A rare case of Moyamoya syndrome in a β-thalassemia major patient

Inati, Adlette; Tourjuman, O. El; Bizri, Diana; Dhayni, Raghida M.; Abbas, Hussein A.; Tfaili, Hassan; Akouri, Grace; Beydoun, Ahmad

doi:10.1016/j.bcmd.2013.05.005

Cited by 6 publications

(2 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Moyamoya syndrome (MMS), occurs in a wide range of clinical conditions including hemoglobinopathies, especially sickle cell disease (SCD). 10 , 11 Few reports of MMS have been reported in TDT, 12 , 13 but more commonly in NTDT, 14 , 15 , 16 and rarely in Eβ‐thalassemia. 17 , 18 …”

Section: Introductionmentioning

confidence: 99%

Moyamoya syndrome in a child with HbEβ‐thalassemia

Zahra

Al‐Abboh

Habeeb

et al. 2022

Clinical Case Reports

View full text Add to dashboard Cite

show abstract

Section: Introductionmentioning

confidence: 99%

Moyamoya syndrome in a child with HbEβ‐thalassemia

Zahra

Al‐Abboh

Habeeb

et al. 2022

Clinical Case Reports

View full text Add to dashboard Cite

show abstract

“…In addition, both studies (the present one and that reported by Musallam et al, 2011) did not observe cases of moyamoya disease, suggesting a rather low risk, at least among asymptomatic b-thalassaemia patients. This observation seems to relegate previously reported cohorts (Mukherjee, 1995;Sanefuji et al, 2006;G€ oksel et al, 2010;Oberoi et al, 2010;Ray & Rodriguez, 2011;Inati et al, 2013;El Beltagi et al, 2014;Akpınar et al, 2015) to anecdotal cases that do not compel, in the absence of concomitant evocative clinical elements, further investigations or follow-up MRA. Considering that our study population ranged from early adulthood to the sixth decade, no conclusion can be obviously driven for older b-thalassaemia patients and further studies are needed before excluding a late involvement of intracranial arteries.…”

Section: Vascular Changes In B-thalassaemiamentioning

confidence: 71%

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

View full text Add to dashboard Cite

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.

show abstract