It always seems impossible until it's done…Nelson Mandela
In this blog we discuss how we can use EEGs and magnetic resonance imaging (MRI) to identify the source of the electrical storm that is epilepsy and, with map in hand, surgically remove the irritable focus and cure the patient. The new epilepsy surgical program at KHSC reflects years of building and harnesses the talents of faculty and staff, many newly hired to achieve the ambitious plan of Queen’s DOM and the Division of Neurosurgery at KHSC. We aspire to move to the forefront of innovation and to offer our patients the latest in care.
Patients with epilepsy have seizures. Each year an average of 15,500 Canadians learn they have epilepsy. Between 0.6 and 1.0% of Canadians live with epilepsy (https://www.epilepsy.ca/epilepsy-facts.html). While most cases of epilepsy are well controlled with medicines, epilepsy can be crippling in some and for many of these patients with refractory epilepsy there is now a cure. However, this cure, a form of neurosurgery that requires specialized centres with robust diagnostic and therapeutic multidisciplinary teams, is significantly under-utilized in Canada.
A person is said to have epilepsy when they have 2 unprovoked seizures occurring more than 24 hours apart, or 1 seizure and epileptiform electro encephalogram (EEG) or a seizure with an associated (causative) abnormality on brain imaging.The International League Against Epilepsy (ILAE) notes that epilepsy often results in recurring seizures (in >60% of patients). In approximately half the people with epilepsy the cause of the seizures is unknown; however, in the other half, the seizure may arise from a lesion identified on MRI, such as a tumor, focal cortical dysplasia or brain injury. As MRI and molecular/genetic diagnostics improve, fewer cases remain unexplained.
For most people epilepsy is a chronic disease that is controlled by medication and they live normal lives. However, for one in three patients with epilepsy these electrical brain storms are recurring and uncontrollable by 2 or more medications. These people are said to have “refractory epilepsy” and they have an impaired quality of life due to their ongoing seizures. On-going seizures are not just an inconvenience-prolonged seizure activity causes ongoing brain injury, so ‘time seizing’ is brain lost!
For people with refractory epilepsy there is new hope. Advanced diagnostic and surgical techniques, in aggregate referred to as epilepsy surgery, offers a cure. Epilepsy surgery is potentially possible in 50% of refractory epilepsy patients. Despite this promise only 2% of Ontario’s 27,000 epilepsy patients receive surgery! The barriers to epilepsy surgery include lack of certified programs and qualified teams, as well as misunderstandings of the risks and benefits of the procedure, as summarized in the Table below.
Prior to 2018 Kingston Health Sciences Centre (KHSC) lacked the designation and resources to perform epilepsy surgery. To identify patients who can benefit from epilepsy surgery one must be designated a district epilepsy centre (DEC). Over several years, with support from the Department of Medicine and KHSC leadership, with Dr. Lysa Lomax serving as a champion, we applied for and were designated a DEC. We then recruited faculty (in neurology, neurosurgery and radiology) and staff (in the EEG lab and neurology service-including nurses, technicians, neuropsychologists and community epilepsy agency liaison personnel) to perform the diagnostics that precede and guide epilepsy surgery. Through philanthropy we acquired the necessary imaging tools to support epilepsy surgery, notably a 3-Tesla MRI machine. With our recent demonstration of the capacity to perform epilepsy surgery and upgrading of our epilepsy monitoring unit (EMU)) our program should arguably be reclassified as a Regional Epilepsy Centre, the province’s highest designation (see diagram below). This is a goal we are working towards.
Dr. Lysa Lomax
The most common target of epilepsy surgery is the temporal lobe (including the amygdala); however, epilepsy surgery can be used for removing lesions in other part of the brain too. Temporal lobe surgery results are excellent, with 75-80% of patients becoming seizure free 1-year post-operation. Benefits are usually sustained, with half these people seizure free at 20 year follow-up. Results are not quite as good for surgeries on other parts of the brain.
Image of the brain with the temporal lobe highlighted
So what does the patient gain by undergoing the procedure? Reduced seizure severity of course. However, equally important reduction/elimination of seizures prevents the decline in the patient’s mental capacity which would otherwise occur (and may even result in cognitive gains). Interestingly there is also a reduction in the incidence of unexplained sudden death, which is a rare, but substantial concern for people with epilepsy. Successful surgery generally improves the patient’s quality of life, and the quality of life for those around them. While there are risks, including a 10% risk of a visual deficit (quadrantanopsia) and a 25% risk of verbal or visuospatial memory deficit, these risks pale compared with the potential benefits of surgery in well selected cases.
Let’s begin with the story of patient who recently underwent epilepsy surgery who agreed to tell us their experience.
Mr. Ozzie DaSilva
Mr. Ozzie DaSilva had a history of seizures since childhood. He experienced about 8-10 years of seizure freedom during his early teen years, but the seizures recurred in his 20’s. He had 2 main seizure types. Initially his seizures occurred 3-7x weekly and were marked by intense dizziness, head pressure, groaning, movements of both arms, and a variable awareness. Over the past 2 years, he began to experience a second seizure type, characterized by the previous symptoms followed by complete loss of awareness and falls. Because of frequent falls he had to wear a helmet to protect his head and his quality of life was severely compromised. Mr. DaSilva was taking many medicine to control his epilepsy including carbamazepine CR and brivaracetam and had tried and failed 4 other medicines.
So how does the team identify a candidate epilepsy surgery patient?
1) The neurologist, in this case, Dr. Lysa Lomax, identifies a patient whose clinical presentation offers evidence of a focal form of epilepsy in a person who has failed to be controlled by two drugs. Temporal lobe epilepsy often results in episodes of lip smacking, facial twitches and a sense of déja vu. Parietal lobe seizures, as in this case, can cause dizziness (known as ictal vertigo) and sensory changes.
2) The neuroradiologist, Dr. Donatella Tampieri in this case, interprets a high resolution (3-Tesla) MRI which shows the abnormality and helps the surgeon determine the feasibility of a surgical approach. In Mr. DaSilva’s case described above, a left parietal and cingulate area of focal cortical dysplasia was found (yellow circle in MRI below).
There is an area of left parietal FLAIR signal change consistent with focal cortical dysplasia. This is the abnormality that was causing Mr. DaSilva’s seizures.
This is a different patient who has left mesial temporal lobe sclerosis (red circle) which indicates the type of well localized lesion that is amenable to epilepsy surgery.
3) The Epilepsy Monitoring unit (EMU), is where the diagnosis of epilepsy is made and presurgical evaluation performed. This unit is directed by Dr. Lysa Lomax and managed by Dr. Helen Driver. This unit performs continuous EEG’s which are produced by the technologists (Head Tech, Lisa Calder, Mike Einspenner, Zaitoon Shivji, Tim Woodford, Lovanie Leroux and Nancy Lane). The Epileptologists, Dr. Lysa Lomax, Dr. Garima Shukla and Dr. Gavin Winston interpret the EEG’s to identify the nature and location of the seizure. The EMU would also not function without the dedicated efforts of the amazing nursing staff on Kidd 7 led by charge nurse, Debby Tuppeny.
L to R Epileptologists: Dr. Garima Shukla, Dr. Gavin Winston, Dr. Lysa Lomax
Neurosurgeon: Dr. Ron Levy
The pattern on this scalp EEG is one of diffuse suppression (right half of trace) which is a non-localizing (i.e. doesn’t’ identify the site of the seizure’s source). This promoted further study to identify the source, using a surgically implanted intracranial EEG.
4) The neuropsychologist Dr. Michelle Keiski, in this case assesses whether any cognitive problem the patient may have can be attributed to the localization of the seizure and the neurological lesion on imaging.
5) Steps 1-4 allow the neurosurgeon, in this case Dr. Ron Levy, to assess whether the lesion is approachable surgically without causing unacceptable risk of brain injury. We have two neurosurgeons with expertise in functional and epilepsy surgery, Dr. Ron Levy and Dr. Faisal Haji.
L to R Dr. Faisal Haji and Dr. Ron Levy
If the lesion is accessible and the patient agrees the surgeons proceed to operation. In many cases, the combination of clinical seizure semiology, neuropsychological test results, EEG and imaging findings are more than sufficient to identify that surgery can remove the seizure causing lesion. However, sometimes, there is uncertainty and the surgeons implant electrodes inside the brain to ensure they have identified the causative lesion, or to outline surgical borders. Surgery itself is guided by intracranial electrodes, which record intraoperative EEGs (image below). For the reader, remember the surgeon can’t see deep inside the brain, so they need to know where the abnormal electrical activity is coming from to safely hit their target. In the future, the Epilepsy Surgery Team would like to employ stereo-EEGs, a special set of electrodes than can be placed deep inside the brain, to enhance precision.
Surgical view of the brain (left). Non-contrast CT showing intracranial electrode (right)
6) Once the operation is complete the neuropathologist, Dr. John Rossiter in this case, establishes the tissue diagnosis. Note that the nice correlation between the abnormal MRI (white signal) and the abnormal histology (the pink surgical specimen below).
Dr. John Rossiter, Department of Pathology and Molecular Medicine
Further microscopic study of the surgical specimen by Dr. Rossiter shows the diagnostic dysmorphic neurons (left) and balloon cells (right) that are diagnostic of a type of epilepsy inducing lesion, called focal cortical dysplasia type IIb (see histology below).
So how did our patient do? Immediately post-op, patient experienced a marked decrease in typical seizures. There were no permanent complications, although there was a temporary right foot sensory loss requiring rehab, which is now resolved. Post op one year the patient has experienced a few auras, but no seizures. He no longer falls and is no longer using helmet!
Thank you ! Team Epilepsy at KHSC !
First row L to R: L. Leroux, H. Driver, D. Tuppenny, Dr. L. Lomax, S. Weatherby, Z. Shivji
Second row, L to RJ. Bailey, Dr. G. Winston, Dr. G. Shukla, M. Keiski, T. Woodford, M. Eispenner, L. Calder, Dr. R. Levy
This story is a remarkable story of patient centered care that required the expertise of doctors from 4 Departments and a host of nonphysician experts. Central to this story is the expertise of newly recruited epileptologists, neuroradiologists and neurosurgeons. It required support from the SELHIN and the MOHLTC. Our epilepsy surgery program would not have been possible without philanthropy. Identification of subtle brain lesions requires a high powered 3-T MRI machine, which costs over $6 million. This required fund raising by the University Hospital Kingston Foundation and a $3million gift from by Brit Smith, Chairman of Homestead Land Holdings (Thank you Mr. Smith!).
Arguably the preparation for the operation on this patient, which only took a few hours, was the culmination of a process that began 7 years ago with the rebuilding our neurology division, following an external review conducted by Dr. Doug Zochodne (University of Alberta). Even with the program in place, the patient’s evaluation and preoperative process took approximately 3-4 months, including wait times for neuropsych assessments, admission to the EMU and waits for OR time. In the march toward one person’s cure, we elevated care for all neurology patients at KHSC. We obtained designation and funding as a district epilepsy Centre, bringing ~$800,000/year to KSHC, and reminding all that innovation pays!. This success story is all about team and collaboration and could not have happened without the labors and passion of neuropsychologists, nurses and EEG technicians. So to all the members of our amazing team a heartfelt thank you!
Patient 1: Mr. Thomas Alston who was our first temporal lobe resection patient.
Postscript: I conclude with a reminder to all leaders of how innovative clinical care programs are born. One needs to create and clearly enunciate a strategic vision that is supported with buy-in from key stakeholders. Only with this foundation can one move a plan for a new program forward. Credit for the work must be broadly shared and partnerships valued. Persistence is always required since medical innovation, no matter how beneficial, is never easily brought to life in Canada’s cost-constrained health care environment.
Finally: Permit me a thought or two on the role of vision. When I arrived as the new Department Head and Program Medical Director late in 2012 I found a Department and a medical centre that was too small (arguably in both person power and vision) to achieve its destiny (see Sunshine Sketches of a Little LHIN). Through a robust strategic planning process, informed by the relevant stakeholders (faculty members, hospital leadership, and others), we created a plan to ensure that all Divisions in the Department of Medicine had sufficient size and expertise to: a) be stable b) provide state of the art care c) reduce wait lists and d) host residency training programs (thus ensuring their future stability by training Next-Gen docs) (see Glimpse of a Vision Blog). I also encouraged both the hospital and my colleagues in the DOM (and other Departments) to shoot high, following a credo that no patient should be referred to Ottawa or Toronto if we can create the program of care in Kingston. Over the past 7 years we have led or partnered in the creation of multiple trans-departmental clinical programs of excellence. Our partners include: the Departments of Surgery, Urology, Radiology, Pathology and Molecular Medicine, Anesthesia, OB-GYN, and Oncology. We have also relied on good working relationships with, and support from, the leadership of SEAMO (Dr. Richard Reznick), KHSC (Dr. David Pichora), Chief of Staff (Dr. Mike Fitzpatrick), and the Head of Medical Affairs (Mr. Chris Gillies).
So how is this ambitious agenda going? Seven years later we have doubled the size of the DOM, which now has ~125 faculty members (en route to a 150 geographic full time faculty by 2023).
Here are some other innovative clinical programs that the Department of Medicine and our partner Departments have built in the past 6 years:
- Allergy: This Division has major strengths in its growing experimental therapeutics program, focused on seasonal allergies. It has also advanced the care and education of local staff and students regarding the management and prevention of anaphylaxis.
- Dermatology: We have launched a new Dermatology division which includes a new Moh’s surgery program for skin cancer patients
- Cardiology: Cardiology has launched new minimally invasive procedures for heart disease (TAVI, Mitraclip) and advanced arrhythmia interventions to cure diseases like atrial fibrillation.
- E-Health: The DOM has expanded into e-health, including video visits for stroke prevention and epilepsy. We provide asynchronous e-visits for our family medicine colleagues, providing rapid consultative access to virtually every division in the DOM.
- Endocrinology has added a new Royal College training program in Endocrinology and will hopefully launch this program in the fall of 2020. The DOM has plans to add training programs in infectious diseases, geriatrics and dermatology.
- Gastroenterology: This division has developed new minimally invasive procedures for achalasia and esophageal tumors (per-oral endoscopic myotomy , POEM, and POET), and leads Canada in this practice. They also have innovative new programs in total parenteral nutrition and hepatology, with a new clinic delivering curative therapy for hepatitis C.
- General Internal Medicine (GIM): Managing ~40% of the beds at KHSC this Division leads the DOM in running our clinical teaching units (CTUs), including two new inpatient CTUs, created in the past year. The CTUS team handles the >5000 annual admissions (a 65% increase in inpatient admission over the past 5 years). GIM has also launched new perioperative and maternal medicine programs and created a new program to teach and apply point of care ultrasound.
- Geriatrics: The Division has doubled the geriatric footprint of inpatient care for patients at Providence Care Hospital.
- Hematology: Hematology has created a trans-departmental malignant hematology program with the Department of Oncology and has expanded its autologous bone marrow transplantation program and increased early repatriation of patients after allogeneic bone marrow transplantation.
- Nephrology: This division has created a Health Canada certified live donor renal transplantation, expanded its home dialysis program and created a new, specialized glomerulonephritis clinic.
- Neurology: Established a surgical program for deep brain stimulation. We have also created endovascular program to reverse a stroke in progress. Neurology has launched a video visit e-health program which is now expanding across all Divisions in Medicine.
- Palliative Care: This Division has created a new human resources plan to provide comprehensive palliative care across the city, linking KHSC and PCH to Hospice Kingston and our community colleagues in Family Medicine. This network will ultimately serve patients who would benefit from palliative care who have chronic diseases other than cancer.
- Respirology: The Division has transformed the approach to lung cancer through a complete revision of its lung diagnostic assessment program (LDAP), creating a new, multidisciplinary “one stop shop” clinic. They also have new programs for lung intervention, including EBUS and cryobiopsy.
- Rheumatology: This division has launched new programs for management of osteoporosis and early rheumatoid arthritis and developed a new joint ultrasound program.
We have made these clinical improvements while successfully launching Competency Based Medical Education (CBME) and creating advanced research platforms, like the Queen’s Cardiopulmonary Unit (QCPU). Moreover we have established a research institute, TIME (the Translational Institute of Medicine), which includes a new translational medicine graduate program, TMED. These later initiatives reflect my belief, shared by all leaders in our Department, that excellence in medical education and research improves the quality of care for all our patients.
This postscript is neither brag nor boast but a reminder to ourselves that the overnight success of the new program, like epilepsy surgery, occurs not only because of the talents of practitioners; but because of dogged pursuit of a vision until it was realized.
As Nelson Mandela said, It always seems impossible until it's done.