High incidence of silent cerebral infarcts in adult patients with beta thalassemia major

Pazgal, Idit; Inbar, Edna; Cohen, Maya; Shpilberg, Ofer; Stark, Pinhas

doi:10.1016/j.thromres.2016.06.010

Cited by 24 publications

(25 citation statements)

References 23 publications

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“…Patients with non‐sickle anemia are not commonly thought to be at risk for neurological injury, however, our analysis showed complete overlap between SCD and non‐sickle anemic patients. Much like patients with SCD, thalassemia patients are at risk for silent strokes, cerebrovascular disease, and cognitive decline . Studies on sickle cell mice have reported increased hypoxic injury thought to be specific to the unique biology of sickle red blood cells .…”

Section: Discussionmentioning

confidence: 99%

Anemia predicts lower white matter volume and cognitive performance in sickle and non‐sickle cell anemia syndrome

et al. 2019

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Severe chronic anemia is an independent predictor of overt stroke, white matter damage, and cognitive dysfunction in the elderly. Severe anemia also predisposes to white matter strokes in young children, independent of the anemia subtype. We previously demonstrated symmetrically decreased white matter (WM) volumes in patients with sickle cell disease (SCD). In the current study, we investigated whether patients with non-sickle anemia also have lower WM volumes and cognitive dysfunction. Magnetic Resonance Imaging was performed on 52 clinically asymptomatic SCD patients (age = 21.4 ± 7.7; F = 27, M = 25; hemoglobin = 9.6 ± 1.6 g/dL), 26 non-sickle anemic patients (age = 23.9 ± 7.9; F = 14, M = 12; hemoglobin = 10.8 ± 2.5 g/dL) and 40 control subjects (age = 27.7 ± 11.3; F = 28, M = 12; hemoglobin = 13.4 ± 1.3 g/dL). Voxel-wise changes in WM brain volumes were compared to hemoglobin levels to identify brain regions that are vulnerable to anemia. White matter volume was diffusely lower in deep, watershed areas proportionally to anemia severity. After controlling for age, sex, and hemoglobin level, brain volumes were independent of disease. WM volume loss was associated with lower Full Scale Intelligence Quotient (FSIQ; P = .0048; r 2 = .18) and an abnormal burden of silent cerebral infarctions (P = .029) in males, but not in females.Hemoglobin count and cognitive measures were similar between subjects with and without white-matter hyperintensities. The spatial distribution of volume loss suggests chronic hypoxic cerebrovascular injury, despite compensatory hyperemia. Neurocognitive consequences of WM volume changes and silent cerebral infarctionwere strongly sexually dimorphic. Understanding the possible neurological consequences of chronic anemia may help inform our current clinical practices.

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Section: Discussionmentioning

confidence: 99%

Anemia predicts lower white matter volume and cognitive performance in sickle and non‐sickle cell anemia syndrome

et al. 2019

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“…Lesions were isolated/single in more than 50% of cases (Taher et al , ), rather small (mean‐size 3·07 ± 2·81 mm) (Karimi et al , ), with very few lesions above 15 mm of maximal diameter (Taher et al , ; Karimi et al , ), none with signal characteristics of acute ischaemia (i.e. positive on DWI) (Karimi et al , ; Pazgal et al , ). Beside blood transfusion regimens, several clinical (e.g.…”

Section: Discussionmentioning

confidence: 99%

“…Asymptomatic β‐thalassaemia patients have been repeatedly shown to present poor cognitive performances compared to normal subjects, suggesting a possible and early involvement of the brain even in the absence of overt strokes (Orsini et al , ; Monastero et al , ; Economou et al , ; Elalfy et al , ). Moreover, MRI studies have identified the common presence of vascular‐like white matter hyperintensities among young asymptomatic patients, although findings varied hugely from 0% (Teli et al , ) to more than 60% (Taher et al , ; Pazgal et al , ), possibly due to the different magnetic field strength of the scanners (from 0·5 to 3·0 T), sample size, age at MRI and treatment regimens. The remarkably wide range of lesion rate, along with the lack of control groups and the high number of disease‐related aspects that have been variably associated with brain involvement, do not currently allow us to determine whether β‐thalassaemia patients (or specific subgroups) present an increased rate of overt or silent cerebrovascular events that can be non‐invasively identified with routine brain and intracranial vascular MR evaluation.…”

Section: Introductionmentioning

confidence: 99%

No evidence of increased cerebrovascular involvement in adult neurologically‐asymptomatic β‐Thalassaemia. A multicentre multimodal magnetic resonance study

Tartaglione¹,

Russo

Elefante

et al. 2019

Br J Haematol

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Summary Multi‐factorial causes jeopardize brain integrity in β‐thalassaemia. Intracranial parenchymal and vascular changes have been reported among young β‐thalassaemia patients but conventional magnetic resonance imaging (MRI) findings are contradictory making early MRI and magnetic resonance angiography (MRA)/venography monitoring a matter of debate. This study prospectively investigated 75 neurologically asymptomatic β‐thalassaemia patients (mean‐age 35·2 ± 10·7 years; 52/75 transfusion‐dependent; 41/75 splenectomised) using a 3T magnetic resonance scanner; clinical, laboratory and treatment data were also collected. White matter ischaemic‐like abnormalities, intracranial artery stenoses, aneurysms and sinus venous thrombosis were compared between patients and 56 healthy controls (mean‐age 33·9 ± 10·8 years). No patient or control showed silent territorial or lacunar strokes, intracranial artery stenoses or signs of sinus thrombosis. White matter lesions were found both in patients (35/75, 46·7%) and controls (28/56, 50·0%), without differences in terms of number (4·0 ± 10·6 vs. 4·6 ± 9·1, P = 0·63), size and Fazekas’ Score. Intracranial aneurysms did not differ between patients and controls for incidence rate (7/75, 9·3% vs. 5/56, 8·9%), size and site. Vascular and parenchymal abnormality rate did not differ according to treatments or clinical phenotype. According to this study, asymptomatic β‐thalassaemia patients treated according to current guidelines do not seem to carry an increased risk of brain and intracranial vascular changes, thus weakening recommendations for regular brain MRI monitoring.

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“…Several reports have demonstrated thalassaemia-related brain damage and neurological abnormalities; e.g. higher incidence of silent ischaemic lesions, thromboembolic events, white matter lesions, intracranial artery stenosis, aneurysms and abnormality in sensory evoked potentials (Incorpora et al, 1999;Taher et al, 2006;Zafeiriou et al, 2006;Karimi et al, 2008Karimi et al, , 2016Musallam et al, 2011Musallam et al, , 2012Qiu et al, 2014;Nemtsas et al, 2015;Pazgal et al, 2016). It has been postulated that such abnormalities may account for cognitive and neuropsychological dysfunction in patients.…”

Section: Discussionmentioning

confidence: 99%

Attention, response inhibition and brain event‐related potential alterations in adults with beta‐thalassaemia major

Raz¹,

Koren

Levin

2019

Br J Haematol

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Summary We investigated neural correlates of cognitive function in adults with beta thalassaemia major (β‐TM) compared to healthy controls using scalp‐recorded event‐related potentials (ERPs). Event‐related potential studies in the field of β‐TM are scarce and mostly limited to children. A stop‐signal task was used to evaluate indices of attention and response inhibition function, considered to be the hallmark of executive control. Correlations between task performance, ERPs and haemoglobin were also examined. Results showed impaired cognitive performance in β‐TM patients, as indicated by longer response times than controls. Haemoglobin was negatively correlated with response times to Go stimuli. Electrophysiological results indicated significant β‐TM‐related alterations in neuronal activity, reflected in greater peak amplitudes of several task‐related ERP components. A possible interpretation of these ERP results is that β‐TM patients need to recruit additional brain resources when dealing with cognitive challenge. Significant correlations were found between levels of haemoglobin and amplitude of all ERP components; the lower the haemoglobin, the more pronounced the ERPs amplitude. The present study represents a novel investigation of cognitive function and related brain dynamics in β‐TM in adult. Integrating neuropsychological assessment and interventions into traditional disease management, may be imperative in achieving a better quality of life for these patients.

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High incidence of silent cerebral infarcts in adult patients with beta thalassemia major

Cited by 24 publications

References 23 publications

Anemia predicts lower white matter volume and cognitive performance in sickle and non‐sickle cell anemia syndrome

Anemia predicts lower white matter volume and cognitive performance in sickle and non‐sickle cell anemia syndrome

No evidence of increased cerebrovascular involvement in adult neurologically‐asymptomatic β‐Thalassaemia. A multicentre multimodal magnetic resonance study

Attention, response inhibition and brain event‐related potential alterations in adults with beta‐thalassaemia major

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