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

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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.

Original languageEnglish
Article number476
JournalScientific Reports
Volume8
Issue number1
DOIs
Publication statusPublished - Dec 1 2018

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Paroxysmal Hemoglobinuria
Blood Vessels
Brain
Adjustment Disorders
Neurologic Examination
Anxiety Disorders
Bipolar Disorder
Venous Thrombosis
Parkinson Disease
Psychiatry
Clone Cells
White Matter
Therapeutics

ASJC Scopus subject areas

  • General

Cite this

@article{b5584f43ba644123bb3b53e46f9c0bea,
title = "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 (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.",
author = "Wilma Barcellini and Elisa Scola and Silvia Lanfranconi and Marika Grottaroli and Francesca Binda and Bruno Fattizzo and Anna Zaninoni and Gloria Valcamonica and Cinnante, {Claudia Maria} and Carla Boschetti and Massimiliano Buoli and Altamura, {Carlo Alfredo} and Nereo Bresolin and Fabio Triulzi and Alberto Zanella and Agostino Cortelezzi",
year = "2018",
month = "12",
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doi = "10.1038/s41598-017-18936-0",
language = "English",
volume = "8",
journal = "Scientific Reports",
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T1 - Paroxysmal Nocturnal Hemoglobinuria (Pnh)

T2 - Brain Mri Ischemic Lesions in Neurologically Asymtomatic Patients

AU - Barcellini, Wilma

AU - Scola, Elisa

AU - Lanfranconi, Silvia

AU - Grottaroli, Marika

AU - Binda, Francesca

AU - Fattizzo, Bruno

AU - Zaninoni, Anna

AU - Valcamonica, Gloria

AU - Cinnante, Claudia Maria

AU - Boschetti, Carla

AU - Buoli, Massimiliano

AU - Altamura, Carlo Alfredo

AU - Bresolin, Nereo

AU - Triulzi, Fabio

AU - Zanella, Alberto

AU - Cortelezzi, Agostino

PY - 2018/12/1

Y1 - 2018/12/1

N2 - 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.

AB - 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.

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JF - Scientific Reports

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