Sleep Terrors Or Nocturnal Frontal Lobe Epilepsy? Gillette Patients Who Have a Pediatric Perspective, Please Send an E Mail to Publications@Gillettechildrens.Com

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2011-20-2 PedsPerspectives 2_06/15-2 Ped Perspectives 9/2/11 2:38 PM Page 1

Author Nonprofit A PEDIATRIC Organization Profiles U.S. Postage A PEDIATRIC Persp ective P A I D Twin Cities, MN Permit No. 5388 Volume 20, Number 2 2011 200 University Ave. E. St. Paul, MN 55101 651-291-2848 John Garcia, M.D. A Pediatric Perspective focuses on specialized topics erspective TTY 651-229-3928 P Volume 20, Number 2 2011 Sleep Medicine Specialist in pediatrics, orthopedics, neurology and rehabilitation 800-719-4040 (toll-free) medicine. www.gillettechildrens.org A board-certified sleep specialist, John Garcia, M.D., works with To subscribe to or unsubscribe from Sleep Terrors or Nocturnal Frontal Lobe Epilepsy? Gillette patients who have A Pediatric Perspective, please send an e mail to [email protected]. disabilities and associated sleep by John Garcia, M.D., and Nicole Williams, M.D. disorders. Such disorders include Editor-in-Chief...... Steven Koop, M.D. obstructive sleep apnea, Editor...... Ellen Shriner sleepwalking, circadian rhythm would not be as common in a school-aged child. Additionally, Designer...... Kim Goodness Introduction disorders, and restless leg Differentiating sleep terrors from nocturnal seizures can ask parents if the child has a history of daytime staring spells John Garcia, M.D. Photographers...... Anna Bittner syndrome. He uses a combination be challenging. In both instances, patients seem to awaken or seizures, unusual posturing, limb jerking, or a change in of behavior management, medications and other therapies ...... Paul DeMarchi suddenly from non-REM sleep; they may scream, appear skin color during the episodes. If the parents observe any in his practice. agitated, and move their arms and legs. However, there are such daytime symptoms, or if the child has a neurodevelop- important differences to look for when making a diagnosis. mental disability, consider a video EEG to establish or rule Garcia is a graduate of the University of Iowa School of Copyright 2011, Gillette Children’s Specialty Healthcare. out seizures. Medicine. He completed a residency in pediatrics and one All rights reserved. Sleep Terrors year of fellowship training in behavioral/developmental Treatment Options pediatrics at Riley Hospital for Children in Indianapolis. He Description Reassure parents that sleep terrors are common and that then completed a sleep fellowship equivalent at the Sleep terrors (also called “night terrors”) are one example of most children outgrow them by the time they are school-age. Minnesota Regional Sleep Disorders Center in Minneapolis. a disorder of arousal, a common type of parasomnia. Other Recommend that parents intervene as gently as possible. His professional associations include the American Board examples include everything from calm sleepwalking to For example, parents should not try to wake up the child. of Sleep Medicine and the American Board of Pediatrics. emotionally agitated or complex behaviors such as dressing Instead, they should simply guide the child back to bed and or eating while asleep. Up to 17 percent of preschool-aged help settle him or her. If the child sleepwalks, recommend UPCOMING CONFERENCES Gillette Welcomes New Sleep children experience disorders of arousal.1 safety precautions such as securing windows, putting a gate Nicole Williams, M.D. at the top of stairs, and adding a motion detector alarm in Health Specialist As its name suggests, during a disorder of arousal the person Pediatric Neurologist For orthopedic surgeons and allied specialists the child’s bedroom doorway to alert parents that the child is Minnesota Memorial Pediatric Orthopaedic Laurel Wills, M.D. is only partially awake. Classic sleep terrors occur in the first sleepwalking. If the sleep terrors are particularly dramatic Nicole Williams, M.D., is a board- Symposium half of the night, usually in the first 60 to 90 minutes of sleep, and nocturnal seizures have been ruled out, consider Sept. 22, 2011 and the child arouses suddenly out of deep non-REM sleep. certified general pediatric Laurel Wills, M.D., is a specialist in pediatric and prescribing a low dose of clonazepam at bedtime. neurologist who specializes in the Typically, the child has no memory of the sleep terror For speech and language pathologists adolescent sleep medicine, with a particular focus care of children who have epilepsy, episode. Cleft Lip/Palate and Craniofacial Care: on caring for children and youth who have Nocturnal Frontal Lobe Epilepsy cerebral palsy, and developmental The Modern Team Approach Conference delays, particularly neurologic Oct. 15, 2011 developmental disabilities. Children experiencing sleep terrors may display some or Description conditions that appear during the all of these symptoms: disorientation; emotional outbursts, Nocturnal Frontal Lobe Epilepsy (NFLE) is characterized by such as screaming and the appearance of fear; motor activity, frontal lobe seizures that occur primarily during sleep and infant and toddler years. For patients and families After receiving her medical degree from Boston Laurel Wills, M.D. Nicole Williams, M.D. such as flailing or running in sleep; and profound autonomic may mimic disorders of arousal in which the patient’s Childhood-Onset Hydrocephalus Conference University School of Medicine, Wills completed her Williams graduated from the University of Minnesota Sept. 24, 2011 discharges, such as flushing, sweating and tachycardia. behavior is agitated. Both sporadic and familial forms of NFLE pediatrics residency at the University of Chicago Medical Center and La Rabida Medical School and completed a pediatric residency and a Because of the intensity of activity during sleep terrors, exist. Although the syndrome’s typical onset occurs between child neurology residency at Stanford University/Lucile Children and Adults Who Have Cerebral Palsy: Children’s Hospital in Chicago. She went on to finish her fellowship in patients can unintentionally hurt themselves or others during 7 and 12 years, NFLE has been documented in patients from Packard Children’s Hospital in Palo Alto, Calif. In addition, The Road Map for Advocacy and Medical Care developmental and behavioral pediatrics, including training in pediatric sleep an episode. The duration of the episodes varies, and each infancy to adulthood. she completed elective clinical rotations in neonatal Oct. 29, 2011 medicine, at Children’s Hospital in Boston. Wills is board-certified in general episode usually ends abruptly, with the child returning to a deep sleep. Typically, sleep terrors decrease in frequency During a NFLE seizure, patients often exhibit behaviors that neurology at the University of California, San Francisco pediatrics, developmental-behavioral pediatrics, and sleep medicine. Her Visit www.gillettechildrens.org/ and dramatic quality as the child gets older, and children resemble sleep terrors: School of Medicine and pediatrics at LAMB Hospital in professional memberships include the American Academy of Pediatrics and to learn more or enroll. 2 Bangladesh. Her studies also included a course in designing MedicalEducation usually outgrow them by the time they reach school age. • Sudden, explosive arousal from non-REM sleep, often within the American Academy of Sleep Medicine. clinical research at the University of California, 30 minutes of falling asleep San Francisco School of Medicine. Diagnosis • Vocalizations, including screaming or laughing Clinical evaluation is sufficient; polysomnograms and video • Arm and leg movements, such as fencer posturing (one arm electroencephalograms (EEGs) are typically not required. extended while the other flexes); kicking or bicycle-pedaling To obtain back issues of A Pediatric Perspective, When evaluating a child for sleep terrors, it is important to motions of legs; rocking; pelvic thrusting; or tonic stiffening log on to Gillette’s website at of the limbs 6 ask if the episodes occur shortly after the onset of sleep or www.gillettechildrens.org/pediatricperspective. later in the night. Another consideration is the age of the • Returning to sleep immediately after the seizure Issues from 1998 to the present are available. child, because sleep terrors usually affect young children and Continued on Page 2 2011-20-2 PedsPerspectives 2_06/15-2 Ped Perspectives 9/2/11 2:38 PM Page 2

To further confuse matters, most patients who have NFLE Case Study – Sleep Terrors have normal CT scans and MRIs. Additionally, EEGs of patients Frontal Lobe Epilepsy Scale (FLEP) experiencing NFLE are often inconclusive and uninformative, History because a large portion of the frontal lobe cortex is undetected Gillette’s Sleep Health Clinic Characteristic Score by routine scalp electrodes, and frequent muscle artifacts The parents of a 3½-year-old boy brought their son to Gillette’s interaction with the patient, it was evident that he was well- Gillette’s Sleep Health Clinic is part of our Center for during motor seizures can obscure the EEG recording. EEGs Sleep Health Clinic for an evaluation of his sleep difficulties. adjusted, and the sleep disruptions were not related to Age of onset > 55 years –1 Pediatric Neurosciences, which offers interdisciplinary recorded during the ictal period might not capture seizure They described a history of nearly nightly episodes in which emotional trauma. Duration < 2 minutes +1 services and advanced neurodiagnostic capabilities activity for as many as 44 percent of patients.3 2-10 minutes 0 their son would seem to wake up screaming within 60 to 90 for patients with neurologic conditions. In keeping Treatment > 10 minutes –2 minutes of falling asleep. When they entered his room, they with our pediatric focus, Gillette’s Sleep Health Clinic Given the similarities in symptoms, it would be possible to His history and symptoms represent a classic case of sleep 1-2 0 would find him sitting upright in bed with his eyes open, yet he is dedicated to meeting the needs of children, teens mistake NFLE for sleep terrors. Children with both disorders Number of events terrors, so education and reassurance seemed the best course 3-5 +1 and young adults who have disabilities. We also care suffer from chronically disrupted sleep, and parasomnias and in a night would not recognize his parents. He often would be sweating, of action. The parents were reassured that their son was not > 5 +2 for typically developing children who have sleep epilepsy may occur in the same individual. However, several breathing fast and looking panicked. The episodes usually having seizures and that parasomnias do not signal emotional Time of night 30 minutes of sleep onset +1 disorders. The Sleep Health Clinic is accredited by the characteristics typically associated with NFLE are not seen lasted less than 15 minutes and ended as abruptly as they distress. Additionally, they were instructed to establish an earlier Aura associated? +2 American Academy of Sleep Medicine. with sleep terrors. and more regular bedtime to decrease their son’s sleep Wander outside bedroom? –2 began. The following day, the patient would have no memory of deprivation and help prevent episodes. As a result of those Complex behaviors? the episode. The family learned that if they did not try to wake steps, the frequency of the night terrors went from nearly nightly Diagnosis Pick up objects? Dress? –2 their child or console him, the episodes were shorter. The to a very tolerable once or twice a week. No medication was During clinical evaluation, several factors point to NFLE rather Dystonic posturing, patient’s parents said they became so accustomed to the required. One year later, their son experienced a 14-night than sleep terrors: tonic limb extension? +1 episodes that they could “set their watch” by them. Because of stretch of sleep terrors when school started because he was • Child is school-age rather than preschool-age. Highly +1 Stereotypic the frequency of the episodes, the parents learned to delay their not getting enough sleep. Re-establishing rigorous sleep • Seizures are brief, lasting seconds to less than two minutes, Uncertain 0 movement hygiene was sufficient to reduce the number of episodes. and frequently occur up to 20 times per night instead of one Variable –1 own bedtime so they could resettle their son first. to two times per night. Recall? Yes +1 • Episodes typically include stereotypic movement. No 0 Evaluation • Some patients experience a nonspecific aura of Visit www.gillettechildrens.org/SleepMedicineVideo Vocalization Coherent speech with –2 When questioned further, the parents said the patient’s bedtime to view a video about the Sleep Health Clinic. somatosensory, sensory, psychic or autonomic symptoms. incomplete or no recall ranged from 8:30 to 10:30 p.m., which indicated he was • Patient may recall episodes. Coherent speech with recall +2 somewhat sleep-deprived. After observing the parents’ • Patient has a history of daytime sleepiness, daytime seizures, or neurodevelopmental disabilities or has a family history of Scores < 0 = Likely to be sleep terrors epilepsy. Scores > 0 = Likely to be NFLE Case Study – NFLE When some of these factors are present, using the Frontal Lobe Epilepsy (FLEP) scale4 can be helpful (see box at right). For History typical parasomnias such as sleep terrors, the FLEP score will Sleep Terrors vs. Nocturnal Frontal After an 11-year-old boy experienced a first generalized The clinical events were consistent with frontal lobe seizures, be less than zero. For NFLE, the score will be greater than zero. Lobe Epilepsy convulsive seizure, his parents brought him to see a Gillette and the patient’s video EEG showed bursts of frontal If the patient history or FLEP score indicates potential NFLE, Parameter Sleep Terrors NFLE neurologist for evaluation. The seizure occurred at school and epileptiform discharges during the episodes — confirming NFLE. an overnight video EEG is required to establish the diagnosis. lasted five to 10 seconds. A routine EEG performed prior to the Typical age of Preschool 7-12 years Treatment Resources onset visit was normal. Treatment Options Oxcarbazepine was started. At a two-month follow-up 1 Ohayon M, Guilleminault C, Priest R. Night terrors, sleepwalking and confusional arousals in the general Antiseizure medications, including carbamazepine, oxcar- Number of 1-2 1-20 or more Evaluation appointment, the patient’s parents reported that he had had population: Their frequency and relationship to other sleep bazepine, lamotrigine, topiramate, and levetiracetam, are used episodes/night When questioned further, the patient’s parents reported that a no further daytime seizures. However, his nocturnal seizures and mental disorders. J Clin Psychiatry 1999; 60 (4):268- to treat NFLE. A third to a half of patients continue to have few times per month, the boy also had episodes during sleep — remained, although they had decreased somewhat in 76. seizures despite medical treatment. In refractory patients, Behaviors Moving arms and legs Moving arms and legs, raising suspicions that he might have NFLE. They characterized frequency. The dose of oxcarbazepine was increased with no epilepsy surgery or a vagus nerve stimulator may be stereotypic movement 2 Kryger M, Roth T, Dement W. the episodes as follows: the boy would wake up one to two further improvement in nocturnal seizure control. Therefore, Principles and practice of considered. Walking around is, sleep medicine. 3rd ed. Philadelphia, (PA): W.B. Saunders lamotrigine was added and his nocturnal seizures disappeared. Possibly walking rare hours after falling asleep, sit up, scream and then speak. Company, 2000. Conclusion around Afterward, he would fall back asleep, and he had no memory 3 Sleep terrors and NFLE seizures have some similarities. Vocalizing, screaming, of the episodes in the morning. Provini F, Plazzi G, Tinuper P, Vandi S, Lugaresi E, and With closer clinical evaluation and proper diagnostic testing, Possibly vocalizing or laughing Montagna P. Nocturnal frontal lobe epilepsy: A clinical and however, the two conditions can be distinguished. The sleep screaming polygraphic overview of 100 consecutive cases. Brain Recall is more likely To help pinpoint a diagnosis, the patient had an overnight video (1999), 122, 1017–1031. health medicine specialists and pediatric neurologists at No recall of episode Gillette Children’s Specialty Healthcare’s Center for Pediatric May have an aura EEG that captured several potential seizures during sleep. The 4 Derry CP, Davey M, Johns M, Kron K, Glencross D, Marini Neurosciences can be a resource for suspected cases of NFLE No aura events were highly stereotyped: the patient started to adjust his C, Scheffer IE, Berkovic SF. Distinguishing sleep disorders or sleep terrors. position in bed, and then made kicking movements, sat up and Arch Neurol Prognosis Usually persists into from seizures: diagnosing bumps in the night. Often resolves by let out a scream. He fell back to sleep immediately afterward. 2006; 63(5):705-9. school age adulthood

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