Jason S. Hauptman, MD PhD
Kaiser Permanente Los Angeles Medical Center
Los Angeles, CA
As surgeons, our greatest ambitions and desires revolve around making the lives of the people we take care of better. In the case of pediatric neurosurgery, we not only focus our efforts on helping children with challenging and sometimes devastating neurological illness, but help the families that care for them. Significant advances in the surgical treatment for intractable seizures are now bringing new hope to a very special subset of these patients. Epilepsy affects more than 300,000 children under the age of 15 and can be incredibly debilitating — some children require intensive, lifelong care for this disease. This, among other reasons, is what inspired me to train in pediatric neurosurgery in the first place.
What are seizures and what is “epilepsy”?
A seizure is what happens when large groups of brain cells are abnormally active, sometimes with large groups of them acting in synchrony. Not all seizures “look” alike! Here are some:
- Classic arm and leg twitching;
- Episodic confusion; and
- Repeated loss of consciousness and muscle tone with multiple falls.
Epilepsy is the disorder of being predisposed to having seizures. Along with the seizures themselves, epilepsy has consequences on the psychological, cognitive, and social well-being of patients. Typically, epilepsy is given as a diagnosis when a child has had two seizures that are “unprovoked” greater than 24 hours apart, or if they are diagnosed with a known syndrome of which epilepsy is a part.1
What is treatment-resistant epilepsy and what are the consequences?
Treatment-resistant epilepsy has been defined as a failure of two appropriately dosed and tolerated AEDs to achieve freedom from seizures. Eight to ten percent of children with newly diagnosed epilepsy will be deemed treatment-resistance within two years.4 Even with initial medical control of seizures after one year, there remains an eight percent chance that these seizures will become refractory within a year. If seizures continued to occur despite being on two to four anti-epileptic drugs (AEDs), the chances of additional medications effectively ending seizures is less than five percent.2 If these seizures are associated with brain abnormalities on an MRI, the chance of additional medication helping is also lower.3 Uncontrolled seizures adversely affect:
- Brain function;
- Social development;
- Growth; and
- Dental health.
We know that uncontrolled epilepsy is harmful to development and brain function. We know, for instance, that uncontrolled seizures early in life can be a risk factor for intellectual disability and low IQ.5,6 We also know that seizures that are inadequately controlled can result in cognitive, social, language and communication challenges.7 Furthermore, ongoing seizures can be related to lower rates of high school completion, employment, marriage, and overall socioeconomic achievement.8
Comprehensive pediatric epilepsy programs provide hope
Children should be seen by a team of pediatric epilepsy experts, of which surgical evaluation is one of many components, when their epilepsy is considered refractory or treatment-resistant. While all children with treatment-resistant epilepsy will not necessarily be candidates for surgical treatment, many may benefit from medical measures suggested by the team as well. Dedicated neurosurgeons have brought about significant advances that include:
- Less invasive surgical approaches;
- Procedures for a wide spectrum of seizure disorders; and
- Better understanding of complication avoidance.
Together these provide many advantages for these patients in need. Early surgical intervention for intractable seizures results in improved IQ scores and developmental indices.9-12 Aggressive control of seizures, sometimes with surgery, can also work to reduce seizure-related morbidity and mortality. Children with refractory epilepsy are at risk of higher rates of morbidity and death, with the mortality risk of uncontrolled epilepsy being 0.5 percent per year and accumulating over a lifetime.13 That means that a child who lives 20 years with uncontrolled epilepsy could have a mortality risk as high as 10 percent.
There are many fantastic neurologists and neurosurgeons around the country collaborating to develop first class pediatric epilepsy centers where children with challenging cases of epilepsy can be thoroughly evaluated and referred for advanced medical and surgical therapy. Together, we can make a huge difference in the lives of the families and children dealing with this challenging and sometimes severe neurological disease. I am excited to see what the future holds for our specialty.
Editor’s note: November is National Epilepsy Awareness Month. To learn more, click here.
- Fisher RS, Acevedo C, Arzimanoglou A, Bogacz A, Cross JH, Elger CE, Engel J Jr, Forsgren L, French JA, Glynn M, Hesdorffer DC, Lee BI, Mathern GW, Moshé SL, Perucca E, Scheffer IE, Tomson T, Watanabe M, Wiebe S. (2014) ILAE official report: a practical clinical definition of epilepsy. Epilepsia. 55:475-82.
- Kwan P, Brodie MJ. (2000) Early identification of refractory epilepsy. N Engl J Med 342:314-319.
- Spooner CG, Berkovic SF, Mitchell LA, Wrennall JA, Harvey AS. (2006) New-onset temporal lobe epilepsy in children: lesion on MRI predicts poor seizure outcome. Neurology 67:2147-2153.
- Berg AT, Shinnar S, Levy SR, Testa FM, Smith-Rapaport S, Beckerman B. (2001) Early development of intractable epilepsy in children: a prospective study. Neurology 56:1445-1452.
- Vasconcellos E, Wyllie E, Sullivan S, Stanford L, Bulacio J, Kotagal P, Bingaman W. (2001) Mental retardation in pediatric candidates for epilepsy surgery: the role of early seizure onset. Epilepsia 42:268-274.
- Cormack F, Cross JH, Isaacs E, Harkness W, Wright I, Vargha-Khadem F, Baldeweg T. (2007) The development of intellectual abilities in pediatric temporal lobe epilepsy. Epilepsia 48:201-204.
- Caplan R, Siddarth P, Mathern G, Vinters H, Curtiss S, Levitt J, Asarnow R, Shields WD. (2002) Developmental outcome with and without successful intervention. Int Rev Neurobiol 49:269-284.
- Sillanpaa M, Jalava M, Kaleva O, Shinnar S. (1998) Long-term prognosis of seizures with onset in childhood. N Engl J Med 338:1715-1722.
- Freitag H, Tuxhorn I. (2005) Cognitive function in preschool children after epilepsy surgery: rationale for early intervention. Epilepsia 46:561-567.
- Loddenkemper T, Holland KD, Stanford LD, Kotagal P, Bingaman W, Wyllie E. (2007) Developmental outcome after epilepsy surgery in infancy. Pediatrics 119:930-935.
- Jonas R, Asarnow RF, LoPresti C, Yudovin S, Koh S, Wu JY, Sankar R, Shields WD, Vinters HV, Mathern GW. (2005) Surgery for symptomatic infant-onset epileptic encephalopathy with and without infantile spasms. Neurology 64:746-750.
- Jonas R, Nguyen S, Hu B, Asarnow RF, LoPresti C, Curtiss S, de Bode S, Yudovin S, Shields WD, Vinters HV, Mathern GW. (2004) Cerebral hemispherectomy: hospital course, seizure, developmental, language, and motor outcomes. Neurology 62:1712-1721.
- Tellez-Zenteno JF, Ronquillo LH, Wiebe S. (2005) Sudden unexpected death in epilepsy: evidence-based analysis of incidence and risk factors. Epilepsy Res 65:101-115.