Paroxysmal Nocturnal Hemoglobinuria (Pnh): Brain Mri Ischemic Lesions In Neurologically Asymtomatic Patients

Abstract This study investigated for the first time brain ischemic involvement in 19 consecutive neurologically asymptomatic PNH patients by non-enhanced cerebral MRI, and by intracranial arterial and venous angio-MRI. Eleven cases (58%, 7 aged <65) showed pathological findings: 9 white matter (W...

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Autores principales:	Wilma Barcellini, Elisa Scola, Silvia Lanfranconi, Marika Grottaroli, Francesca Binda, Bruno Fattizzo, Anna Zaninoni, Gloria Valcamonica, Claudia Maria Cinnante, Carla Boschetti, Massimiliano Buoli, Carlo Alfredo Altamura, Nereo Bresolin, Fabio Triulzi, Alberto Zanella, Agostino Cortelezzi
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2018
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/bc52bf4ad1bc4926aa228fea99dfcf3e
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spelling	oai:doaj.org-article:bc52bf4ad1bc4926aa228fea99dfcf3e2021-12-02T15:08:03ZParoxysmal Nocturnal Hemoglobinuria (Pnh): Brain Mri Ischemic Lesions In Neurologically Asymtomatic Patients10.1038/s41598-017-18936-02045-2322https://doaj.org/article/bc52bf4ad1bc4926aa228fea99dfcf3e2018-01-01T00:00:00Zhttps://doi.org/10.1038/s41598-017-18936-0https://doaj.org/toc/2045-2322Abstract This study investigated for the first time brain ischemic involvement in 19 consecutive neurologically asymptomatic PNH patients by non-enhanced cerebral MRI, and by intracranial arterial and venous angio-MRI. Eleven cases (58%, 7 aged <65) showed pathological findings: 9 white matter (WM) abnormalities related to chronic ischemic small vessel disease, 2 a focal abnormality >5 mm, and 5 cases a score >4 by the age-related white matter changes (ARWMC) scale. Compared with age and sex-matched controls (1:2 ratio), patients showed an increased frequency of periventricular WM vascular degeneration (32% versus 5.2%, p = 0.04) and of severe lesions (ARWMC scale score >4) (26% versus 2.6%, p = 0.05), and a higher overall ARWMC scale score (3.5 ± 1.07 versus 2.0 ± 0.8, mean ± SD, p < 0.0001). Notably, vascular abnormalities suspected for prior partial venous thrombosis, were observed in PNH cases only. MRI lesions were not related to blood counts, hemolytic markers, clone size, disease duration, and therapy with eculizumab. Neurological examination was unremarkable in all patients but one (Parkinson disease). Psychiatric assessment revealed a case of generalized anxiety disorder, 1 bipolar disorder type 2, and 1 adjustment disorder. In conclusion, brain MRI may be useful at diagnosis and during the course of the disease to explore subclinical neurological involvement.Wilma BarcelliniElisa ScolaSilvia LanfranconiMarika GrottaroliFrancesca BindaBruno FattizzoAnna ZaninoniGloria ValcamonicaClaudia Maria CinnanteCarla BoschettiMassimiliano BuoliCarlo Alfredo AltamuraNereo BresolinFabio TriulziAlberto ZanellaAgostino CortelezziNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 8, Iss 1, Pp 1-8 (2018)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Wilma Barcellini Elisa Scola Silvia Lanfranconi Marika Grottaroli Francesca Binda Bruno Fattizzo Anna Zaninoni Gloria Valcamonica Claudia Maria Cinnante Carla Boschetti Massimiliano Buoli Carlo Alfredo Altamura Nereo Bresolin Fabio Triulzi Alberto Zanella Agostino Cortelezzi Paroxysmal Nocturnal Hemoglobinuria (Pnh): Brain Mri Ischemic Lesions In Neurologically Asymtomatic Patients
description	Abstract This study investigated for the first time brain ischemic involvement in 19 consecutive neurologically asymptomatic PNH patients by non-enhanced cerebral MRI, and by intracranial arterial and venous angio-MRI. Eleven cases (58%, 7 aged <65) showed pathological findings: 9 white matter (WM) abnormalities related to chronic ischemic small vessel disease, 2 a focal abnormality >5 mm, and 5 cases a score >4 by the age-related white matter changes (ARWMC) scale. Compared with age and sex-matched controls (1:2 ratio), patients showed an increased frequency of periventricular WM vascular degeneration (32% versus 5.2%, p = 0.04) and of severe lesions (ARWMC scale score >4) (26% versus 2.6%, p = 0.05), and a higher overall ARWMC scale score (3.5 ± 1.07 versus 2.0 ± 0.8, mean ± SD, p < 0.0001). Notably, vascular abnormalities suspected for prior partial venous thrombosis, were observed in PNH cases only. MRI lesions were not related to blood counts, hemolytic markers, clone size, disease duration, and therapy with eculizumab. Neurological examination was unremarkable in all patients but one (Parkinson disease). Psychiatric assessment revealed a case of generalized anxiety disorder, 1 bipolar disorder type 2, and 1 adjustment disorder. In conclusion, brain MRI may be useful at diagnosis and during the course of the disease to explore subclinical neurological involvement.
format	article
author	Wilma Barcellini Elisa Scola Silvia Lanfranconi Marika Grottaroli Francesca Binda Bruno Fattizzo Anna Zaninoni Gloria Valcamonica Claudia Maria Cinnante Carla Boschetti Massimiliano Buoli Carlo Alfredo Altamura Nereo Bresolin Fabio Triulzi Alberto Zanella Agostino Cortelezzi
author_facet	Wilma Barcellini Elisa Scola Silvia Lanfranconi Marika Grottaroli Francesca Binda Bruno Fattizzo Anna Zaninoni Gloria Valcamonica Claudia Maria Cinnante Carla Boschetti Massimiliano Buoli Carlo Alfredo Altamura Nereo Bresolin Fabio Triulzi Alberto Zanella Agostino Cortelezzi
author_sort	Wilma Barcellini
title	Paroxysmal Nocturnal Hemoglobinuria (Pnh): Brain Mri Ischemic Lesions In Neurologically Asymtomatic Patients
title_short	Paroxysmal Nocturnal Hemoglobinuria (Pnh): Brain Mri Ischemic Lesions In Neurologically Asymtomatic Patients
title_full	Paroxysmal Nocturnal Hemoglobinuria (Pnh): Brain Mri Ischemic Lesions In Neurologically Asymtomatic Patients
title_fullStr	Paroxysmal Nocturnal Hemoglobinuria (Pnh): Brain Mri Ischemic Lesions In Neurologically Asymtomatic Patients
title_full_unstemmed	Paroxysmal Nocturnal Hemoglobinuria (Pnh): Brain Mri Ischemic Lesions In Neurologically Asymtomatic Patients
title_sort	paroxysmal nocturnal hemoglobinuria (pnh): brain mri ischemic lesions in neurologically asymtomatic patients
publisher	Nature Portfolio
publishDate	2018
url	https://doaj.org/article/bc52bf4ad1bc4926aa228fea99dfcf3e
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Paroxysmal Nocturnal Hemoglobinuria (Pnh): Brain Mri Ischemic Lesions In Neurologically Asymtomatic Patients

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