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Redefining the Pulvinar Sign in Fabry Disease (74 views)

Cocozza S, Russo C, Pisani A, Olivo G, Riccio E, Cervo A, Pontillo G, Feriozzi S, Veroux M, Battaglia Y, Concolino D, Pieruzzi F, Mignani R, Borrelli P, Imbriaco M, Brunetti A, Tedeschi E, Palma G

Ajnr Am J Neuroradiol (ISSN: 1936-959xelectronic, 0195-6108linking), 2017 Oct 19; N/D: N/D-N/D.

Abstract
BACKGROUND AND PURPOSE: The pulvinar sign refers to exclusive T1WI hyperintensity of the lateral pulvinar. Long considered a common sign of Fabry disease, the pulvinar sign has been reported in many pathologic conditions. The exact incidence of the pulvinar sign has never been tested in representative cohorts of patients with Fabry disease. The aim of this study was to assess the prevalence of the pulvinar sign in Fabry disease by analyzing T1WI in a large Fabry disease cohort, determining whether relaxometry changes could be detected in this region independent of the pulvinar sign positivity. MATERIALS AND METHODS: We retrospectively analyzed brain MR imaging of 133 patients with Fabry disease recruited through specialized care clinics. A subgroup of 26 patients underwent a scan including 2 FLASH sequences for relaxometry that were compared with MRI scans of 34 healthy controls. RESULTS: The pulvinar sign was detected in 4 of 133 patients with Fabry disease (3.0%). These 4 subjects were all adult men (4 of 53, 7.5% of the entire male population) with renal failure and under enzyme replacement therapy. When we tested for discrepancies between Fabry disease and healthy controls in quantitative susceptibility mapping and relaxometry maps, no significant difference emerged for any of the tested variables. CONCLUSIONS: The pulvinar sign has a significantly lower incidence in Fabry disease than previously described. This finding, coupled with a lack of significant differences in quantitative MR imaging, allows hypothesizing that selective involvement of the pulvinar is a rare neuroradiologic sign of Fabry disease.

Affiliations ▼
*** IBB - CNR Affiliation

From the Departments of Advanced Biomedical Sciences (S.C., C.R., G.O., A.C., G.P., M.I., A.B., E.T.)., From the Departments of Advanced Biomedical Sciences (S.C., C.R., G.O., A.C., G.P., M.I., A.B., E.T.) camilla_russo@hotmail.it., Public Health (A.P., E.R.), Nephrology Unit, University "Federico II," Naples, Italy., Department of Neuroscience (G.O.), Uppsala University, Uppsala, Sweden., Institute of Biostructure and Bioimaging (G.P.), National Research Council, Naples, Italy., Nephrology and Dialysis Department (S.F.), Belcolle Hospital, Viterbo, Italy., Department of Medical and Surgical Sciences and Advanced Technologies (M.V.), University Hospital of Catania, Catania, Sicily, Italy., Department of Specialized Medicine (Y.B.), Division of Nephrology and Dialysis, St. Anna Hospital-University, Ferrara, Italy., Department of Pediatrics (D.C.), University Magna Graecia, Catanzaro, Italy., Nephrology Unit (F.P.), University of Milano-Bicocca, Milan, Italy., Nephrology and Dialysis Department (R.M.), Infermi Hospital, Rimini, Italy., IRCCS SDN (P.B.), Naples, Italy.,

Details ▼
Impact factor: 3.55, 5-year impact factor: 3.888

Paper type: Journal Article,

Keywords: Fabry Disease, Pulvinar Sign,

Url: Not available.

References ▼
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* Default mode network modifications in Fabry disease: A resting-state fMRI study with structural correlations (5 views)
Cocozza S, Pontillo G, Quarantelli M, Sacca F, Riccio E, Costabile T, Olivo G, Brescia Morra V, Pisani A, Brunetti A, Tedeschi E
Hum Brain Mapp (ISSN: 1097-0193electronic, 1065-9471linking), 2018 Jan 9; N/D: N/D-N/D.
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* Author response: Alterations of functional connectivity of the motor cortex in Fabry disease: An RS-fMRI study (7 views)
Cocozza S, Pisani A, Brunetti A, Quarantelli M, Tedeschi E
Neurology (ISSN: 1526-632xelectronic, 0028-3878linking), 2017 Oct 24; 89(17): 1842-1843.
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* Cardiac sympathetic neuronal damage precedes myocardial fibrosis in patients with Anderson-Fabry disease (30 views)
Imbriaco M, Pellegrino T, Piscopo V, Petretta M, Ponsiglione A, Nappi C, Puglia M, Dell'Aversana S, Riccio E, Spinelli L, Pisani A, Cuocolo A
Eur J Nucl Med Mol Imaging (ISSN: 1619-7089electronic, 1619-7070linking, 1619-7070print), 2017 Jul 22; N/D: N/D-N/D.
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* Alterations of functional connectivity of the motor cortex in Fabry's Disease: an RS-fMRI study. Neurology (38 views)
Cocozza S, Pisani A, Olivo G, Saccà F, Ugga L, Riccio E, Migliaccio S, Brescia Morra V, Brunetti A, Quarantelli M, Tedeschi E
Neurology, 2017 May; 88: 1822-1829.
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* Relationship between left ventricular diastolic function and myocardial sympathetic denervation measured by (123)I-meta-iodobenzylguanidine imaging in Anderson-Fabry disease (70 views)
Spinelli L, Pellegrino T, Pisani A, Giudice CA, Riccio E, Imbriaco M, Salvatore M, Trimarco B, Cuocolo A
Eur J Nucl Med Mol Imaging (ISSN: 1619-7070, 1619-7089, 1619-7070print), 2016 Apr; 43(4): 729-739.
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* First experience of simultaneous PET/MRI for the early detection of cardiac involvement in patients with Anderson-Fabry disease (78 views)
Nappi C, Altiero M, Imbriaco M, Nicolai E, Giudice CA, Aiello M, Diomiaiuti CT, Pisani A, Spinelli L, Cuocolo A
Eur J Nucl Med Mol Imaging (ISSN: 1619-7070, 1619-7089, 0340-6997), 2015; 42(7): 1025-1031.
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* A thermodynamic assay to test pharmacological chaperones for Fabry disease (73 views)
Andreotti G, Citro V, Correra A, Cubellis MV
Biochim Biophys Acta (ISSN: 0304-4165, 0006-3002, 0925-4439), 2014 Mar; 1840(3): 1214-1224.
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* Fabry_CEP: a tool to identify Fabry mutations responsive to pharmacological chaperones (99 views)
Cammisa M, Correra A, Andreotti G, Cubellis MV
Orphanet Journal Of Rare Diseases (ISSN: 1750-1172), 2013 Jul 24; 8: 111-111.
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* Role of cardiac MRI in evaluating patients with Anderson-Fabry disease: Assessing cardiac effects of long-term enzyme replacement therapy (57 views)
Messalli G, Imbriaco M, Avitabile G, Russo R, Iodice D, Spinelli L, Dellegrottaglie S, Cademartiri F, Salvatore M, Pisani A
Radiol Med (ISSN: 0033-8362, 1826-6983, 1826-6983electronic), 2012 Feb; 117(1): 19-28.
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* Therapy of Fabry disease with pharmacological chaperones: from in silico predictions to in vitro tests (71 views)
Andreotti G, Citro V, De Crescenzo A, Orlando P, Cammisa M, Correra A, Cubellis MV
Orphanet Journal Of Rare Diseases (ISSN: 1750-1172), 2011 Oct 17; 6: 66-66.
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* Prediction of the responsiveness to pharmacological chaperones: lysosomal human alpha-galactosidase, a case of study (86 views)
Andreotti G, Guarracino MR, Cammisa M, Correra A, Cubellis MV
Orphanet Journal Of Rare Diseases (ISSN: 1750-1172), 2010 Dec 8; 5: 36-36.
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* Cardiac magnetic resonance imaging illustrating Anderson-Fabry disease progression (56 views)
Imbriaco M, Messalli G, Avitabile G, Cuocolo A, Maurea S, Soscia F, Pisani A
Br J Radiol (ISSN: 0007-1285, 1748-880xelectronic, 0007-1285linking), 2010 Dec; 83(996): e249-e249.
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* Effects of enzyme-replacement therapy in patients with Anderson-Fabry disease: a prospective long-term cardiac magnetic resonance imaging study (74 views)
Imbriaco M, Pisani A, Spinelli L, Cuocolo A, Messalli G, Capuano E, Marmo M, Liuzzi R, Visciano B, Cianciaruso B, Salvatore M
Heart (ISSN: 1355-6037, 1468-201x), 2009 Jul; 95(13): 1103-1107.
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* Cardiac performance during exercise in patients with Fabry's disease (61 views)
Spinelli L, Nicolai E, Acampa W, Imbriaco M, Pisani A, Rao MA, Scopacasa F, Cianciaruso B, De Luca N, Cuocolo A
European Journal Of Clinical Investigation (ISSN: 0014-2972, 1365-2362), 2008 Dec; 38(12): 910-917.
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* MRI characterization of myocardial tissue in patients with Fabry's disease (63 views)
Imbriaco M, Spinelli L, Cuocolo A, Maurea S, Sica G, Quarantelli M, Pisani A, Liuzzi R, Cianciaruso B, Sabbatini M, Salvatore M
Ajr Am J Roentgenol (ISSN: 1546-3141, 0361-803x, 1546-3141electronic), 2007 Mar; 188(3): 850-853.
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* Myocardical Tissue Chatacterization By Magnetic Resonance Imaging In Patients With Anderson Fabry Disease (71 views)
Imbriaco M, Spinelli L, Cuocolo A, Maurea S, Sica G, Quarantelli M, Pisani A, Liuzzi R, Sabatini M, Salvatore M
American Journal Of Roentgenology, 2006; 0: N/D-N/D.
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16 Records (11 excluding Abstracts and Conferences).
Total impact factor: 75.454 (55.683 excluding Abstracts and Conferences).
Total 5-year impact factor: 71.724 (48.825 excluding Abstracts and Conferences).



Your bibliography query: (([btitle, keywords, abstract] FABRY AND [btitle, keywords, abstract] DISEASE)) AND NOT [id] = 53619



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