y. Tags: Genetics, Immunology (including allergy), Drugs: CNS (not psychiatric), Epilepsy and seizures, Neuromuscular disease, Peripheral nerve disease, Stroke, Ophthalmology Editorial commentaries Source Type: research

Light chain amyloidosis: Experience in a tertiary hospital: 2005–2013
Conclusions Cardiac involvement is the major determinant of prognosis. Yield of involved organ biopsy is high (100% heart biopsies). Antineoplastic treatment with bortezomib and/or autologous bone marrow transplantation achieves hematological responses with improvements in organ impairment.
Source: Revista Clinica Espanola - November 27, 2014 Category: Internal Medicine Source Type: research

Targeting S100B in Cerebral Ischemia and in Alzheimer's Disease.
Authors: Mori T, Asano T, Town T Abstract S100B is an EF-hand calcium-binding protein that exerts both intracellular and extracellular effects on a variety of cellular processes. The protein is predominantly expressed in the central nervous system by astrocytes, both physiologically and during the course of neurological disease. In the healthy adult brain and during development, constitutive S100B expression acts as a trophic factor to drive neurite extension and to referee neuroplasticity. Yet, when induced during central nervous system disease, the protein can take on maladaptive roles and thereby exacerbate brai...
Source: Cardiovascular Psychiatry and Neurology - November 25, 2014 Category: Psychiatry Tags: Cardiovasc Psychiatry Neurol Source Type: research