The Quarterly Consultis a quarterly publication highlighting pediatric clinical expertise. If you have an item of interest to include in the Quarterly Consult, contact Dr. Steve Dassel via Children's Physician Relations at 206-987-5765.

August 2010: First Seizure Clinic with Rusty Novotny, MD

Seattle Children's Hospital Neurology Department has started a First Seizure Clinic. I talked with the medical director of the Epilepsy Program, Dr. Edward "Rusty" Novotny, the principal investigator of the Center for Integrative Brain Research team at Seattle Children's Research Institute, about it.

Dr. Novotny grew up in Cucamonga, California. He did his undergraduate work at University of California, Irvine, went to medical school at St. Louis University and completed a residency in pediatrics at University of California at Davis. Following that, he completed a residency at Stanford in neurology and a fellowship in epilepsy/clinical neurophysiology. He went to Yale for a fellowship in magnetic resonance research held jointly in the Departments of Molecular Biophysics and Biochemistry and the Department of Neurology, and spent the next 20 years there before coming to Children's. He is professor of pediatrics and neurology at the University of Washington, with adjunct appointments to neurosurgery and radiology.

Question:Dr. Novotny, please describe First Seizure Clinic for us.

Answer:The clinic started April of 2010. It is located at the main hospital campus, drawing only from the contiguous counties of King, Pierce, Snohomish, Island and Kitsap. We serve families with children having nonfebrile "first spell seizures." These patients come from the Emergency Department via their primary care providers or directly from their providers.

Question:Please sort out "first spells."

Answer:The "spell" may be an epileptic event, a sudden neuronal discharge causing some sort of behavioral change, such as tonic-clonic activity or staring activity, or it may be a tic, other movement disorder or syncope. First Seizure Clinic can be helpful in sorting all this out.

Question:How often is this first seizure a tonic-clonic event?

Answer:One-third of the time. Other events may be brief periods of inattentiveness 10% of the time; involuntary movements, focal seizures or "myoclonic" seizures 10% of the time; or may be just a change in behavior such as disorientation or amnesia 10% to 20% of the time.

Question:What is the etiology of sudden onset tonic-clonic seizure?

Answer:By far the most common etiology is idiopathic. That is about 70% to 80% of tonic-clonic seizures. Less common and in second place are developmental anomalies. This is especially true when the onset of the seizures are at an early age, less than 5 years. Examples are cortical dysplasias such as tuberous sclerosis. Other etiologies are brain injury such as in utero or perinatal strokes, vascular anomalies and tumors.

Question:What kind of information do you want from us as we refer this patient with a "first seizure"?

Answer:Paramount is a really good history. Was the seizure generalized or focal? If possible, try to distinguish between the focal event that rapidly spreads to become generalized versus the seizure that began generalized. The accurate seizure duration is important. So are the clinical behaviors during the prodrome, measured in minutes to hours, before the event. Important are such things as fever, illness and behavioral changes. Also - any changes in the clinical status during the few days before the day of the seizure. Then the clinical features during the postictal period, such as how long it took to return to normal level of alertness and the presence of any weaknesses, mouth drooping, clumsiness, etc.

Question:Give us some examples on how this information is of use to you.

Answer:If we have a good, accurate description of the generalized tonic-clonic episode, as outlined above, or of an absence episode, we may not need imaging studies and would go directly to electroencephalogram. This information can also have an impact on prognosis and provide information on the chance that relatives are more susceptible to a seizure. Of course, a child for whom there is an accurate description of a focal seizure has a much greater chance of having underlying specific pathologies such as tumor, vascular anomaly and so on. The electroencephalogram may confirm the focal abnormality, and neuroimaging becomes a high priority in the diagnostic evaluation.

Question:What should we look for on physical?

Answer:Focal neurologic findings such as weakness, any clumsiness or changes in language function.

Question:I remember being taught that the EEG done acutely would be abnormal just because of the seizure, and that we should "let the brain calm down" before getting it. Please comment.

Answer:Yes, the "hard to interpret" too-early EEG argument. Actually, we're pretty good at interpreting that. An early EEG can help differentiate between a generalized and a focal seizure. The latter finding would make brain imaging a critical part of the diagnostic evaluation.

Question:What are your criteria for getting an EEG initially?

Answer:For a first tonic-clonic episode, one ideally should get an EEG in the first 24 to 48 hours. An EEG will also shed light on the nontonic-clonic event such as eye blinking or rolling, absence episodes and urinary incontinence as opposed to just inattentive behavior. Recent studies have shown that a one-hour EEG is absolutely diagnostic in true absence seizures greater than 95% of the time.

Question:Any other routine labs or imaging studies you would like to have us obtain?

Answer:No. Routine studies are of little help.

Question:Changing the gears a bit: Why the need for a "First Seizure Clinic"? How is its focus different than a seizure clinic?

Answer:There are a number of unique issues to focus on. Predominant is the strong emotional impact of a first seizure on a family. Being seen in a timely manner alleviates much of the concern a family may have. Also important is the need for education about seizures, which is provided by the clinic. A significant portion of the First Seizure Clinic is education about seizure first aid and seizure diagnosis and prognosis, including chances of having another seizure, and so on. There is a discussion of what to look for if another seizure occurs, seizure recognition and differential diagnosis of seizures. We set aside up to an hour block to handle all of the education, if necessary. Also a part of that appointment is a slot in the clinical neurophysiology lab for an EEG, if necessary.

Question:What would a flow chart look like for a patient being referred to First Seizure Clinic?

Answer:The patient is seen for the first time within a week of the referral. We recently doubled our slots to accommodate an increased demand. That first visit is with a nurse practitioner, who provides the aforementioned education and gathers information. There is a case conference, which is held within 24 hours, consisting of the nurse practitioner, a general neurologist and an epileptologist. From this conference, the decision may be made for a patient to go on to have an EEG, or a patient may receive a referral to general neurology clinic (tics and movement disorders), cardiology clinic (syncope or a cardiac etiology), neurodevelopmental clinic (autism), and so on. A focal seizure patient may be sent for an MRI and an EEG.

Question:Is there another visit after the First Seizure Clinic visit?

Answer:There will be another visit within approximately two weeks. The patient and family will meet with the same nurse practitioner for continuity or with a general neurologist or epileptologist.

Question:Does the nurse practitioner in First Seizure Clinic have any specialized training?

Answer:Yes, the Neurology nurse practitioners have unique experiences and expertise with regard to epilepsy. They spend several weeks a year on the inpatient Epilepsy Monitoring Unit with the epileptologists and working with the ketogenic diet program that is part of the Epilepsy program.

Question:Finally, is there anything that I didn't ask that you would like to pass along?

Answer:Early intervention is critical in a first seizure situation. This provides the best opportunity to obtain accurate history and clinical information and give education to facilitate making the correct diagnosis. There are better long-term outcomes when the best and appropriate treatments are started early. Hopefully, inappropriate treatment can be avoided, and protocols to make better use of diagnostic procedures can be developed.

Question:Is any research being thought about for First Seizure Clinic?

Answer:In the past, seizure clinics have generally seen the most difficult cases, and a skewed notion of the spectrum of epilepsy results. First Seizure Clinic will provide a better concept of the spectrum of epilepsy in the population. We also will be able obtain more information of the natural history of epilepsy from its inception, specifically: How does simple epilepsy progress into a more problematic situation? We hope to get a better handle on optimum first treatment options for seizure patients.

Question:Thanks, Dr. Novotny.

Addendum:An EEG lab is available at the new Bellevue Clinic and Surgery Center. There is easy access to this lab with appointments available, and PCPs can refer patients directly.

If you have an item of interest to include in the Quarterly Consult, contact Dr. Steve Dassel via Children's Physician Relations at 206-987-5765.