TRANSITION OF EPILEPSY CARE FROM CHILDREN TO ADULTS
Transition issues for benign epilepsy with centrotemporal spikes, nonlesional focal epilepsy in otherwise normal children, childhood absence epilepsy, and juvenile myoclonic epilepsy *†Carol S. Camfield, ‡Anne Berg, §Ulrich Stephani, and ¶Elaine C. Wirrell Epilepsia, 55(Suppl. 3):16–20, 2014 doi: 10.1111/epi.12706
SUMMARY
Dr. Carol Camfield is professor emeritus at Dalhousie University.
This chapter covers the syndromes of benign epilepsy with centrotemporal spikes (BECTS), nonlesional focal epilepsy in otherwise normal children (NLFN), and the genetic generalized epilepsies. BECTS is an epilepsy syndrome that always enters terminal remission before the general age of a planned transition of adolescents. This is also the case for the majority (65%) of those with childhood absence epilepsy (CAE). Approximately 15% of patients with CAE who initially remit during their childhood years later develop juvenile myoclonic epilepsy (JME) as teenagers. They will have many issues for continuing medical care and transition, because their seizure disorder generally persists into adulthood. A significant minority of NLFN (~35%) and most patients with JME continue to have active epilepsy into adulthood. In addition, CAE, JME, and NLFN patients are at risk of a number of significant adverse social outcomes that require ongoing advice and counseling. KEY WORDS: Benign epilepsy with centrotemporal spikes, Nonlesional focal epilepsy, Childhood absence epilepsy, Juvenile myoclonic epilepsy, Transition, Adulthood, Adolescence.
Benign Epilepsy with Centrotemporal Spikes (BECTS) BECTS is characterized primarily by nocturnal seizures at sleep onset or just before awakening. In 75% of cases the disorder begins between 7 and 10 years of age. During the active phase, cognitive, linguistic, and behavioral difficulties of minimal to moderate intensity may occur. In all
cases, medical, educational (if needed), and psychological advice (if needed) with support to patients and families is important and should regularly be reviewed during childhood. Less than 2% of patients have a more severe course associated with treatment difficulties prior to terminal remission, at times with a rare evolution to an epileptic encephalopathy. Remission usually occurs within 3–4 years of onset and almost always before the age of 16 years.1 This excellent outcome is independent of any form of antiepileptic treatment. In addition, a recent population-based study found that 30 years after initial diagnosis of BECTS, all patients were in complete remission and continued off all antiepileptic medications.2 The social outcomes of education, employment, poverty, psychiatric disorders, or loneliness
Accepted May 30, 2014. *Department of Pediatrics, Dalhousie University, Halifax, Nova Scotia, Canada; †Department of Pediatrics, IWK Health Centre, Halifax, Nova Scotia, Canada; ‡Epilepsy Center, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, Illinois, U.S.A.; §Neuropediatric Department, University of Kiel, Kiel, Germany; and ¶Department of Neurology, Child and Adolescent Neurology, Mayo Clinic, Rochester, Minnesota, U.S.A. Address correspondence to Carol S. Camfield, Department of Pediatrics, Dalhousie University and the IWK Health Centre, University Ave., Halifax, NS, Canada B3K 6R8. E-mail: [email protected] Wiley Periodicals, Inc. © 2014 International League Against Epilepsy
16
17 Transition: Generalized and Benign Focal Epilepsies
Key Points BECTS will enter terminal remission by the age of 16 years and the social outcome in adulthood reflects that of the general population. No transition plans need to be made. CAE and NLFN are outgrown by late adolescence in 65% and persist for the remainder. JME usually persists during adolescence. In many with CAE, NLFN, and JME long-lasting care and/or psychosocial assistance is required. and isolation, marriage, and divorce were similar to in the general population. Although occasional cognitive difficulties were apparent during the active phase of their epilepsy in childhood, these did not appear to have significant longlasting effects. In general, neurologists or psychiatrists do not follow or treat young adults with BECTS for any epilepsy-related social or medical issues.
Nonlesional Focal Epilepsy in Otherwise Normal Children (NLFN) This epilepsy group presents with focal seizures (electroencephalography [EEG] and semiology) and normal clinical anatomic imaging, neurologic examination, and intellectual ability. Young people with NLFN represent 20–30% of children with epilepsy.3–5 After long-term follow-up (>10 years after the initial diagnosis), two thirds will enter terminal remission (TR), defined as having at least 5 years seizurefree and off antiepileptic drugs (AEDs)3–5 (also called “complete remission”) (Table 1). Although there is always the potential for subsequent relapse, the risk of relapse is low once TR been achieved: 10 year follow-up
19% with >10 year follow-up
– 67 33
110/131 (84%) 67 33
7
~13
12
Wirrell et al., Berg et al., Camfield and Camfield.
5
Epilepsia, 55(Suppl.3):16–20, 2014 doi: 10.1111/epi.12706
18 C. S. Camfield et al. remaining one third, seizures persist either as absences alone (10–15%), absences with either generalized tonic– clonic or myoclonic seizure (5–15%), or evolution to JME (5–15%). In considering a diagnosis of CAE, several, less common forms of absence epilepsy that are often therapy resistant must be excluded. These include myoclonic absence epilepsy, epilepsy with eyelid myoclonia (Jeavon’s syndrome), perioral myoclonia with absence, as well as absences associated with specific metabolic (glucose transporter deficiency) or chromosomal (ring chromosome 20, microdeletion 15q13.3) anomalies. Cognitive concerns are reported in several large case series14 and include subtle cognitive deficits and linguistic difficulties, which are frequently complicated by ADHD or associated with affective/anxiety disorders. There appears to be a greater risk with longer illness duration and higher seizure frequency. In addition, certain genetic abnormalities portend a greater risk of intellectual disability.15 The social outcome of children from the Nova Scotia Childhood Epilepsy Study with CAE, who were >18 years of age at time of study (n = 56) were compared to young adults with an underlying chronic disorder, juvenile rheumatoid arthritis (n = 61).7 Surprisingly, those with prior CAE were more likely to require special educational help (p < 0.02), have below average academic performance (p < 0.01) or repeat a grade (p < 0.005), become inadvertently pregnant (p < 0.001), and less likely to graduate from high school (p < 0.005) or attend college/university (p < 0.001). No studies have yet followed children with CAE into adulthood to evaluate social success.
Juvenile Myoclonic Epilepsy (JME) JME usually begins in adolescence between 12 and 18 years, although it may occur earlier in childhood or even in the third or later decades.16 Approximately 5–15% were previously diagnosed with CAE, experienced TR (seizurefree and >5 years off AED treatment), and then present with recurrent seizure types that met the criteria for JME. JME has been considered for many years to be a lifelong disorder with a high recurrence rate after withdrawal of AEDs.16 Two large case series document that up to 25% can discontinue AEDs entirely and remain seizure-free.17,18 In addition, myoclonic seizures often may decrease or disappear in the fourth decade. However, a pharmacoresistant course is observed in ~15%. A population-based study reported the medical and social outcomes of JME patients (n = 23) with at least 25 years follow-up after initial diagnosis (average age of 36 years).8 At the end of the followup, 13% (3/23) had intractable epilepsy, whereas 26% (6/23) were seizure free and off AED treatment (mean 17 years). In addition, 13% (3/23) experienced only mild myoclonic seizures, which persisted for up to 22 years after stopping AED treatment. The myoclonus was not considEpilepsia, 55(Suppl.3):16–20, 2014 doi: 10.1111/epi.12706
ered problematic by these adults. In summary, 39% of the cohort eventually stopped AEDs and became seizure free or experienced only mild myoclonic seizures. Although most people with JME have a lifelong disorder, this study indicates that there are some with a relatively short active phase (4–5 years) followed by a long-term remission with no requirement for continued treatment. It seems reasonable to consider a trial of stopping AED treatment in late adolescence or early adulthood for people with JME who are well controlled, depending on life issues such as driving. It is possible that the relatively optimistic rate of remission is related to age, inasmuch as seizure-provoking factors such as binge drinking and sleep deprivation are likely to decrease over time.
Suggestions at the Time of the Initial Transition Meeting Although the adolescent with normal intelligence and neurologic examination without neuroimaging abnormalities has an already established diagnosis and management plan, the time of transition is an opportunity for reassessment. Issues to be considered or reexamined are listed in subsequent text; however, there is little evidence to document effectiveness of this approach (Table 2). 1 Is the diagnosis correct? Is the present control of seizures adequate? Is the current AED choice and dosage appropriate? 2 Are side effects present? Are there any possible interactions with oral contraceptives, alcohol, or other drugs, and have they been discussed with the adolescent or young adult? Many children with epilepsy are diagnosed in their early childhood years and may have never personally received counseling regarding potential adverse effects of their medication. Furthermore, in adolescence and early adulthood, certain potential side effects may be
Table 2. Suggestions for discussion at the time of transition of adolescents with normal anatomic imaging, neurologic examination, and intellectual ability Education about epilepsy, treatments, and their side effects Ensure appropriate counseling regarding substance abuse, contraception, and sexual health and sleep hygiene has taken place Evaluate the adolescent’s psychological and social well-being Assess the home or living environment How cohesive, supportive and appropriate is it? How long will the adolescent remain in this setting and what is planned in the future? Discuss the learning environment and its appropriateness to the teen’s cognitive ability. Is neuropsychological testing needed? Is comorbid ADHD present and appropriately treated? Are vocational and higher education plans and goals reasonable? When appropriate, sensitively discuss the risk of death associated with SUDEP
19 Transition: Generalized and Benign Focal Epilepsies more concerning, in particular the risk of polycystic ovarian syndrome and teratogenicity with valproate, slowness of verbal processing with topiramate or zonisamide, potential interaction of AEDs with oral contraceptives, effect of AEDs on bone health and body mass index, and potential interactions with alcohol or other drugs. 3 Although not specifically linked to sudden unexplained death in epilepsy (SUDEP), the discussion of the potential risk of SUDEP in young people sometimes arises. For both CAE and NLFN-TR, the risk of death to the individual is comparable to the leading causes of death in the population, including accidents, homicide, suicide, cancer, and heart disease.19 The JME group with persistent, frequent generalized tonic–clonic seizures, however, represents those at particular risk of this devastating outcome. For these individuals, a serious but sensitive discussion of the risks and possible suggestions for decreasing the risk of SUDEP should take place with not only the parents, but also the adolescent. 4 A successful process requires both a well-thought out process and plan, which allows the teenager to assume greater self-empowerment and independence, and a team of professionals, which may include physicians, nurses, social workers, and psychologists/psychiatrists. Topics for discussion should cover: a. Education about epilepsy and treatments and their side effects. b. Discussion of the learning environment and its appropriateness to the teen’s cognitive ability. Children and teens with epilepsy have higher rates of subtle cognitive deficits and ADHD, which can affect academic success. One must assess whether the teen’s learning environment is appropriate for their cognitive level, and whether neuropsychological testing is needed. In addition, comorbid ADHD may require treatment. Young adults with epilepsy are less likely to be employed in their field of study and often have job dissatisfaction. 20 Thus, future goals and vocational counseling as well as plans for higher education should be discussed. c. Evaluation of the adolescent’s psychological and social well-being is needed. Affective disorders including anxiety and depression are known to affect a significant minority of young adults with epilepsy and often remain untreated. Screening for such a disorder should be performed at the time of transition and involve psychiatry/social work, as needed. d. Evaluation of the present home environment. Is it adequate, cohesive, and able to be supportive? How long will the adolescent remain in this setting and what is planned for the future? e. Ensure appropriate counseling regarding substance abuse, contraception and family planning, genetic counseling, sexual health, and sleep hygiene.
Addressing each of these areas is difficult in a single session and is often overwhelming for both the adolescent and family. It may be helpful to have several meetings with a multidisciplinary team to be certain that all of the correct information is obtained by the Transition Team and understood by the teen and his/her family.
Disclosure or Conflict of Interest Carol Camfield, Peter Camfield, and Elaine Wirrell have no disclosures. Anne Berg has received speaker honoraria and travel support from BIAL and Medical University of South Carolina, travel support from the International League Against Epilepsy (ILAE), and an honorarium from The American Academy of Neurology, Continuum. She serves on advisory boards for Citizens United for Research in Epilepsy (CURE) and Eisai, serves on the editorial boards of Epilepsy & Behavior and Neurology, and is supported by funding from the National Institute of Neurological Disorders & Stroke (NINDS) (Grant R37-NS31146) and the Pediatric Epilepsy Research Foundation. Ulrich Stephani serves on advisory boards of the pharmaceutical companies Desitin, Eisai, and Viropharma and receives support from Deutsche Forschungsgemeinschaft DFG (German Research society) and the German chapter of the ILAE. We confirm that we have read the Journal’s position on issues involved in ethical publication and affirm that this article is consistent with those guidelines.
References 1. Bouma PA, Bovenkerk AC, Westendorp RG, et al. The course of benign partial epilepsy of childhood with centrotemporal spikes: a meta-analysis. Neurology 1997;48:430–437. 2. Camfield CS, Camfield PR. Rolandic epilepsy has little effect on adult life 30 years later: a population-based study. Neurology 2014;82:1162–1169. 3. Wirrell EC, Grossardt BR, So EL, et al. A population-based study of long-term outcomes of cryptogenic focal epilepsy in childhood: cryptogenic epilepsy is probably not symptomatic epilepsy. Epilepsia 2011;52:738–745. 4. Berg AT, Testa FM, Levy SR. Complete remission in nonsyndromic childhood-onset epilepsy. Ann Neurol 2011;70:566–573. 5. Camfield CS, Camfield PR. Adult outcome childhood onset, cause unknown (cryptogenic) MRI-negative, focal epilepsy. In Arts W, Arzimanoglou A, Brouwer OF, Camfield C, Camfield P (Eds) Progress in epileptic disorders; Vol 12: outcome of childhood epilepsies. Montrouge, France: John Libbey Eurotext, 2013:173– 179. 6. Berg AT, Vickrey VG, Testa TM, et al. How long does it take for epilepsy to become intractable? A prospective investigation. Ann Neurol 2006;60:73–79. 7. Wirrell EC, Camfield CS, Camfield PR, et al. Long-term psychosocial outcome in typical absence epilepsy. Sometimes a wolf in sheeps’ clothing. Arch Pediatr Adolesc Med 1997;151:152–158. 8. Camfield CS, Camfield PR. Juvenile myoclonic epilepsy 25 years after seizure onset: a population-based study. Neurology 2009;73:1041–1045. 9. Fastenau PS, Johnson CS, Perkins SM, et al. Neuropsychological status at seizure onset in children: risk factors for yearly cognitive deficits. Neurology 2009;18:526–534. 10. Speechley KN, Ferro MA, Camfield CS, et al. Quality of life in children with new-onset epilepsy. Neurology 2012;79:1548–1555. 11. Berg AT, Vickrey BG, Testa FM, et al. Behavior and social competency in children with idiopathic and cryptogenic epilepsy. Dev Med Child Neurol 2007;49:487–489. 12. Wirrell EC, Camfield CS, Camfield PR, et al. Long-term prognosis of typical childhood absence epilepsy. Neurology 1996;47:912–918. 13. Trinka E, Baumgartner S, Unterberger I, et al. Long-term prognosis for childhood and juvenile absence epilepsy. J Neurol 2004;251:1235– 1241. Epilepsia, 55(Suppl.3):16–20, 2014 doi: 10.1111/epi.12706
20 C. S. Camfield et al. 14. Caplan R, Siddarth P, Stahl L, et al. Childhood absence epilepsy: behavioral, cognitive, and linguistic comorbidities. Epilepsia 2008;19:1838–1846. 15. Muhle H, Mefford HC, Obermeier T, et al. Absence seizures with intellectual disability as a phenotype of the 15q13.3 microdeletion syndrome. Epilepsia 2011;52:e194–e198. 16. Kasteleijn-Nolst Trenite DG, Schmitz B, Janz D, et al. Consensus on diagnosis and management of JME: from founder’s observations to current trends. Epilepsy Behav 2013;28(Suppl. 1): S87–S90.
Epilepsia, 55(Suppl.3):16–20, 2014 doi: 10.1111/epi.12706
17. Geithner J, Schneider F, Wang Z, et al. Predictors for long-term seizure outcome in juvenile myoclonic epilepsy: 25–63 years of follow-up. Epilepsia 2012;53:1379–1386. 18. Baykan B, Altindag EA, Bebek N, et al. Myoclonic seizures subside in the fourth decade in juvenile myoclonic epilepsy. Neurology 2008;70:2123–2129. 19. Berg AT, Nickels K, Wirrell EC, et al. Mortality risks in new-onset childhood epilepsy. Pediatrics 2013;132:124–131. 20. Sillanp€a€a M, Jalava M, Kaleva O, et al. Long-term prognosis of seizures with onset in childhood. N Engl J Med 1998;338:1715–1722.
Transition issues for benign epilepsy with centrotemporal spikes, nonlesional focal epilepsy in otherwise normal children, childhood absence epilepsy, and juvenile myoclonic epilepsy *†Carol S. Camfield, ‡Anne Berg, §Ulrich Stephani, and ¶Elaine C. Wirrell Epilepsia, 55(Suppl. 3):16–20, 2014 doi: 10.1111/epi.12706
SUMMARY
Dr. Carol Camfield is professor emeritus at Dalhousie University.
This chapter covers the syndromes of benign epilepsy with centrotemporal spikes (BECTS), nonlesional focal epilepsy in otherwise normal children (NLFN), and the genetic generalized epilepsies. BECTS is an epilepsy syndrome that always enters terminal remission before the general age of a planned transition of adolescents. This is also the case for the majority (65%) of those with childhood absence epilepsy (CAE). Approximately 15% of patients with CAE who initially remit during their childhood years later develop juvenile myoclonic epilepsy (JME) as teenagers. They will have many issues for continuing medical care and transition, because their seizure disorder generally persists into adulthood. A significant minority of NLFN (~35%) and most patients with JME continue to have active epilepsy into adulthood. In addition, CAE, JME, and NLFN patients are at risk of a number of significant adverse social outcomes that require ongoing advice and counseling. KEY WORDS: Benign epilepsy with centrotemporal spikes, Nonlesional focal epilepsy, Childhood absence epilepsy, Juvenile myoclonic epilepsy, Transition, Adulthood, Adolescence.
Benign Epilepsy with Centrotemporal Spikes (BECTS) BECTS is characterized primarily by nocturnal seizures at sleep onset or just before awakening. In 75% of cases the disorder begins between 7 and 10 years of age. During the active phase, cognitive, linguistic, and behavioral difficulties of minimal to moderate intensity may occur. In all
cases, medical, educational (if needed), and psychological advice (if needed) with support to patients and families is important and should regularly be reviewed during childhood. Less than 2% of patients have a more severe course associated with treatment difficulties prior to terminal remission, at times with a rare evolution to an epileptic encephalopathy. Remission usually occurs within 3–4 years of onset and almost always before the age of 16 years.1 This excellent outcome is independent of any form of antiepileptic treatment. In addition, a recent population-based study found that 30 years after initial diagnosis of BECTS, all patients were in complete remission and continued off all antiepileptic medications.2 The social outcomes of education, employment, poverty, psychiatric disorders, or loneliness
Accepted May 30, 2014. *Department of Pediatrics, Dalhousie University, Halifax, Nova Scotia, Canada; †Department of Pediatrics, IWK Health Centre, Halifax, Nova Scotia, Canada; ‡Epilepsy Center, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, Illinois, U.S.A.; §Neuropediatric Department, University of Kiel, Kiel, Germany; and ¶Department of Neurology, Child and Adolescent Neurology, Mayo Clinic, Rochester, Minnesota, U.S.A. Address correspondence to Carol S. Camfield, Department of Pediatrics, Dalhousie University and the IWK Health Centre, University Ave., Halifax, NS, Canada B3K 6R8. E-mail: [email protected] Wiley Periodicals, Inc. © 2014 International League Against Epilepsy
16
17 Transition: Generalized and Benign Focal Epilepsies
Key Points BECTS will enter terminal remission by the age of 16 years and the social outcome in adulthood reflects that of the general population. No transition plans need to be made. CAE and NLFN are outgrown by late adolescence in 65% and persist for the remainder. JME usually persists during adolescence. In many with CAE, NLFN, and JME long-lasting care and/or psychosocial assistance is required. and isolation, marriage, and divorce were similar to in the general population. Although occasional cognitive difficulties were apparent during the active phase of their epilepsy in childhood, these did not appear to have significant longlasting effects. In general, neurologists or psychiatrists do not follow or treat young adults with BECTS for any epilepsy-related social or medical issues.
Nonlesional Focal Epilepsy in Otherwise Normal Children (NLFN) This epilepsy group presents with focal seizures (electroencephalography [EEG] and semiology) and normal clinical anatomic imaging, neurologic examination, and intellectual ability. Young people with NLFN represent 20–30% of children with epilepsy.3–5 After long-term follow-up (>10 years after the initial diagnosis), two thirds will enter terminal remission (TR), defined as having at least 5 years seizurefree and off antiepileptic drugs (AEDs)3–5 (also called “complete remission”) (Table 1). Although there is always the potential for subsequent relapse, the risk of relapse is low once TR been achieved: 10 year follow-up
19% with >10 year follow-up
– 67 33
110/131 (84%) 67 33
7
~13
12
Wirrell et al., Berg et al., Camfield and Camfield.
5
Epilepsia, 55(Suppl.3):16–20, 2014 doi: 10.1111/epi.12706
18 C. S. Camfield et al. remaining one third, seizures persist either as absences alone (10–15%), absences with either generalized tonic– clonic or myoclonic seizure (5–15%), or evolution to JME (5–15%). In considering a diagnosis of CAE, several, less common forms of absence epilepsy that are often therapy resistant must be excluded. These include myoclonic absence epilepsy, epilepsy with eyelid myoclonia (Jeavon’s syndrome), perioral myoclonia with absence, as well as absences associated with specific metabolic (glucose transporter deficiency) or chromosomal (ring chromosome 20, microdeletion 15q13.3) anomalies. Cognitive concerns are reported in several large case series14 and include subtle cognitive deficits and linguistic difficulties, which are frequently complicated by ADHD or associated with affective/anxiety disorders. There appears to be a greater risk with longer illness duration and higher seizure frequency. In addition, certain genetic abnormalities portend a greater risk of intellectual disability.15 The social outcome of children from the Nova Scotia Childhood Epilepsy Study with CAE, who were >18 years of age at time of study (n = 56) were compared to young adults with an underlying chronic disorder, juvenile rheumatoid arthritis (n = 61).7 Surprisingly, those with prior CAE were more likely to require special educational help (p < 0.02), have below average academic performance (p < 0.01) or repeat a grade (p < 0.005), become inadvertently pregnant (p < 0.001), and less likely to graduate from high school (p < 0.005) or attend college/university (p < 0.001). No studies have yet followed children with CAE into adulthood to evaluate social success.
Juvenile Myoclonic Epilepsy (JME) JME usually begins in adolescence between 12 and 18 years, although it may occur earlier in childhood or even in the third or later decades.16 Approximately 5–15% were previously diagnosed with CAE, experienced TR (seizurefree and >5 years off AED treatment), and then present with recurrent seizure types that met the criteria for JME. JME has been considered for many years to be a lifelong disorder with a high recurrence rate after withdrawal of AEDs.16 Two large case series document that up to 25% can discontinue AEDs entirely and remain seizure-free.17,18 In addition, myoclonic seizures often may decrease or disappear in the fourth decade. However, a pharmacoresistant course is observed in ~15%. A population-based study reported the medical and social outcomes of JME patients (n = 23) with at least 25 years follow-up after initial diagnosis (average age of 36 years).8 At the end of the followup, 13% (3/23) had intractable epilepsy, whereas 26% (6/23) were seizure free and off AED treatment (mean 17 years). In addition, 13% (3/23) experienced only mild myoclonic seizures, which persisted for up to 22 years after stopping AED treatment. The myoclonus was not considEpilepsia, 55(Suppl.3):16–20, 2014 doi: 10.1111/epi.12706
ered problematic by these adults. In summary, 39% of the cohort eventually stopped AEDs and became seizure free or experienced only mild myoclonic seizures. Although most people with JME have a lifelong disorder, this study indicates that there are some with a relatively short active phase (4–5 years) followed by a long-term remission with no requirement for continued treatment. It seems reasonable to consider a trial of stopping AED treatment in late adolescence or early adulthood for people with JME who are well controlled, depending on life issues such as driving. It is possible that the relatively optimistic rate of remission is related to age, inasmuch as seizure-provoking factors such as binge drinking and sleep deprivation are likely to decrease over time.
Suggestions at the Time of the Initial Transition Meeting Although the adolescent with normal intelligence and neurologic examination without neuroimaging abnormalities has an already established diagnosis and management plan, the time of transition is an opportunity for reassessment. Issues to be considered or reexamined are listed in subsequent text; however, there is little evidence to document effectiveness of this approach (Table 2). 1 Is the diagnosis correct? Is the present control of seizures adequate? Is the current AED choice and dosage appropriate? 2 Are side effects present? Are there any possible interactions with oral contraceptives, alcohol, or other drugs, and have they been discussed with the adolescent or young adult? Many children with epilepsy are diagnosed in their early childhood years and may have never personally received counseling regarding potential adverse effects of their medication. Furthermore, in adolescence and early adulthood, certain potential side effects may be
Table 2. Suggestions for discussion at the time of transition of adolescents with normal anatomic imaging, neurologic examination, and intellectual ability Education about epilepsy, treatments, and their side effects Ensure appropriate counseling regarding substance abuse, contraception, and sexual health and sleep hygiene has taken place Evaluate the adolescent’s psychological and social well-being Assess the home or living environment How cohesive, supportive and appropriate is it? How long will the adolescent remain in this setting and what is planned in the future? Discuss the learning environment and its appropriateness to the teen’s cognitive ability. Is neuropsychological testing needed? Is comorbid ADHD present and appropriately treated? Are vocational and higher education plans and goals reasonable? When appropriate, sensitively discuss the risk of death associated with SUDEP
19 Transition: Generalized and Benign Focal Epilepsies more concerning, in particular the risk of polycystic ovarian syndrome and teratogenicity with valproate, slowness of verbal processing with topiramate or zonisamide, potential interaction of AEDs with oral contraceptives, effect of AEDs on bone health and body mass index, and potential interactions with alcohol or other drugs. 3 Although not specifically linked to sudden unexplained death in epilepsy (SUDEP), the discussion of the potential risk of SUDEP in young people sometimes arises. For both CAE and NLFN-TR, the risk of death to the individual is comparable to the leading causes of death in the population, including accidents, homicide, suicide, cancer, and heart disease.19 The JME group with persistent, frequent generalized tonic–clonic seizures, however, represents those at particular risk of this devastating outcome. For these individuals, a serious but sensitive discussion of the risks and possible suggestions for decreasing the risk of SUDEP should take place with not only the parents, but also the adolescent. 4 A successful process requires both a well-thought out process and plan, which allows the teenager to assume greater self-empowerment and independence, and a team of professionals, which may include physicians, nurses, social workers, and psychologists/psychiatrists. Topics for discussion should cover: a. Education about epilepsy and treatments and their side effects. b. Discussion of the learning environment and its appropriateness to the teen’s cognitive ability. Children and teens with epilepsy have higher rates of subtle cognitive deficits and ADHD, which can affect academic success. One must assess whether the teen’s learning environment is appropriate for their cognitive level, and whether neuropsychological testing is needed. In addition, comorbid ADHD may require treatment. Young adults with epilepsy are less likely to be employed in their field of study and often have job dissatisfaction. 20 Thus, future goals and vocational counseling as well as plans for higher education should be discussed. c. Evaluation of the adolescent’s psychological and social well-being is needed. Affective disorders including anxiety and depression are known to affect a significant minority of young adults with epilepsy and often remain untreated. Screening for such a disorder should be performed at the time of transition and involve psychiatry/social work, as needed. d. Evaluation of the present home environment. Is it adequate, cohesive, and able to be supportive? How long will the adolescent remain in this setting and what is planned for the future? e. Ensure appropriate counseling regarding substance abuse, contraception and family planning, genetic counseling, sexual health, and sleep hygiene.
Addressing each of these areas is difficult in a single session and is often overwhelming for both the adolescent and family. It may be helpful to have several meetings with a multidisciplinary team to be certain that all of the correct information is obtained by the Transition Team and understood by the teen and his/her family.
Disclosure or Conflict of Interest Carol Camfield, Peter Camfield, and Elaine Wirrell have no disclosures. Anne Berg has received speaker honoraria and travel support from BIAL and Medical University of South Carolina, travel support from the International League Against Epilepsy (ILAE), and an honorarium from The American Academy of Neurology, Continuum. She serves on advisory boards for Citizens United for Research in Epilepsy (CURE) and Eisai, serves on the editorial boards of Epilepsy & Behavior and Neurology, and is supported by funding from the National Institute of Neurological Disorders & Stroke (NINDS) (Grant R37-NS31146) and the Pediatric Epilepsy Research Foundation. Ulrich Stephani serves on advisory boards of the pharmaceutical companies Desitin, Eisai, and Viropharma and receives support from Deutsche Forschungsgemeinschaft DFG (German Research society) and the German chapter of the ILAE. We confirm that we have read the Journal’s position on issues involved in ethical publication and affirm that this article is consistent with those guidelines.
References 1. Bouma PA, Bovenkerk AC, Westendorp RG, et al. The course of benign partial epilepsy of childhood with centrotemporal spikes: a meta-analysis. Neurology 1997;48:430–437. 2. Camfield CS, Camfield PR. Rolandic epilepsy has little effect on adult life 30 years later: a population-based study. Neurology 2014;82:1162–1169. 3. Wirrell EC, Grossardt BR, So EL, et al. A population-based study of long-term outcomes of cryptogenic focal epilepsy in childhood: cryptogenic epilepsy is probably not symptomatic epilepsy. Epilepsia 2011;52:738–745. 4. Berg AT, Testa FM, Levy SR. Complete remission in nonsyndromic childhood-onset epilepsy. Ann Neurol 2011;70:566–573. 5. Camfield CS, Camfield PR. Adult outcome childhood onset, cause unknown (cryptogenic) MRI-negative, focal epilepsy. In Arts W, Arzimanoglou A, Brouwer OF, Camfield C, Camfield P (Eds) Progress in epileptic disorders; Vol 12: outcome of childhood epilepsies. Montrouge, France: John Libbey Eurotext, 2013:173– 179. 6. Berg AT, Vickrey VG, Testa TM, et al. How long does it take for epilepsy to become intractable? A prospective investigation. Ann Neurol 2006;60:73–79. 7. Wirrell EC, Camfield CS, Camfield PR, et al. Long-term psychosocial outcome in typical absence epilepsy. Sometimes a wolf in sheeps’ clothing. Arch Pediatr Adolesc Med 1997;151:152–158. 8. Camfield CS, Camfield PR. Juvenile myoclonic epilepsy 25 years after seizure onset: a population-based study. Neurology 2009;73:1041–1045. 9. Fastenau PS, Johnson CS, Perkins SM, et al. Neuropsychological status at seizure onset in children: risk factors for yearly cognitive deficits. Neurology 2009;18:526–534. 10. Speechley KN, Ferro MA, Camfield CS, et al. Quality of life in children with new-onset epilepsy. Neurology 2012;79:1548–1555. 11. Berg AT, Vickrey BG, Testa FM, et al. Behavior and social competency in children with idiopathic and cryptogenic epilepsy. Dev Med Child Neurol 2007;49:487–489. 12. Wirrell EC, Camfield CS, Camfield PR, et al. Long-term prognosis of typical childhood absence epilepsy. Neurology 1996;47:912–918. 13. Trinka E, Baumgartner S, Unterberger I, et al. Long-term prognosis for childhood and juvenile absence epilepsy. J Neurol 2004;251:1235– 1241. Epilepsia, 55(Suppl.3):16–20, 2014 doi: 10.1111/epi.12706
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