Anti Leucine Rich Glioma inactivated-1 (LGI1) antibody associated encephalitis has traditionally been associated with limbic encephalitis and Faciobrachial seizures with hyponatremia in elderly males. They can also present as paraneoplastic disorders and is classically seen to be linked with thymoma. Here, we present a case of a 5-year-old child who presented with episodic ataxia and behavioural abnormalities and was found to be having LGI1 antibodies in the serum and CSF. The patient responded dramatically to immune modulation. This case report strengthens the fact that there can be varying and atypical presentations of a single spectrum disorder and a high index of suspicion must be there to reach the correct diagnosis so that right treatment can be started.
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