Infantile Spasms: What Pediatricians Should Know

Though seizures in children are always worrisome, pediatricians should be especially watchful for infantile spasms, a type of epilepsy that occurs in young infants typically between ages 3 and 8 months, a CHOC neurologist says.

These seizures should be considered a medical emergency due to the potentially devastating consequences on the developing brain, Dr. Mary Zupanc says. Many children with infantile spasms go on to develop other forms of epilepsy because a developing brain undergoing an epileptic storm essentially becomes programmed for ongoing seizures and cognitive/motor delays.

To that end, here’s what pediatricians should look for:

  • Infantile spasms often occur in clusters, with each spasm occurring every five to 10 seconds over a period of minutes ranging from three to 10 minutes or longer.
  • Though there is almost always a cluster of spasms in the morning when the child awakens from sleep, infantile spasms can occur at any time during the day or night.

Infantile spasms can be easily missed because they can mimic common symptoms and conditions such as sleep disturbances, gastroesophageal reflux, startle and shuddering attacks.

Diagnosis, treatment

If infantile spasms are suspected, a pediatrician should quickly refer the child to a pediatric neurologist. CHOC neurologists admit these children urgently for long-term video electroencephalogram (EEG) monitoring to confirm the diagnosis.

Infantile spasms are diagnosed on the basis of clinical spasms, in association with a markedly abnormal EEG showing a hypsarrhythmia pattern. A hypsarrhythmia pattern is characterized by very high amplitude electrical activity and multifocal areas of the brain demonstrating epileptic discharges.

High-dose adrenocorticotropic hormone, or ACTH, is CHOC neurologists’ first line of treatment for infantile spasms, per the American Academy of Neurology’s 2004 practice parameter. Vigabatrin (Sabril), the parameter states, is probably effective in the treatment of infantile spasms, especially in children with tuberous sclerosis and infantile spasms.

If started within four to six weeks of seizure onset therapy has better success at stopping spasms, eliminating the hypsarrhythmia pattern and improving developmental outcomes regardless of etiology.

The course of treatment is approximately six weeks. During this time, and for two to three months after the ACTH course, immunizations should not be administered. The effectiveness of ACTH may be as high as 85 percent, though a recent published study placed the efficacy at a slightly lower percentage, regardless of etiology.

Side effects, causes

Side effects of ACTH, a steroid, include high blood pressure, increased appetite and weight gain, increased sugar in the blood, temporary suppression of the immune system, and sometimes gastritis. All side effects are monitored during the time of the ACTH, and they disappear after the course of treatment.

Successful therapy is marked by two achievements: the cessation of the infantile spasms and the elimination of the hypsarrhythmia pattern. But because clinical spasms can be very subtle and the hypsarrhythmia pattern may sometimes only be seen during deep sleep, therapy’s success can only be confirmed through objective long-term video EEG monitoring.

The etiologies for infantile spasms can include:  tuberous sclerosis; cortical dysplasias; stroke; infection including meningitis and encephalitis; hypoxic-ischemic injury; trauma; or genetic conditions such as Down syndrome and metabolic disorders.

ROSA Robot Assists in CHOC Patient’s Successful Epilepsy Surgery

Five-year-old Ian Higginbotham recently enjoyed his best summer yet.  He experienced his first family vacation. He learned to swim and ride a bike. He got himself ready for kindergarten.  These are milestones most kids and parents, alike, eagerly welcome.  But there was a time when Ian’s parents weren’t certain their son, who was born seemingly healthy, would enjoy such happy pastimes.

Ian began talking and walking in his sleep as a toddler.  When the episodes, including night terrors, increased in frequency and severity, his mom Lisa made an appointment with the pediatrician.  One day, Lisa knew something just wasn’t right and didn’t want to wait for the appointment to get Ian checked out.  She and her husband Derek took him to the Julia and George Argyros Emergency Department at CHOC Hospital.  To her surprise, doctors diagnosed her son with epilepsy.    Ian’s “sleepwalking” and “night terrors” were actually seizures.

The family was referred to CHOC’s comprehensive epilepsy program.  A national leader in pediatric epilepsy care, CHOC’s comprehensive epilepsy program offers cutting-edge diagnostics, innovative medical approaches and advanced surgical interventions.  CHOC was the first children’s hospital in the state to be named a Level 4 epilepsy center by the National Association of Epilepsy Centers, signifying the highest-level medical and surgical evaluation and treatment for patients with complex epilepsy.

CHOC Children's

Ian’s neurologist Dr. Andrew Mower suspected he was experiencing complex partial seizures, which was confirmed by video EEG monitoring.  Complex partial seizures start in a small area of the temporal or frontal lobe of the brain, and quickly involve the areas of the brain affecting alertness and awareness.  The pattern of Ian’s seizures suggested they were originating from the right frontal lobe.  Dr. Mower knew Ian and his family were in for a tough journey.

“I really don’t think the general public understands the impact epilepsy has on a child and his family.  Its effects are multifaceted and extensive.  Our team’s goal is to reduce or eliminate our patients’ seizures, helping improve their quality of life,” explains Dr. Mower, who placed Ian on a series of medications.

The medications reduced Ian’s seizures, but did not control them.  Dr. Mower was concerned about the seizures affecting Ian’s development, and presented his case to the epilepsy team.   The multidisciplinary team agreed Ian was a candidate for epilepsy surgery.  For children who fail at least two medications, surgery may be considered early in treatment versus as a last resort.  Surgery can result in an improvement in seizure control, quality of life, and prevent permanent brain damage.  Ian’s surgery was going to be performed by CHOC neurosurgeon Dr. Joffre Olaya.

While the thought of surgery was frightening to Lisa and her husband, they were confident in the team and comforted to know their son was going to benefit from innovative technology, like the ROSA™ Robot. Considered one of the most advanced robotized surgical assistants, ROSA includes a computer system and a robotic arm.  The computer system offers 3D brain mapping to aid surgeons in locating the exact areas they need to reach and planning the best surgical paths.  The robotic arm is a minimally invasive surgical tool that improves accuracy and significantly reduces surgery/anesthesia time.

Dr. Olaya used ROSA to accurately place electrodes in the area of Ian’s brain suspected to be the source of his seizures.  By using the robot, Dr. Olaya avoided performing a craniotomy.

“ROSA is an amazing tool that yields many benefits for our patients, including less time under anesthesia in the operating room.  It reduces blood loss and risk of infections.  Patients tend to recover faster than they would if they had craniotomy,” says Dr. Olaya.

Lisa was amazed at the outcome. “I couldn’t believe how great Ian looked after the placements of the electrodes with ROSA.  He wasn’t in any pain, there was no swelling.  It was wonderful!”

She and her husband were also amazed at how well Ian did following his epilepsy surgery.

“We got our boy back,” says Lisa. “There were no more side effects from medication and, more importantly, no more seizures!  He started developing again and doing all the things a child his age should do.”

Ian’s care team isn’t surprised by his recovery.

“Children are resilient, and their brains are no different.  In fact, the plasticity of a young brain allows it to adapt to changes and heal more easily than an adult brain,” explains Dr. Mower.

Learning to ride a bike and swim were among the first of many milestones Ian quickly reached following surgery.  He enjoys playing with his younger brother and his friends.  And, whether inspired by his experience with ROSA or not, Ian loves robots.

Meet Dr. Anjalee Warrier Galion

CHOC wants its referring physicians to get to know its specialists. Today, meet Dr. Anjalee Galion, a pediatric neurologist and sleep specialist.

CHOC Children's

Q: What is your education and training?
A:  I attended the University of Medicine and Dentistry of New Jersey- New Jersey Medical School, and completed my residency in pediatrics at University of San Francisco, Fresno.  During my academic year, I worked for Walter Reed Army Institute of Research to help identify a vaccine for malaria. My second day was September 11, 2011, and it was an amazing, humbling, and scary experience to be a part of the military for this day. My first fellowship was in pediatric neurology at the University of California, Irvine (UC Irvine), and my second fellowship was in sleep medicine at the University of California Los Angeles- Cedars Sinai Sleep Medicine Fellowship.

Q: What are your administrative appointments?
A: Assistant clinical professor at UC Irvine, assistant program director for the UC Irvine child neurology residency program, chair of the junior faculty leadership council, and co-chair of the sleep workgroup for the National Autism Treatment Network.

Q: What are your special clinical interests?
A: Sleep disorders in children with neurologic diseases such as epilepsy and autism, as well as sleep and cognition.

Q: Are you involved in any current research?

A: Evaluation of efficacy of specific sedative hypnotics in children with Autism spectrum disorder, and Identification of sleep architecture and pathology in children with epilepsy.

Q: How long have you been on staff at CHOC?
A: Four years.

Q: What are some new programs or developments within your specialty?
A: CHOC is one of the few, if only, hospitals in the country doing combined long-term video EEG as well as polysomnography (sleep study).  This allows us a very unique opportunity to look at the brain activity and pathology in sleep. Multidisciplinary sleep clinics involving psychology and pulmonology are also unique. Also, identification and treatment of a variety of pediatric sleep disorders including all types of insomnia, narcolepsy and parasomnias, such as sleepwalking/sleep talking/night terrors.

Q: What are your most common diagnoses?
A: Insomnia, narcolepsy, sleepwalking (or somnambulism).

Q: What would you most like community/referring providers to know about you or your division at CHOC?
A: We treat all types of sleep-related disorders and are providing state-of-the-art care for children with sleep disorders. It is estimated that more than 30 percent of children have sleep-related disorders, and improvement in sleep is essential for learning and cognition. Research suggests improved sleep supports optimal athletic performance as well. If there is any concern for a sleep-related disorder we are happy to help evaluate these children.

Q:  What inspires you most about the care being delivered here at CHOC?
A: We treat every child with the highest level of care and the physicians genuinely care for the patients and our community.

Q: Why did you decide to become a doctor?
A: I had been interested in neuroscience since I was a Howard Hughes fellow at the University of Maryland, having done work in spinal cord regeneration. I heard a talk in my first year of medical school about pediatric neurology and haven’t looked back since.

Q: If you weren’t a physician, what would you be and why?
A: If I was not a physician I would mostly likely be a PhD working in the field of neurobiology and sleep medicine. Both my parents were PhDs and I grew up hearing about fascinating advances in the world of science, so I have been drawn to science and research from a young age. For quite a few years I was strongly considering becoming a professional flute player. I was fortunate enough to travel through Italy with my youth symphony and performed around the country in orchestras, but science drew me in by the time I was in college.

Q: What are your hobbies/interests outside of work?
A: I enjoy hosting parties and events for family and friends. Our family enjoys traveling and spending time together.

Q: What have you learned from your patients?
A: The best part about working with pediatric patients is that you are constantly reminded to enjoy life and be grateful for every day. The smallest things can make a child happy, like playing with a light-up toy or seeing bubbles, and it is a great reminder to take pleasure in all the simple things around us every day.

Meet Dr. Andrew Mower

CHOC wants its referring physicians to get to know its specialists. Today, meet Dr. Andrew Mower, a pediatric neurologist.

Dr.Andrew_Mower_0699_2

Q: What is your education and training?
A: I attended medical school at St. George’s University School of Medicine in Grenada, West Indies. My residency in pediatrics was at Maimonides Medical Center in New York.  My residency in child neurology was at State University of New York Downstate (SUNY Downstate) in New York. My fellowship in clinical neurophysiology was at Columbia University in New York.

Q: What are your administrative appointments?
A: Quality Improvement Committee since 2016; Ancillary and Diagnostic services since 2015.

Q: What are your special clinical interests?
A: Epilepsy and epilepsy surgery

Q: How long have you been on staff at CHOC?
A: Two years

Q: What are some new programs or developments within your specialty?
A: Stereotactic EEG for epilepsy surgery

Q: What are your most common diagnoses?
A: Epilepsy and headaches

Q: What would you most like community/referring providers to know about you or your division at CHOC?
A: We take on the most challenging epilepsy cases to search for cures, and, if not, at least an improvement in the child and family’s quality of life. We work together as a team to use all of our expertise to help the child and family.

Q:  What inspires you most about the care being delivered here at CHOC?
A: I feel that we offer families hope when hope had been abandoned before.

Q: Why did you decide to become a doctor?
A: I decided to become a doctor to help people in need. I chose neurology as my specialty because I had a fascination with the nervous system and wanted to break misperceptions that little can be done for patients with neurological problems.

Q: If you weren’t a physician, what would you be and why?
A: Farmer. I love the accomplishment of creating a garden and growing produce.

Q: What are your hobbies/interests outside of work?
A: Gardening, running and hiking

Q: What was the funniest thing a patient told you?
A: “I don’t eat apples, doctor.”

“Why?”

“Because they keep the doctor away, and I like you, Dr. Mower.”

CHOC Grand Rounds Video: Cognitive Side to Mental Health and the Psychology Behind Concussions

A concussion or mild traumatic brain injury is defined as a transient neurologic change resulting from a biomechanical impact to the head. Given this broad definition, it is not surprising that concussion represents the most common type of traumatic brain injury (TBI). Concussions can be complicated and multifaceted, as patients usually present with various combinations of neurologic, cognitive and psychiatric symptoms, Drs. Sharief Taraman and Jonathan Romain said in a recent grand rounds presentation at CHOC.

Adolescents represent a commonly seen subgroup within the concussion population, most notably because of their frequent involvement in sports and higher-risk activities. Additionally, when injuries do occur at the high school and college level, the impact velocities tend to be at a higher rate than is seen in younger athletes, potentially resulting in more pronounced concussions. Further complicating the situation is that adolescents tend to have busy schedules and multiple responsibilities throughout the school year (when most concussions occur). Thus, when a concussion is sustained, the student athlete not only needs to deal with the immediate symptoms of the injury, but also the potential for academic and social derailment during the recovery process. Combine these issues with a strong body of literature suggesting adolescents tend to have slower resolution than do adults, and you have the recipe for a very bumpy recovery.

The doctors explain that cognitive symptoms manifest as slower processing speed, feeling foggy, and occasional forgetting or transient confusion.  Psychiatric symptoms often include irritability, liability and sadness. A child may have one or many of these symptoms, although more often these symptoms overlap. The patient and their family may not recognize how persistent symptoms of headache and dizziness, for example, can contribute to memory problems and difficulty concentrating, irritability, and feelings of depression and hopelessness. Children with prolonged symptoms also can feel isolated from their peers while they are sitting out of play and school.

Learn more about CHOC’s Concussion Program.

View previous grand rounds videos.