Syndromes of Status Epilepticus

It was recently confirmed by Bertrand's pediatrician that his most recent EEG was abnormal, and significantly so, during sleep.  His epileptologist wants him to be admitted for a high-dose valium protocol (also known as the Riviello Protocol).  However, the admission process has been tricky since many of the neurologists on-call do not feel comfortable administering and overseeing this rarely used protocol.

It consists, in lay-mommy terms, of cycling between a month of high-dose valium and a month off.  The idea behind it being that it gives the brain time to rest and reset normal pathways.  That said, here is a good presentation on the issues with Bertrand's electrical status epilepticus of sleep (ESES) and the various potential treatments of it, including this high-dose valium (benzodiazepine) protocol.  Enjoy!

SPECIAL SYNDROMES OF STATUS EPILEPTICUS, PRESENTED BY JAMES J. RIVIELLO, JR, MD

Clarifying the Syndromes of Status Epilepticus 

I'll be talking about the off-label use of valproate, clonazepam, benzodiazepines, ethosuximide, prednisone, adrenocorticotropic hormone (ACTH), and intravenous gamma globulin.

We've heard some talk tonight about status epilepticus and convulsive and nonconvulsive status epilepticus. I refer to what's been discussed as overt status epilepticus. This would be the status epilepticus syndromes that are associated with acute seizures or an acute encephalopathy.

I call some of these epileptiform encephalopathies. These would be specific epileptic syndromes that are associated with frequent epileptiform activity on the electroencephalogram (EEG). Frequently, we may see these during sleep, so we may have an EEG that becomes very sleep-activated. This would be called electrical status epilepticus of sleep (ESES). There's also an epileptic syndrome that's called ESES, which is important to differentiate from an EEG, which may be a very sleep-activated EEG. These can be referred to as epileptiform encephalopathies.

I use epileptiform encephalopathy to define something in which the primary clinical manifestations result from the epileptic activity, the epileptiform features on the EEG, and its resultant dysfunction, rather than an actual clinical seizure. Sometimes it can be difficult in differentiating these.

What we're focusing on are patients who may clinically not have many seizures, but have EEGs that are very epileptiform EEGs. Not all of these patients may have overt clinical seizures.

One thing that can be very important in these disorders is regression in either intellectual or cognitive ability. If you see regression in either intellectual or cognitive abilities, that has to raise the suspicion that we may be dealing with some of these very sleep-activated epileptiform encephalopathies.

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