ASSESSMENT OF THE AUTONOMIC AND PERIPHERAL NERVE FUNCTION IN PATIENTS WITH THALASSAEMIC SYNDROME IN A TERTIARY CARE HOSPITAL - A CROSS SECTIONAL STUDY

Abstract

INTRODUCTION: β-thalassaemia is an inherited disorder of haemoglobin characterized by an absence or reduced synthesis of the β-globin chain. The net result is an excess of α-chains, which precipitate and destroy the red cell precursors, leading to anaemia, skeletal changes, splenomegaly and numerous other complications. Treatment is by regular blood transfusion coupled with iron chelation therapy to prevent iron accumulation and organ damage. AIM: To assess the autonomic and peripheral nerve function in patients with thalassaemic syndrome. MATERIALAND METHODS: Patients attending the Thalassaemia Clinic of Medical College Hospital, Kolkata. Indoor patients from Medicine st th ward and Institute of Haematology and Transfusion Medicine wards of Medical College Hospital, Kolkata from 1 May 2008 to 30 April 2009. After inclusion patients were divided into following groups: Group1 (THAL1): Patients requiring regular blood transfusion and are either receiving inadequate iron chelation or no iron chelation at all. Group2 (THAL2): Patients who require regular blood transfusion and are receiving adequate iron chelation. Group3 (THAL3): Patients who do not require regular blood transfusion RESULT: The median value of distal latency of right common peroneal nerve is slightly increased in all 3 groups. The normal value should be < 4.55ms, thus increase in distal latency is seen in all 3 groups. The median values of distal latency of left common peroneal nerve as well as distal latency of common peroneal nerve as a whole is within normal limits. Distal latency of common peroneal nerve (p value=0.012). Proximal latency of left common peroneal nerve(p value=0.013). Proximal latency of common peroneal (p value=0.001) CONCLUSION: There is demyelinating type of neuropathy of right common peroneal nerve of all the groups. This indicates presence of motor peripheral neuropathy in patients of thalassaemia. All other latencies and amplitudes of both motor and sensory nerves are within normal limits. There were statistically increased motor latencies in patients receiving iron chelation therapy with adequate chelation when compared to the other groups, although the overall latency was within acceptable limits of the laboratory standards. This may be due to subclinical affection of the motor nerves in the form of demyelination. Therefore, the role of toxic effects of iron chelator on peripheral nerves needs to be studied further.