INSR and Dravet syndrome: Thus, cases that lead to neonatal or early childhood death are usually classified as DS, while those that do not lead to such early death are usually diagnosed as RMS.[2,3] We report the case of a male child with 2 novel INSR mutations (c.2246delG and c.2646 + 5G > A) identified by whole-exome genetic testing who was diagnosed with severe INSR-related insulin resistance syndromes (SIR).