Neurological complications of beta-thalassemia

Nemtsas, P.; Arnaoutoglou, Marianthi; Perifanis, Vassilios; Koutsouraki, Euphrosyni; Orologas, Anastasios

doi:10.1007/s00277-015-2378-z

Cited by 40 publications

(27 citation statements)

References 54 publications

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“…Neurological complications have been attributed to various factors, such as chronic hypoxia, bone marrow expansion, iron overload, and desferrioxamine neurotoxicity. It has been postulated that such β‐TM–related neurological complications may be responsible for impaired cognitive and neuropsychological functioning . However, it is not clear whether cognitive impairments can be directly attributed to neurological complications or to other educational, environmental, emotional, or personality factors associated with severe chronic illness .…”

Section: Discussionmentioning

confidence: 99%

“…It has been postulated that such ␤-TM-related neurological complications may be responsible for impaired cognitive and neuropsychological functioning. [37][38][39][40][41][42][43] However, it is not clear whether cognitive impairments can be directly attributed to neurological complications or to other educational, environmental, emotional, or personality factors associated with severe chronic illness. 15,44 Clearly, future research should carefully examine the possible associations between specific ␤-TM-related neurological complications and cognitive impairments, using advanced morphological and functional brain-imaging techniques, such as high-density event-related potentials, magnetic resonance imaging (MRI), and functional MRI.…”

Section: Discussionmentioning

confidence: 99%

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Cognitive functions in adults with β‐thalassemia major: before and after blood transfusion and comparison with healthy controls

Raz

Koren

Dan

et al. 2016

Annals of the New York Academy of Sciences

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While β-thalassemia major (β-TM)-related physiological complications have been well established, less is known about implications for neuropsychological and cognitive function. The few existing studies have focused almost exclusively on children. We evaluated cognitive function in adult β-TM patients compared to healthy controls (study 1) and in β-TM patients before and after blood transfusion (study 2). Performance intelligence quotient (IQ) was evaluated with four subtests from the Wechsler Adult Intelligence Scale (WAIS-III). Attention functions were evaluated using the online continuous performance test (OCPT). The results of study 1 revealed poorer performance of β-TM patients on three of the four intelligence subtests, with significantly lower total performance IQ scores compared with controls. The percentage of participants with abnormal performance IQ (<85) was almost five times higher in the β-TM group (58%) than in the control group (12%). In study 2, significant differences were found in OCPT performance as a function of blood transfusion. Before transfusion, patients had higher rates of omission and commission errors, slower response times (RTs), and lower RT consistency than after transfusion. As β-TM patients' life expectancy is increasing, assessment and treatment of neurocognitive functions should become an integral part of appropriate follow-up to improve patients' quality of life.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Cognitive functions in adults with β‐thalassemia major: before and after blood transfusion and comparison with healthy controls

Raz

Koren

Dan

et al. 2016

Annals of the New York Academy of Sciences

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“…As life expectancy increases, chronic hypoxia, thromboembolic phenomena, neurotoxic drugs, and other factors, can cause damage which may manifest as clinically apparent (such as cognitive impairment or peripheral neuropathy) or subclinical findings (abnormal evoked potentials) [52]. This is an important area for future preventative research, in order to optimize the level of functioning and quality of life of thalassemia patients.…”

Section: Improving Quality Of Lifementioning

confidence: 99%

Thalassemia 2016: Modern medicine battles an ancient disease

Rund

2015

American J Hematol

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Thalassemia was first clinically described nearly a century ago and treatment of this widespread genetic disease has greatly advanced during this period. DNA‐based diagnosis elucidated the molecular basis of the disease and clarified the variable clinical picture. It also paved the way for modern methods of carrier identification and prevention via DNA‐based prenatal diagnosis. Every aspect of supportive care, including safer blood supply, more regular transfusions, specific monitoring of iron overload, parenteral and oral chelation, and other therapies, has prolonged life and improved the quality of life of these patients. Significant advances have also been made in allogenic bone marrow transplantation, the only curative therapy. Recently, there has been a rejuvenated interest in studying thalassemia at the basic science level, leading to the discovery of previously unknown mechanisms leading to anemia and enabling the development of novel therapies. These will potentially improve the treatment of, and possibly cure the disease. Pathways involving activin receptors, heat shock proteins, JAK2 inhibitors and macrophage targeted therapy, among others, are being studied or are currently in clinical trials for treating thalassemia. Novel types of genetic therapies are in use or under investigation. In addition to the challenges of treating each individual patient, the longer survival of thalassemia patients has raised considerations regarding worldwide control of thalassemia, since prevention is not universally implemented. This review will trace a number of the original medical milestones of thalassemia diagnosis and treatment, as well as some of the most recent developments which may lead to innovative therapeutic modalities. Am. J. Hematol. 91:15–21, 2016. © 2015 Wiley Periodicals, Inc.

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“…Neurological complications have been attributed to various factors, such as chronic hypoxia, iron overload, and desferrioxamine neurotoxicity. [22][23][24][25][26][27][28][29] It has been postulated that such abnormalities may be responsible for impaired cognitive and neuropsychological functioning.…”

Section: Introductionmentioning

confidence: 99%

Executive function and neural activation in adults with β‐thalassemia major: an event‐related potentials study

Raz

Koren

Dan

et al. 2016

Annals of the New York Academy of Sciences

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In recent years, there has been growing interest in understanding the involvement of the nervous system and neurological complications in β-thalassemia major (β-TM). Several reports have demonstrated β-TM-related neurological abnormalities, and these have been postulated to be responsible for impaired cognitive and neuropsychological functioning. We investigated neural correlates of cognitive function in adults with β-TM and healthy controls using scalp-recorded event-related potentials (ERPs). To date, there have been no ERP studies in β-TM adult patients. We identified ERP correlates of executive function by using a complex task-switching paradigm in which participants have to quickly and effectively switch between two different task sets. The results indicated poorer cognitive performance of β-TM patients, resulting in overall higher error rates, longer response times, and increased switch costs compared with controls. Hemoglobin levels were negatively correlated with error rates and response times. Electrophysiological results indicated significant alterations in peak amplitudes of the ERP components P1, N1, and P2 in β-TM patients relative to controls. P2 amplitude correlated with hemoglobin levels. This novel investigation of executive function and related brain mechanisms and dynamics in adults with β-TM underscores the usefulness of ERP methodology as a sensitive measure for the study of neurocognitive processes in β-TM.

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Neurological complications of beta-thalassemia

Cited by 40 publications

References 54 publications

Cognitive functions in adults with β‐thalassemia major: before and after blood transfusion and comparison with healthy controls

Cognitive functions in adults with β‐thalassemia major: before and after blood transfusion and comparison with healthy controls

Thalassemia 2016: Modern medicine battles an ancient disease

Executive function and neural activation in adults with β‐thalassemia major: an event‐related potentials study

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