Cognitive involvement in beta-thalassaemia is strikingly controversial and poorly studied in adulthood. This multicentre prospective study investigated 74 adult neurologically-asymptomatic beta-thalassaemia patients (mean-age 34 center dot 5 +/- 10 center dot 3 years; 53 transfusion-dependent [TDT], 21 non-transfusion dependent [NTDT]) and 45 healthy volunteers (mean-age 33 center dot 9 +/- 10 center dot 7 years). Participants underwent testing with Wechsler Adult Intelligence Scale-Fourth Edition (WAIS-IV), Brief Psychiatric Rating Scale (BPRS) and multiparametric brain 3T-magnetic resonance imaging (MRI) for parenchymal, vascular and iron content evaluation. Patients had lower Full-Scale Intelligence Quotient (FSIQ) than controls (75 center dot 5 +/- 17 center dot 9 vs. 97 center dot 4 +/- 18 center dot 1, P < 0 center dot 0001) even after correction for education level. Compared to TDT, NTDT showed a trend of higher FSIQ (P = 0 center dot 08) but a similar cognitive profile at WAIS-subtests. FSIQ correlated with total and indirect bilirubin (P P = 0 center dot 002, respectively); no correlation was found with splenectomy, intracranial MRI/magnetic resonance-angiography findings, brain tissue iron content or other disease-related clinical/laboratory/treatment data. FSIQ did not correlate with BPRS scores, although the latter were higher among patients (28 center dot 74 +/- 3 center dot 1 vs. 27 center dot 29 +/- 4 center dot 8, P = 0 center dot 01) mainly because of increased depression and anxiety levels. Occupation rate was higher among controls (84 center dot 4% vs. 64 center dot 9%, P = 0 center dot 004) and correlated with higher FSIQ (P = 0 center dot 001) and education level (P = 0 center dot 001). In conclusion, Italian adult beta-thalassaemia patients seem to present a characteristic cognitive profile impairment and an increased rate of psychological disorders with possible profound long-term socio-economic consequences.
Brain functional impairment in beta-thalassaemia: the cognitive profile in Italian neurologically asymptomatic adult patients in comparison to the reported literature
Tartaglione I.;Casale M.;Russo A. G.;Cirillo M.;Esposito F.;Gritti P.;Perrotta S.
2019
Abstract
Cognitive involvement in beta-thalassaemia is strikingly controversial and poorly studied in adulthood. This multicentre prospective study investigated 74 adult neurologically-asymptomatic beta-thalassaemia patients (mean-age 34 center dot 5 +/- 10 center dot 3 years; 53 transfusion-dependent [TDT], 21 non-transfusion dependent [NTDT]) and 45 healthy volunteers (mean-age 33 center dot 9 +/- 10 center dot 7 years). Participants underwent testing with Wechsler Adult Intelligence Scale-Fourth Edition (WAIS-IV), Brief Psychiatric Rating Scale (BPRS) and multiparametric brain 3T-magnetic resonance imaging (MRI) for parenchymal, vascular and iron content evaluation. Patients had lower Full-Scale Intelligence Quotient (FSIQ) than controls (75 center dot 5 +/- 17 center dot 9 vs. 97 center dot 4 +/- 18 center dot 1, P < 0 center dot 0001) even after correction for education level. Compared to TDT, NTDT showed a trend of higher FSIQ (P = 0 center dot 08) but a similar cognitive profile at WAIS-subtests. FSIQ correlated with total and indirect bilirubin (P P = 0 center dot 002, respectively); no correlation was found with splenectomy, intracranial MRI/magnetic resonance-angiography findings, brain tissue iron content or other disease-related clinical/laboratory/treatment data. FSIQ did not correlate with BPRS scores, although the latter were higher among patients (28 center dot 74 +/- 3 center dot 1 vs. 27 center dot 29 +/- 4 center dot 8, P = 0 center dot 01) mainly because of increased depression and anxiety levels. Occupation rate was higher among controls (84 center dot 4% vs. 64 center dot 9%, P = 0 center dot 004) and correlated with higher FSIQ (P = 0 center dot 001) and education level (P = 0 center dot 001). In conclusion, Italian adult beta-thalassaemia patients seem to present a characteristic cognitive profile impairment and an increased rate of psychological disorders with possible profound long-term socio-economic consequences.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
