Topiramate is effective for status epilepticus and seizure control in neuraminidase deficiency.

Sialidosis, a rare lysosomal storage disorder is caused by a deficiency of the enzyme α-N-acetyl neuraminidase, resulting from mutations in the NEU1 gene. Its main phenotypes are Sialidosis types I (milder form) and II (earlier onset). Sialidosis type II is characterized by developmental delay, macular cherry-red spot, visceromegaly, coarse facies, dysostosis multiplex, and myoclonus. We report a case of status epilepticus (SE) in a patient with Sialidosis type II which had good response to topiramate.