Introduction:

Thalassemia, is the most common monogenic hemolytic anemia, which leads to multiorgan dysfunction with a variety of clinical and neurological manifestations.

Materials and methods:

At our thalassemia center, we describe myriad of neurological manifestations detected using 3 Tesla Discovery 750w scanner, between 2015 - 2018. Carotid Doppler was performed using a high resolution linear transducer on iu22 USG machine.

Results:

Fifty-six patients with short stature and lack of development of secondary sexual characteristics were noted to have significant pituitary iron overload in excess of controls (p < 0.05). Iron deposition in pituitary is responsible for hypogonadotrophic hypogonadism and stunted growth, and has only recently been evaluated by MR R2, R2*. These techniques allow early pituitary iron estimation, providing an opportunity to “rescue” pituitary function.

Two cases of intracranial extramedullary hematopoiesis presenting with headache and vomiting and another of intraspinal extramedullary hematopoiesis with paraparesis were detected.

Two cases presenting with tetany and seizures having hypoparathyroidism due to iron deposition, were noted to have intracranial calcifications in basal ganglia, thalami and grey-white interface.

One patient who presented with hemichorea, was found to have radiological features of Moya-moya syndrome and choroid plexus siderosis was observed in another.

Carotid intima-media thickness and arterial stiffness indices, studied by carotid Doppler in 53 patients, were significantly increased as compared to controls (p <0.001), indicative of early atherogenic changes.

Conclusion: Thalassemia patients can develop several neurological complications which are now increasingly recognized due to their longer survival. Familiarity with these manifestations can help early detection and institution of prompt treatment to improve quality of life.