Abstract
INTRODUCTION: Friedreich's ataxia is an autosomal-recessive, neurodegenerative disease that primarily affects the nervous systemand heart with an incidence of 1 in 50,000 individuals. Mutation in the gene for Frataxin results in the expansion of trinucleotide GAA repeats in the intron 1.Characterised by progressive gait and limb ataxia, dysarthria, areflexia, decreasedposition and vibration sense, muscular weakness in legs, and extensor plantar response, non-neurological signs including hypertrophic cardiomyopathy and diabetes mellitus. We present a case of primigravida with pre-eclampsia, K/C/O Friedreich Ataxia for emergency LSCS.

CASE REPORT: 32yr old primigravida with 38weeks amenorrhoea with foetal distress posted for emergency caesarean section. A year back she was diagnosed to have Friedreich’s Ataxia when she presented with ataxic gait, abnormal joint position sense, Romberg’s test positive, plantar reflex extensor, Bilateral upper limb Pseudo-athetosis, with no cardiovascular, respiratory and musculoskeletal abnormalities. MRI spine: Mild Atrophy of entire spinal cord, ill-defined hyperintense signals in cord parenchyma. Case was started under sub-arachnoid block,intraoperatively patient threw convulsions, immediately intubated and converted to GA. Extubated once the procedure was done. Patient is on regular follow up & there are no new neurological deficits till date.

CONCLUSION: Friedreich’s Ataxia is a rare disorder, very few case reports of anaesthesia administered to these patients. Here is one such rare case report of Friedreich’s Ataxia, managed successfully & safely under regional and general anaesthesia, without any prolongation of neuromuscular blockade, as anticipated.