Headache: an important symptom possibly linked to white matter lesions in thalassaemia

Abstract

Neurological manifestations are reported only occasionally in patients with thalassaemia and are given much less prominence than the complications related to anaemia and iron overload. White matter changes (WMCs) on magnetic resonance imaging (MRI) in patients with thalassaemia were first reported two decades ago but the significance of these lesions remains unclear. We studied the neurological and cognitive manifestations in 82 older patients with thalssaemia [25 Thalassaemia major (TM), 24 thalassaemia intermedia (TI) and 33 haemaglobin E b thalassaemia (EBT)] and 80 controls, and found that headaches were more common in thalassaemia patients (50/82, 61%) than in controls (18/80, 22Á5%: P < 0Á001). WMCs on MRI were found in 20/82 (24Á3%) patients and 2/29 (6Á9%) controls had (P = 0Á078). WMC were more common among those with headaches (17/50: 34%) than in those without headache (3/32; 9Á3%) (P = 0Á023). WMCs were not associated with reduction of cognition. Nevertheless, cognition was lower in the TI and EBT groups compared with those with TM (P = 0Á002). The association of headache with WMC in thalassaemia has not been reported before and warrants further study.

Modified Magnetic Resonance Imaging Burden of Cerebral Small Vessel Disease and Related Risk Factors in Patients With Thalassemia

“…In comparison, the onset of WMH in people with thalassemia is earlier, and the incidence higher. Premawardhena et al 11 found that the incidence of WMH in patients with thalassemia was significantly higher than that in the non-thalassemia control group (24.3% vs. 6.9%), but there was no significant difference between the 2 groups. Two other studies involving brain atrophy in patients with TI, in which the age ranged from 9 to 48 years and 18 to 54 years, indicated that the incidence of brain atrophy was 31% and 37%, respectively 3,14 …”

“…Therefore, a macrovascular disease may account for the occurrence of SCI in patients with thalassemia. 8 The incidence of ischemic WMH in adult patients with moderate and severe thalassemia ranges from 15.8% to 61%, 9,[11][12][13][14] whereas the incidence of asymptomatic WMH is ~0% to 11% in healthy people under 50 years old. 14 With the increase in age, the incidence rate increases and the degree of lesions gradually worsens.…”

“…Thrombosis is one of its complications, including deep vein thrombosis, cerebral vascular thrombosis, portal vein thrombosis, and pulmonary embolism . Most of the ischemic changes in patients with thalassemia include silent cerebral infarction (SCI) or white matter lesions, 3–14 suggesting that most of them may be cerebral small vessel disease (CSVD).…”

Asymptomatic intracranial aneurysms in beta-thalassemia: a three-year follow-up report

“…The literature review about intracranial aneurysms in beta-thalassemia reveals that data are strikingly scarce and controversial. Even though beta-thalassemia patients had shown a high rate of intracranial aneurysms (17.2%) [7] the study lacked a control group, it was not confirmed by subsequent larger case-control studies, [8,9] and it considered a subgroup of patients (NTDT patients) undergoing a specific surgical procedure (splenectomy) that is nowadays more and more delayed or even prevented. Therefore, the current literature leaves still unsolved whether such a high rate of intracranial aneurysms were limited to environmental, treatment or genetic factors of a specific world region or it truly unveiled a disease-related vascular vulnerability of adult beta-thalassemia patients.…”

“…The latter have been found in 5/29 (17.2%) splenectomized NTDT adults in the first observational transverse MR-angiography study [7]. However, this finding was not confirmed by subsequent case-control studies which reported no cases of intracranial aneurysms in 80 thalassemia patients (transfusion dependent, NTDT and E-beta-thalassemia patients) [8] or no significantly increased incidence rate in 73 transfusion dependent and NTDT beta-thalassemia patients compared to healthy controls (9.3% vs 8.9%, respectively) [9]. Beside the unsolved issue of an increased incidence rate of intracranial aneurysms, no information is currently available regarding their natural course, whenever they are incidentally discovered.…”