Merrf Syndrome and Refractory Status Epilepticus

Abstract: MERRF syndrome (or Myoclonic Epilepsy with Ragged Red Fibers) is a mitochondrial disease maternally-inherited. In this progressive disorder, status epilepticus is common and highly resistant to treatment including generalized anesthesia. We report two cases with Merrf syndrome (sister and brother), presented with status epilepticus refractory even to Thiopental, Propofol and Midazolam. In the same family is and another girl, diagnosed with Merrf syndrome. Given that it wasnt possible to stop the refractory status in these cases, we suggest Ketamine as an alternative to general anesthesia as for blocking seizure activity, as for the potential neuroprotective effect.

Keywords: Merrf syndrome, status epilepticus, ketamine

Edition: Volume 5 Issue 4, April 2016,

Pages: 492 - 493

How to Download this Article?

Type Your Valid Email Address below to Receive the Article PDF Link

Verification Code will appear in 2 Seconds ... Wait

Merrf Syndrome and Refractory Status Epilepticus

Similar Articles with Keyword 'ketamine'

Examination of Cognitive Deficits Produced by Sub-Chronic Ketamine Exposure in Rats

Comparison of Caudal Bupivacaine Alone with Dexmedetomidine as an Adjuvant with Bupivacaine for Post Operative Analgesia in Paediatric Patient Undergoing Inguinal Region Surgeries