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epilepsy

A tendency towards recurrent seizures. Defined as >= 2 unprovoked seizures > 24 hours apart or 1 unprovoked seizure with a risk of further seizures of >= 60% [2] Classification: Epilepsy syndromes: 1) benign childhood epilepsy 2) juvenile myoclonic epilepsy 3) idiopathic generalized epilepsy 4) focal epilepsy, epilepsia partialis continuans 5) temporal lobe epilepsy (most common syndrome in adults) 6) post-traumatic epilepsy 7) febrile seizures * International League Against Epilepsy has updated its system for classifying epilepsies [16] - focal is now used instead of partial - focal seizures are now classified by awareness - the terms dyscognitive, simple partial, complex partial, psychic, & secondarily generalized are no longer used [16] Etiology: - inherited syndromes - congenital brain malformations - focal cortical dysplasia - infection - head trauma (most common cause in children)* [14] - brain tumors - stroke (most common in elderly) [14,22] - neurodegenerative disease, dementia [14,22] - risk factors for late-life epilepsy [22] - hypertension - diabetes mellitus - highest risk: black patients with diabetes - smoking - apoE4 allele [22] - reduced risk associated with - physical activity is a negative risk factor - moderate alcohol consumption is a negative risk factor [22] * no mention of febrile seizures [14] Epidemiology: - cumulative lifetime incidence in U.S. is 3% [2] - 44% of patients with epilepsy report seizures under control [21] Clinical manifestations: - risk of recurrent seizures is > 60% [2] Diagnostic criteria: - diagnosis of epilepsy requires 2 or more unprovoked seizures 24 hours apart [2,8], or - one unprovoked seizure plus >= 60% likelihood of additional seizure(s) within 10 years (a single seizure 1 month after a stroke), or - presence of an epilepsy syndrome * resolution of epilepsy may be considered if - a patient has outgrown an age-dependent epilepsy syndrome - 10 years without a seizure & off anticonvulsants for 5 years [8] Laboratory: - avoid routine drug levels in patients with epilepsy [23] - see ARUP consult [3] Special laboratory: - electroencephalogram (EEG) - do not routinely order EEG as initial part of syncope workup [23] - negative results do not exclude epilepsy [2] - electroencephalography is 40-50% sensitive in diagnosing epilepsy [2] - video EEG monitoring should be considered - patients not responding to 2 or more anticonvulsants - seizures requiring further characterization [2] - gold standard for classifying type of epilepsy Radiology: - magnetic resonance imaging - negative results do not exclude epilepsy [2] - do not routinely obtain neuroimaging after an acute seizure in a patient with established epilepsy [23] Complications: - major depression - bipolar disorder - cognitive impairment - increased risk of bone fractures - increased risk of sudden, unexplained death [2,6,9] - 11 year mortality ~ 0.9% [9] - up to 1% with intractable seizures, multiple anticonvulsants [2] - 3-fold risk of unnatural mortality [20] - 5-fold risk of unintentional medication overdose - opioid & psychotropic major implicated drugs - anticonvulsants not implicated in overdoses [20] - increased risk of major cardiovascular events (RR=1.6) [25,26] Management: - see specific form of seizure - anticonvulsant therapy - avoid valproic acid in women of child-bearing age [23] - levetiracetam or lamotrigine* - anticonvulsants of choice in women of child-bearing age - discovery of pregnancy is not reason enough to stop anticonvulsant [2] - uncontrolled seizure can result in fetal anoxia & death [2] - no significant interactions with hormonal contraceptives [2] - dosage reduction in patients with renal failure [2] - levetiracetam, lamotrigine & gabapentin are anticonvulsants of choice in older patients [2] - do not prescribe long-term anticonvulsant therapy to patients with withdrawal seizures [23] - patients who do not respond to combination of 2 anticonvulsants are considered refractory [2] - patients with refractory epilepsy should be referred to an epilepsy center for evaluation [2] - avoid drugs the lower seizure threshold - among antibiotics Zosyn with lower risk of triggering seizures than levofloxacin, cefepime, imipenem - medical marijuana may improve seizure control & quality of life in refractory epilepsy [17] - high out-of-pocket costs & inconvenient access cited as reasons for discontinuation - behavioral interventions can reduce seizures in patients with refractory epilepsy [19] - muscle relaxation with diaphragmatic breathing (29%) - control focused-attention activity with extremity movements (25%) - neurosurgery to remove epileptic focus for patients with refractory focal seizures [2] - laser ablation when location of seizure activity can be pinpointed is FDA-approved but not approved by Aetna [18] * avoid valproic acid, phenobarbital, phenytoin, topiramate, carbamazepine in pregnant women & women of child-bearing age

Interactions

disease interactions

Related

hormonal effects on epilepsy

Specific

Amish infantile epilepsy syndrome benign familial infantile convulsions benign familial neonatal epilepsy childhood absence epilepsy epilepsy during pregnancy epilepsy female-restricted with mental retardation (convulsive disorder & mental retardation) epilepsy X-linked with variable learning disabilities & behavior disorders (XELBD) epileptic encephalopathy early infantile type 2; atypical CDKL5-related Rett syndrome focal epilepsy generalized epilepsy & paroxysmal dyskinesia generalized epilepsy with febrile seizures (GEFS) idiopathic generalized epilepsy infantile epileptic encephalopathy intractable childhood epilepsy with generalized tonic-clonic seizures (ICEGTC) myoclonic epilepsy myoclonic epilepsy with ragged-red fibers (MERRF) syndrome nocturnal epilepsy pyridoxine-dependent epilepsy; neonatal epileptic encephalopathy; pyridoxine-5'-phosphate oxidase deficiency; PNPO deficiency status epilepticus

General

chronic neurologic disease seizure; epileptic seizure

References

  1. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 1034-35
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2012, 2015, 2018, 2021. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  3. ARUP Consult: Seizure Disorders - Epilepsy deprecated reference
  4. French JA, Pedley TA. Clinical practice. Initial management of epilepsy. N Engl J Med. 2008 Jul 10;359(2):166-76 PMID: 18614784
  5. Kwan P, Brodie MJ. Early identification of refractory epilepsy. N Engl J Med. 2000 Feb 3;342(5):314-9. PMID: 10660394
  6. Nei M, Hays R. Sudden unexpected death in epilepsy. Curr Neurol Neurosci Rep. 2010 Jul;10(4):319-26 PMID: 20446062
  7. Arain AM, Abou-Khalil BW. Management of new-onset epilepsy in the elderly. Nat Rev Neurol. 2009 Jul;5(7):363-71 PMID: 19578343
  8. Herman AO Epilepsy Case Definition Revised Physician's First Watch, April 15, 2014 David G. Fairchild, MD, MPH, Editor-in-Chief Massachusetts Medical Society http://www.jwatch.org - Fisher RS et al A practical clinical definition of epilepsy. Epilepsia. April 14, 2014 PMID: 24730690 http://onlinelibrary.wiley.com/doi/10.1111/epi.12550/full
  9. Selassie AW et al Premature Deaths Among Children with Epilepsy - South Carolina, 2000-2011 MMWR Weekly November 7, 2014 / 63(44);989-994 http://www.cdc.gov/mmwr/preview/mmwrhtml/mm6344a2.htm
  10. Gaffield ME, Culwell KR, Lee CR. The use of hormonal contraception among women taking anticonvulsant therapy. Contraception. 2011 Jan;83(1):16-29 PMID: 21134499
  11. Glauser T, Ben-Menachem E, Bourgeois B et al Updated ILAE evidence review of antiepileptic drug efficacy and effectiveness as initial monotherapy for epileptic seizures and syndromes. Epilepsia. 2013 Mar;54(3):551-63. PMID: 23350722
  12. Jette N, Quan H, Tellez-Zenteno JF et al Development of an online tool to determine appropriateness for an epilepsy surgery evaluation. Neurology. 2012 Sep 11;79(11):1084-93. Epub 2012 Aug 15. PMID: 22895589
  13. Maschio M, Dinapoli L. Patients with brain tumor-related epilepsy. J Neurooncol. 2012 Aug;109(1):1-6 PMID: 22528794
  14. Kao A Medscape: Quiz: January 12, 2016 Test Your Knowledge of Epilepsy and Seizure-related Conditions. http://reference.medscape.com/viewarticle/856770_2
  15. Ko DY, Benbadis SR Medscape: Epilepsy and Seizures. http://emedicine.medscape.com/article/1184846-overview
  16. Scheffer IE et al ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology. Epilepsia. March 8, 2017 PMID: 28276062 http://onlinelibrary.wiley.com/doi/10.1111/epi.13709/full - Fisher RS et al Instruction manual for the ILAE 2017 operational classification of seizure types. Epilepsia. March 8, 2017 PMID: 28276064 http://onlinelibrary.wiley.com/doi/10.1111/epi.13671/full - Fisher RS et al Operational classification of seizure types by the International League Against Epilepsy: Position Paper of the ILAE Commission for Classification and Terminology. Epilepsia. March 8, 2017 PMID: 28276060 http://onlinelibrary.wiley.com/doi/10.1111/epi.13670/full - Zuberi SM, Perucca E. A new classification is born. Epilepsia. March 8, 2017 PMID: 28276059 http://onlinelibrary.wiley.com/doi/10.1111/epi.13694/full
  17. Basen R. Epilepsy Patients Failing Regular Meds Improved with Medical Cannabis - Reported better overall health as well as seizure control in pilot study MedPage Today, Dec 05, 2017 - Papalia A, et al Has the New York Medical Marijuana Program benefited medically refractory epilepsy patients? American Epilepsy Society (AES) 2017; Abstract 2.186.
  18. Drash W Girl has words for Aetna after brain surgery was denied. CNN. Dec 11, 2017 https://www.msn.com/en-us/news/us/girl-has-words-for-aetna-after-brain-surgery-was-denied/ar-BBGzd2w
  19. George J. Stress-Reducing Techniques Lead to Fewer Seizures. Muscle relaxation, focused attention help patients with medication-resistant epilepsy. MedPage Today. Feb 15, 2018 https://www.medpagetoday.com/neurology/seizures/71192 - Haut SR, Lipton RB, Cornes S et al Behavioral interventions as a treatment for epilepsy: A multicenter randomized controlled trial. Neurology. 2018 Feb 14 PMID: 29444968
  20. Gorton HC, Webb RT, Carr MJ et al Risk of Unnatural Mortality in People With Epilepsy. JAMA Neurol. Published online April 9, 2018. PMID: 29630689 https://jamanetwork.com/journals/jamaneurology/fullarticle/2677847 - Devinsky O, Singh A, Friedman D. Deaths in Epilepsy. What We Are Missing. JAMA Neurol. Published online April 9, 2018. PMID: 29630704 https://jamanetwork.com/journals/jamaneurology/fullarticle/2677846
  21. Tian N, Boring M, Kobau R, Zack MM, Croft JB. Active Epilepsy and Seizure Control in Adults - United States, 2013 and 2015. MMWR Morb Mortal Wkly Rep 2018;67:437-442 https://www.cdc.gov/mmwr/volumes/67/wr/mm6715a1.htm
  22. Johnson EL, Krauss GL, Lee AK et al Association Between Midlife Risk Factors and Late-Onset Epilepsy. Results From the Atherosclerosis Risk in Communities Study. JAMA Neurol. Published online July 23, 2018. PMID: 30039175 https://jamanetwork.com/journals/jamaneurology/fullarticle/2688301
  23. Choogin Wisely. Five Things Physicians and Patients Should Question. American Epilepsy Society. Aug 15, 2018 http://www.choosingwisely.org/societies/american-epilepsy-society/
  24. Dobrin S. Seizures and epilepsy in adolescents and adults. Dis Mon. 2012 Dec;58(12):708-29. PMID: 23149523
  25. Greb E More Evidence Epilepsy Tied to Risk for Major Cardiovascular Events. Medscape - Jul 07, 2021. https://www.medscape.com/viewarticle/954347 - Lee-Lane E, Torabi F, Lacey A et al Epilepsy, antiepileptic drugs, and the risk of major cardiovascular events. Epilepsia 2021. May 27 PMID: 34046890 https://onlinelibrary.wiley.com/doi/10.1111/epi.16930
  26. Li J, Shlobin NA, Thijs RD et al Antiseizure Medications and Cardiovascular Events in Older People With Epilepsy. JAMA Neurol. 2024 Sep 30. PMID: 39348143 https://jamanetwork.com/journals/jamaneurology/fullarticle/2824203
  27. The Human Epilepsy Project http://www.humanepilepsyproject.org/
  28. NINDS Epilepsy Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Epilepsy-Information-Page
  29. Centers of Disease Control and Prevention (CDC) Epilepsy http://www.cdc.gov/epilepsy/index.html
  30. Epilepsy Foundation: State Driving Laws Database http://www.epilepsy.com/driving-laws