Picturing the Future of Epilepsy Care

Picturing the Future of Epilepsy Care

Dr. Rusty Novotny opens the door to better epilepsy carethrough his focus on integrated, multimodal imaging.

Daniel Kelley and brother Jack

Daniel Kelley's recent epilepsy surgery
left the 3-year-old (pictured with younger
brother, Jack) free from seizures and
with his motor skills intact.

Epilepsy is as puzzling as it is frightening.In seven out of 10 cases, the cause ofthe condition’s sudden and dangerousseizures remains unknown.

The encouraging news is thatdoctors are constantly improvingepilepsy treatment while collecting newclues about the causes for the disease.Nationally respected neurologistDr. Rusty Novotny, medical director ofour epilepsy program, is helping SeattleChildren’s become a leader in betterunderstanding this mysterious braindisorder.

Dr. Rusty Novotny

Dr. Rusty Novotny is a leader in
developing new ways to combine
images from different imaging
systems – like PET scans, MRIs
and CT scans – to create a clearer
picture of what is happening in a
person's brain.

Novotny joined Children’s fromYale University, where he led theYale–New Haven Hospital pediatricepilepsy program and developed waysto combine images from differentimaging systems to create a moreaccurate picture of a patient’s brain.This work opens the door to improvingthe diagnosis of different forms ofepilepsy and better gauging howtherapies are working. It also helpssurgeons better map critical areasof the brain before operating.

“We had some of the most advancedimaging systems available, but weweren’t maximizing them,” notesDr. Jeff Ojemann, neurosurgeon anddirector of surgery for the epilepsyprogram. “Rusty is helping us marrythose technologies and gather criticalinformation that wouldn’t otherwisebe apparent.”

Hearing the whispers

Normal brain function is made possibleby millions of tiny electrical chargespassing between nerve cells in thebrain and to all parts of the body.When a person has epilepsy, suddenand intense bursts of electrical energyinterrupt this process.

The bursts cause seizures that range from momentary lapses of attention toprolonged convulsions. Whether theyoccur every few months or dozens oftimes a day, the seizures can interferewith a child’s learning and developmentand even cause permanent braindamage.

To identify forms of epilepsy anddecide how to treat it, doctors use avariety of imaging systems to studythe brain structure of and metabolicactivity in affected patients. Eachimaging system relies on different datasources – gamma rays from positronemission tomography (PET) scans,radio waves from magnetic resonanceimaging (MRI) scans or X-rays fromcomputed tomography (CT) scans – to produce separate snapshots.

Integrated imaging combines thisdisparate data – plus results from othersources such as electroencephalogram (EEG) tests – to produce a singledigital picture and then processes itto identify features that separateimages don’t show on their own.

Better pictures help identifyvarious forms of epilepsy, bettergauge how therapies are workingand better map critical areas ofthe brain before operating.

Ojemann compares the differencebetween integrated imaging andconventional imaging to listeningto five different people whisper. “You might not be able to hear themif they whisper alone, but if they allwhisper at the same time, you can.”

In other words, the whole (anintegrated image) is more revealingthan the sum of the parts (differentimages viewed individually).

“We have our boy back”

Tristan Carroll and Dr. Russell Saneto

Tristan Carroll, 8, was having
up to 50 seizures a day until
Dr. Russell Saneto prescribed
the exacting high-fat, low-carb
ketogenic diet.

Strokes, brain tumors and traumacause most epilepsy in adults, but theunderlying cause of most epilepsy inchildren is genetic. Although there’sno cure for most forms of the disease,medication helps two out of threechildren control their seizures. Ourepilepsy program offers the latestdrugs – and several alternatives ifmedications don’t work.

Options include surgically removingor disconnecting misfiring tissue,implanting an electronic device knownas a vagus nerve stimulator, and puttingchildren on a special high-fat, low-carbdiet. Known as a ketogenic diet, thistherapy is helping Tristan Carroll stayseizure-free.

Tristan, the 8-year-old son of Jimand Christine Carroll of Duvall, wasdiagnosed with epilepsy when hewas 2. Over the years, he tried manymedications, but none providedpermanent relief.

A year ago the seizures begancoming faster and faster until Tristandropped to the floor with convulsions50 times in one day. After a trip to Children’s emergency department, hisneurologist, Dr. Russell Saneto, decidedhe might be helped by a ketogenic dietin combination with medication.

Tristan has had only two days withseizure activity since starting the dieta year ago. He played baseball thissummer and attended his first sleepover. “The seizures were preventing him fromdoing so many things,” says his mom. “He’s a lot more independent now.We’re so grateful to Dr. Saneto andChildren’s Hospital. We have ourboy back.”

Ketogenic diets and medicationsdon’t cure epilepsy, so doctors trackpatients like Tristan closely as theygrow up. Novotny envisions anexpanding role for integrated imagingin following a child’s progress andwatching for possible side effects fromtreatment. Doctors could, for instance,use different MRI technologies to lookat brain function and brain chemistryconcurrently to see how the chemicalchanges that occur with treatmentrelate to memory, speech and otherneurological development.

“We’re not doing that yet, but it’s anexample of how integrated imaging canhelp us monitor and adjust treatmentto optimize outcomes,” Novotny says.

“In good hands”

Dr. Jeff Ojemann

Neurosurgeon Dr. Jeff Ojemann.

Daniel Kelley, the son of Jeff andBrenda Kelley of Bonney Lake, was 6months old when he was diagnosedwith epilepsy. Medication controlledhis seizures for a while, but they soonreturned – a pattern that was repeatedwith every new medication.

Daniel was lucky in one respect.An MRI indicated the seizures wereoriginating from a specific location – a lesion on the right side of his brain.That meant Ojemann could performsurgery if he could determine theprecise boundary between the lesionand surrounding tissue that controlsthe movement of Daniel’s hands.

To find a separation between thelesion and the sensitive tissue, Ojemannblended data from an EEG withintegrated imaging from a PET scanand a special type of MRI. Known as anfMRI, or functional MRI, it is taken whilepatients perform tasks such as readingor tapping their fingers. The resultsreveal areas of the brain that must beavoided during surgery.

Daniel’s surgery left the 3-year-oldfree from seizures and his motor skillsintact. “We were nervous,” says Daniel’smom, “but we knew we were in goodhands because Dr. Ojemann was so careful and specific about how heprepared for and performed the surgery.”

Gift aids recruitment

The Alvord, Gerlich and Rhodes FamilyEndowed Chair in Pediatric Epilepsyhelped bring Novotny to Children’s.The endowed chair was establishedwith a gift from the family of the lateDr. Buster Alvord (see below).

“We were already known as an excellentregional referral center for epilepsy,” saysDr. Sidney Gospe, who leads Children’sNeurology Department. “We recruitedRusty to help us become a nationalreferral center that improves treatmentand contributes new knowledge aboutthis disease. We couldn’t have attractedsomeone of his stature without theresources this gift provides and thestatement it makes about ourcommitment to fighting epilepsy.”

Novotny says Children’s is a great fit,providing state-of-the-art imagingsystems, a deep pool of informationtechnology talent and a host of skilledmedical specialists – both at thehospital and the University ofWashington. Another attraction:working in a hospital devoted solely tocaring for children in a region with agrowing population.

“There is so much promise hereto build an epilepsy program thatbreaks new ground and translateswhat we learn into better patient care,”Novotny says.

Exciting progress

Under Novotny, our epilepsy programestablished a fellowship program totrain doctors in this important specialtyand began using telemedicine todigitally collaborate with communityphysicians in distant locations.

We also joined a nationwide study – the Epilepsy Phenome/Genome Project – which seeks to betterunderstand which genes influenceepilepsy and how. The goals ofthe study include finding whichmedications work best with whichforms of epilepsy, and identifyingcertain forms of epilepsy that disappearas children grow older – knowledgethat would help avoid needless surgeryand limit duration of medical treatment.

“That’s what makes epilepsy suchan exciting field to be in,” Novotny says. “What we learn through research canbe directly transferred into improvedpatient care.”

Gift Continues the Work of a Pioneer

Nancy and Buster Alvord

Nancy Alvord and her late
husband, Buster.

The late Ellsworth C. “Buster” AlvordJr. was known throughout the worldfor his pioneering contributions tothe field of neuropathology. Alvord served as head of neuropathology atthe University of Washington Schoolof Medicine for 40 years and providedclinical care at the UW and at SeattleChildren’s. Two of his books on braindevelopment and neuropathologyare still considered classics, and hisresearch helped improve the care ofpeople living with multiple sclerosis.

“Research, teaching and patientcare were equally important to him,”recalls Dr. Bob Hevner, a researcherat Seattle Children’s ResearchInstitute and one of Alvord’s illustriousprotégés. “He enjoyed his work morethan anyone I know and his enthusiasmand positive attitude spilled over toeveryone he worked with.”

Over the years, the Alvord familyhas maintained close ties to Children’s,raising money, volunteering andhosting luncheons and meetings.They also created the Alvord, Gerlichand Rhodes Family Endowed Chair inPediatric Epilepsy.

“Our family knows how muchwork there is to do to advance carefor kids with epilepsy,” notes his wife, Nancy Alvord. “We are proud to helpbring talented, visionary leaders toChildren’s. Our endowment is a wayof carrying forward the work thatBuster started.”