Blog # 169: Balancing Reality with Hope in Epilepsy Treatment

 

 

 

Experience in our neurology clinics is that half of the epilepsy population in the United States, i.e. more than three million Americans (similar percent of the global population afflicted with epilepsy) can control their epilepsy. When the epilepsy is controlled with just the initial trial of anticonvulsant drugs (ACD) the long term prognosis for that person’s epilepsy is very good. We physicians start treatment with a commonly used ACD that from experience has shown effectiveness at the commonly used dosage. Side-effects such as drowsiness and laboratory tests of liver function, blood counts, etc. hopefully won’t be significant and the patient will adapt to the treatment quickly.

 

Edward Faught, MD, wrote on the reality and the hope in treatment.¹ As our patients are no doubt familiar, the course of epilepsy is variable. Experience shows that not achieving good control averages one-third of the epilepsy population, despite multiple different ACD treatments at various dosages. Identifying the presence or absence of brain lesions can still lead us astray as some patients do well despite significant abnormalities on scans. Younger age at onset, abnormal EEGs and very frequent seizures can adversely affect the prognosis.

 

According to the International League Against Epilepsy, drug resistant epilepsy is suggested when 2 ACDs appropriate for their type of epilepsy, as judged by your experienced neurologist, at tolerated dosages fail, then a third drug probably will also fail to control all seizures, too (10%).² However, other studies suggest hope. Schiller and Najjar noted that even after 2 to 5 ACDs had failed another drug could possibly produce seizure control. That was found in 16% of those patients deemed therapeutic failures.³

 

Keep in mind that a highly successful treatment that can cure epilepsy is brain surgery. In highly selected patients studied to rule out adverse side effects that would result from surgery, these procedures are safe. Post surgery life can be normal. Refer to my previous surgery-related blogs #155 (Successful surgery with robot assistant:  https://lancefogan.blogspot.com/2017/12/blog-89-surgical-removal-of-seizure.html), # 145 (Epilepsy patient passes driving test after brain surgery for poorly controlled epilepsy https://lancefogan.blogspot.com/2022/08/blog-145-epilepsy-patient-passes.html), #121 (…Epilepsy surgery is safe.  https://lancefogan.blogspot.com/2020/08/blog-121-if-your-seizures-arent.html).

 

In conclusion we should keep in mind that there is always hope in epilepsy therapy.          

 

1.     Faught E. Balancing reality with hope in epilepsy therapy. Neurology 2018;91: p989-990.

2.     Kwan P, Arzimanoglou A, Berg AT, et.al. Definition of Drug-Resistant Epilepsy Epilepsia 2010; 51: 1069-1077.

3.     Schiller Y, Najjar Y. Quantifying the response to antiepileptic drugs: effect of past treatment history. Neurology 2008; 70: 54-65.

Lance Fogan, M.D. is Clinical Professor of Neurology at the David Geffen School of Medicine at UCLA. His hard-hitting emotional family medical drama, “DINGS, is told from a mother’s point of view. “DINGS” is his first novel. Aside from acclamation on internet bookstore sites, U.S. Report of Books, and the Hollywood Book Review, DINGS has been advertised in recent New York Times Book Reviews, the Los Angeles Times Calendar section and Publishers Weekly. DINGS teaches epilepsy and is now available in eBook, audiobook, soft and hard cover editions.

 

 

Blog #142: DID YOU FIND YOUR ACCESS TO EPILEPSY CARE AFFECTED BY COVID?

 

 

Did the COVID-19 pandemic affect your ability to obtain adequate care for your epilepsy? The National Association of Epilepsy Centers (NAEC) noted significant impact by the pandemic.¹ Most evaluations and procedures in the United States for drug-resistant epilepsy, i.e., for persons whose epilepsy is not free of break-through seizures despite treatment, decreased in number.

 

Epilepsy centers saw pandemic-caused widespread changes in hospital practice. Access to specialty epilepsy care decreased in 2020 compared to 2019 with 21,515 fewer epilepsy monitoring unit admissions in 2020, a 23% decline. This resulted from restrictions on elective admissions, reduced staffing, and patient reluctance for elective admission.

 

Aggregate surgical treatment for epilepsy declined by 371 cases (5.7%) with the largest reduction for vagus nerve stimulation implantations (2622 and 2136 cases in 2019 and in 2020, respectively, a 19% decline). Temporal lobectomies (1465 and 1238 in 2019 and 2020, respectively, a 16% decline. All other procedure volumes increased: the number of corpus callosotomies (splitting the right and left cerebral hemispheres connections) increased from 2019 to 2020 by 35%.

 

All these studies had the limitation of self-reporting of administrative data which is subject to inaccuracies or bias.

 

1)     AM Ahrens, AP Ostendorf, FA Lado, et.al. Impact6 of the COVID-19 Pandemic on Epilepsy Center Practice in the United States. Neurology. 2022; 98 (19):e1893e1901

 

Lance Fogan, M.D. is Clinical Professor of Neurology at the David Geffen School of Medicine at UCLA. His hard-hitting emotional family medical drama, “DINGS, is told from a mother’s point of view. “DINGS” is his first novel. Aside from acclamation on internet bookstore sites, U.S. Report of Books, and the Hollywood Book Review, DINGS has been advertised in a recent Publishers Weekly, New York Times Book Review and the Los Angeles Times Calendar section. DINGS teaches epilepsy and is now available in eBook, audiobook, and soft and hard cover editions.

 

 

 

Blog #106: LEARN ABOUT THE FEBRILE SEIZURE

            Young, healthy children can have seizures when they get high fevers such as with ear and upper respiratory infections. These seizures are more likely if family members have had them. Four percent of young children in the United States experience febrile seizures.

            The typical age range is 3 months to 5 or 6 years. The peak incidence of febrile seizures is between 18-22 months of age. They occur during the rising phase of fever and during the first day of the illness. Only one febrile seizure that day is typical. Seizures that occur later in the febrile illness suggest some other condition. Only 2 percent of youngsters who have had a febrile seizure go on to develop epilepsy by age 7 years.¹  The possibility of developing epilepsy later in life is increased if there is a prior neurologic developmental abnormality; if the convulsion lasts longer than 3-5 minutes; if convulsive jerking involves just one part of the body (focal) rather than a generalized convulsion; and if the EEG (electroencephalogram) is abnormal.

            Convulsions that occur with fevers after age 5 or 6 years should not be considered benign febrile seizures. These children should receive a thorough evaluation to search for some causative condition and if none is found, then epilepsy is likely. Children who have benign febrile convulsions are not considered to have epilepsy (epilepsy is defined as more than one seizure often due to no identifiable cause).

            Among children who experience their first febrile seizure before their first birthday, half will have at least one more. Among children who are older than 1 year when the first febrile seizure occurs, about 1 in 4 will have more. The overall prognosis, however, is excellent.

            Febrile convulsions are diagnosed when no other condition is present that can cause high fevers and the seizures occur before age six years. Meningitis and encephalitis must be considered. Clinical judgment determines if the spinal fluid is to be examined by a lumbar puncture. Serious causes of a convulsion associated with fever are considered if the convulsion occurs after the initial fever-peak or if there are multiple convulsions. Convulsions associated with a vaccination-induced fever are typically benign febrile seizures unless confused with a rare encephalopathic reaction to the immunization.²

            Acute treatment of the convulsion is usually not necessary unless it lasts 3-5 minutes. Diazepam (Valium) can be administered intravenously, intramuscularly or per rectum. Treatment should be given if it is a prolonged seizure.

            Prophylactic treatment (i.e. daily antiepileptic medications), can be used if the child under 6 years old experiences frequent convulsions during the rising phase of fevers. Daily administration may be indicated because seizures can occur before the fever is detected and then administration of the drug is too late. If prophylaxis is chosen it’s usually continued 1-2 years after the last febrile convulsion and then tapered off over 1-2 months. The problem with prophylactic treatment is that only one-third of the population of young children will experience another seizure with a subsequent febrile illness. There is no evidence that prophylaxis reduces the risk of future epilepsy.

            Risk factors for future epilepsy diagnosis in children who experience febrile seizures includes a family history of non-febrile epileptic seizures, pre-existing neurological conditions or developmental delay, seizures lasting longer than 3-5 minutes, focal localized seizures and multiple seizures in one day.

1.      Nelson KB, Ellenberg JH: Predictors of epilepsy in children who have experienced febrile seizures. N Engl J Med 295:1029, 1976.

2.      Engel, Jr J: Seizures and Epilepsy. Philadelphia, PA: F.A.Davis Co. 1989, p 181-183.

Lance Fogan, M.D. is Clinical Professor of Neurology at the David Geffen School of Medicine at UCLA. “DINGS” is his first novel. It is a mother’s dramatic story that teaches epilepsy, now available in eBook, audiobook and soft cover editions.

Blog #98: Highlights from the Epilepsy Symposium: University of Southern California. September 15, 2018

Ten percent of the American population will have at least one seizure in their lifetime and one in 26 Americans will develop epilepsy; epilepsy is diagnosed when more than one seizure occurs. Americans with epilepsy number 3.4 million, 65 million people world-wide have epilepsy. There are 48 newly diagnosed cases/100,000 population each year.

• The Communicable Disease Center (CDC) found the death rate for all Americans in 2017 was 844.0 deaths per 100,000 population or 0.84 percent.¹  This statistic is not that dissimilar to the 500 to 1000 deaths per 100,000 people with epilepsy that die each year. However, life-expectancy for people with epilepsy averages 19 years fewer than the general population. In 2014, SUDEP (sudden unexplained death in epilepsy) was listed as cause of death for only 2650 people. Death certificates don’t always list accurate causes of death, but SUDEP is thought to occur in one out of 75-150 Americans, or 30,000, of all ages with epilepsy annually (SUDEP is discussed in my blogs #13, #57 and #68), especially in those whose epilepsy is poorly controlled. Fifty percent of the epilepsy population have more than one seizure/year, i.e., their epilepsy is active and not in remission.
• One-third of the epilepsy population is NOT under the care of a neurologist, the physician-specialty that is more expert in caring for epilepsy patients.
• The greatest number of newly diagnosed patients with epilepsy currently is over 60 years of age, not the pediatric population (see LanceFogan.com blog #15).
• Pregnant women, and those who are considering getting pregnant, should take 400 micrograms of Folic Acid vitamin daily. Folic Acid can lower the incidence of nervous system and other malformations in the fetus. Verbal abilities in children of mothers on antiepileptic drugs (AEDs) who started daily Folic Acid at least four weeks before conception exceed verbal abilities the children of mothers with epilepsy not taking Folic Acid.²

GENETICS IN EPILEPSY:

• Genes that connect with actions of certain anticonvulsants (AED) are now being identified. If these genes are present in the patient a more effective AED can be chosen. Some of these effective medications are not even AEDs but medicines used for other diseases. To date, these genetic laboratories are rare. When two AEDs have been unsuccessful in controlling one’s epilepsy, genetic testing should be done. Genetic testing is expensive, but its use is growing and should benefit many uncontrolled patients. As of 2018, seventy percent of people with epilepsy have no identifiable cause. Genetic screening will probably result in many more identifiable causes.

SURGICAL EPILEPSY TREATMENT:

• Epilepsy is now considered a surgical disease. Epilepsy centers evaluate patients with sophisticated equipment. If an abnormal brain area can be located as the origin of seizures, 60-70 percent are cured of their epilepsy or markedly improved. As scary as brain surgery sounds side effects, as significant thinking, memory, motor or sensory problems, are uncommon. Nonetheless, surgical treatment is underutilized, probably because of fear and expense.

OTHER DEVICES AND TREATMENTS IN EPILEPSY:

• Vagus Nerve Stimulation: in use since 1997. Its effectiveness in decreasing seizures increases over time with its use independent of AEDs. Side effects include cough, hoarseness and shortness of breath because the vagus nerve stimulates these functions of the vocal cords and the respiratory functions. Usually an out-patient procedure.

·         Responsive Neurostimulator: a portion of the external skull surface bone is scooped out for a battery-powered computer (batteries need replacement every 3-5 years) to fit in this bone-bed, then covered with scalp tissue. Thin stimulators are placed into the area of the brain where the seizure originates via two small drill-holes through the skull. Whenever the apparatus detects the beginnings of a seizure the stimulator probes “fire.” This “short-circuits” and halts the seizure. Researchers believe cognition increases over time, probably because fewer seizures occur that interfere with thought. Over one-half of the patients decrease their seizure frequency by 60 percent.

Ketogenic Diet:

            Mostly reserved for intractable epilepsy. Not ideal for good surgical candidates. Effective across the age spectrum. In use since Mayo Clinic doctors in 1924 found this diet efficacious. It consists of 90% of calories from fat and only 10% from carbohydrates (sugars) and protein. The diet is safe and can be effective in children and adults. Ten percent of patients become seizure-free & 40 percent achieve more than 50 percent decrease in seizure frequency at one year. Forty percent remained on the diet at one year due to their successful control. Of 53 percent of the people who discontinued the diet, half was due to poor tolerance and half due to poor seizure control. Response to the diet may take a month to occur.

• Excellent response in most genetic and primary epilepsies (Juvenile Myoclonic, brain malformations and trauma, Dravet, West and Lennox-Gastaut syndromes, infantile spasms, tuberous sclerosis, and others). The ketogenic diet should not be used when certain other conditions exist, usually metabolic diseases.

1)   https://www.cdc.gov/nchs/fastats/deaths.htm 

2)     Husebye ESN, Gilhus NE, Riedel B, et.al. Verbal Abilities in Children of Mothers with Epilepsy. Neurology 2018: 91:e811-e821.

3)     A. Bergqvist in Epilepsy and the Ketogenic Diet; edited  by Stafstrom & Rho, 2004

Lance Fogan, M.D. is Clinical Professor of Neurology at the David Geffen School of Medicine at UCLA. “DINGS” is his first novel. It is a mother’s dramatic story that teaches epilepsy, now available in eBook, audiobook and soft cover editions.

Blog #61: Epilepsy Treatment Is an Art Just as Much as Science

            Medical practice is based as much on your doctor’s experience as it is guesswork. You probably have already come to this conclusion. Teamwork―the collaboration between you (the patient), and your epilepsy-treating physician—is how many decisions are made about whether treatment should be started and, if so, when? Other considerations include the kind of treatment you should receive, how to adjust treatment, and when to stop it?

            Treatment options will vary from patient to patient. Variables to consider include: age, gender, pregnancy considerations, allergies, other illnesses and conditions, and whether the patient needs to drive. For example, a middle-aged person who has had epilepsy since his or her teens has suffered a seizure only when antiepileptic drug blood levels became low and now has been seizure-free for eight years despite occasional low drug blood levels can the anti-seizure medication(s) be stopped? This is a reasonable question: we should not take any medication that’s not indicated. What if the brain scan and EEG are normal? Would this mean the person will remain seizure-free once the medication is stopped? Are the drug levels, despite being low, still protective for that person and if the medication is stopped will seizures recur? It is common for the EEG to be normal in epilepsy. This occurs in up to half of patients because epileptiform activity may not happen during the 60-90 minutes of recording in the EEG laboratory. So, normal EEGs do not discount an epilepsy diagnosis. Consequently, the diagnosis of epilepsy is a clinical one, based on the history of what happens to the patient and not based on any laboratory test.

            Not enough data is available to accurately counsel patients about when and if antiepilepsy medications can safely be discontinued.  If a patient wants to taper off medications after being seizure-free for many years, and the EEG is normal, the advice we can give our patient is, at best, really only guesswork.

Lance Fogan, M.D. is Clinical Professor of Neurology at the David Geffen School of Medicine at UCLA. “DINGS” is his first novel. It is a mother’s dramatic story that teaches epilepsy, now available in eBook, audiobook and soft cover editions.

Blog #41: New Hope for Refactory Epilepsy

(This blog was originally posted on December 24, 2013)

 

 

Epilepsy is a brain disorder causing seizures. It affects 1 percent, or three million, of the American population. Only half of these people can control their seizures with anticonvulsant medications or with surgical removal of the offending brain area.  The treatment efforts in approximately 20-30 percent of people with epilepsy are not effective. A new device offers hope for those whose seizures originate in parts of the brain not amenable to surgical removal. It is not recommended in generalized epilepsy where no isolated seizure focus can be detected.

The U.S. Food and Drug Administration (FDA) approved an implantable neuro-stimulator in November, 2013. The device, called the RNS System (Neuropace, Inc.), is surgically implanted into the skull bone close to the site where the seizures are determined to originate.  Lead wire electrodes from this stimulator extend through the skull bone and are placed on, or sometimes in, the brain. The battery-powered stimulator monitors brain activity. When the onset of abnormal activity is detected, even before the person is aware of the oncoming seizure, the electrodes deliver stimuli that short-circuit the abnormal brain activity and normalize it before a seizure can manifest. The RNS System activates only when it detects seizure activity.

The FDA based its approval on clinical trials of the RNS System involving 191 patients with drug-resistant seizures. They were aged 18-70 and many had uncontrolled epilepsy for years. Half of this patient population had their stimulator turned on and the other half’s stimulator was kept off. After 3 months the average number of seizures per month in the group with the activated stimulator decreased a median of 34 percent. For the patients with the turned-off device, the median reduction in seizure frequency was only 19 percent.

Researchers were encouraged by the significant improved quality of life provided by the RNS System, even allowing many to safely drive vehicles.

The device is designed for people 18 and older whose epileptic seizure onset can be localized by various neurological testing, e.g., EEG and various brain scanning.

According to Neuropace president and CEO Frank Fischer, NeuroPace has done studies involving a total of 256 patients who were monitored for 2-9 years without any significant problems. Its batteries can last several years and be replaced in an out-patient clinic. The cost of the RNS System is still under evaluation.

 

 

Lance Fogan, M.D. is Clinical Professor of Neurology at the David Geffen School of Medicine at UCLA. DINGS is his first novel.

Blog #33: The Tonic-Clonic Seizure

(This blog was originally posted on May 20, 2013)

 

 

 

When people think of a seizure, a convulsing body on the ground is what typically comes to mind. This kind of generalized or “tonic-clonic” seizure is believed to begin with abnormal electrical discharges emanating from deep at the center of the brain—probably in the thalamic region—which spread to the cortex (the brain cell outer-layer) of both hemispheres (halves) of the brain. The thalami are the way-stations for every neurocortical function in the body; sensations are perceived and movement regulation involves this region. Tonic-clonic seizures literally affect all parts of the body, and people often turn blue because the brain “turns off” the breathing response until the seizure has run its course. It is interesting that this type of seizure usually does not affect normal function of the heart.

When this type of seizure occurs, it starts without any warning and usually lasts one or two minutes. The person suddenly stiffens and falls, then jerks and shakes. It is common to bite the lip or tongue, and the person may lose control of the bladder or bowel. It can be very frightening to witness someone having a tonic-clonic seizure; however, the event rarely has serious consequences for the patient unless the person injures part of the body when he or she falls. The seizure stops when it “exhausts itself” and the person is then confused and is unaware of what happened. It can take a few hours until he or she returns to “normal,” and it is common to experience a headache and just want to sleep.

There is no “treatment” for a routine tonic-clonic seizure while it is happening. The immediate concern is to prevent injury by guiding the person to the floor. Turn the individual onto his or her side so any vomit or excessive saliva would fall out from the mouth rather than choking on it and aspirating it into the lungs. This could cause the person to fatally choke or result in pneumonia. It is also important to prevent contact with hot surfaces or water to avoid risk of serious burn or drowning. Convulsions lasting more than several minutes, however, can be dangerous. These interfere with breathing and can cause brain injury. Medical support and anti-seizure medication injections to stop the convulsion then are necessary.

 

Lance Fogan, M.D. is Clinical Professor of Neurology at the David Geffen School of Medicine at UCLA. DINGS is his first novel.

 

 

Blog #23: Epilepsy Treatment: How Effective?

(This blog was originally posted on May 29, 2012)

 

 

        Nearly one percent of the world’s population experiences recurrent seizures, i.e., epilepsy.  That’s approximately seventy million people, including three million in the United States. As patients and their physicians know, treatment often is unsatisfactory.

The May 15, 2012 issue of Neurology reports a study in which Brodie, et al. (1) followed almost 1100 newly-diagnosed epilepsy patients, ages 9-93, between July1, 1982 and March 31, 2008. Their epilepsy was due to various identified structural brain abnormalities (including injuries, strokes and scars, abnormal blood vessels and others), presumptive genetic causes, and unidentified causes.

Treatment consisted of initiation of a single antiepileptic drug considered appropriate for that patient. If the seizures continued then a different antiepileptic drug was substituted or added to the drug regimen. If seizures persisted a third antiepileptic drug was substituted for the other single drug or it was added on.

The researchers concluded that 68% of all of the seizure patients were seizure-free, i.e. had no seizures for at least one year without having to change the treatment. Among these almost 62% were on a single drug.  Forty-nine percent of the study population was seizure-free on the first antiepileptic drug tried. Any individual patient’s treatment success or failure usually became clear soon after initiation of treatment.

Brodie and co-investigators found there was a higher probability of freedom from seizures if only one drug was required for control; there was less confidence of full seizure control if more than one drug was required. Even less seizure control was experienced if more than two drug attempts were necessary. Less than 2% of patients who required multiple trials of medications, however, became seizure free.

This data is helpful for physicians in their discussions with patients with epilepsy. It can reinforce patient compliance with treatment. We can tell our patients that there is significant hope for a good prognosis in treating their newly diagnosed epilepsy.

1.Brodie, MJ, Barry, SJE, Bamagous, GA, et al. Patterns of treatment response in newly diagnosed epilepsy. Neurology. 2012; 78: 1548-1554.

 

 

Lance Fogan, M.D. is Clinical Professor of Neurology at the David Geffen School of Medicine at UCLA. DINGS is his first novel.