epilepsy
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Introduction
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:
- benign childhood epilepsy
- juvenile myoclonic epilepsy
- idiopathic generalized epilepsy
- focal epilepsy, epilepsia partialis continuans
- temporal lobe epilepsy (most common syndrome in adults)
- post-traumatic epilepsy
- 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
- 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
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
Diagnostic procedures
- electroencephalogram (EEG)
- 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]
- 11 year mortality ~ 0.9%[9]
- increased risk of major cardiovascular events (RR=1.6)[25][26]
- disease interaction(s) of ADHD with epilepsy & febrile seizures
- disease interaction(s) of epilepsy with cardiovascular events
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]
- 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
More general terms
More specific terms
- 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
Additional terms
References
- ↑ Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 1034-35
- ↑ 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 2.13 2.14 2.15 2.16 2.17 2.18 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 - ↑ 3.0 3.1 ARUP Consult: Seizure Disorders - Epilepsy deprecated reference
- ↑ French JA, Pedley TA. Clinical practice. Initial management of epilepsy. N Engl J Med. 2008 Jul 10;359(2):166-76 PMID: https://www.ncbi.nlm.nih.gov/pubmed/18614784
- ↑ Kwan P, Brodie MJ. Early identification of refractory epilepsy. N Engl J Med. 2000 Feb 3;342(5):314-9. PMID: https://www.ncbi.nlm.nih.gov/pubmed/10660394
- ↑ 6.0 6.1 Nei M, Hays R. Sudden unexpected death in epilepsy. Curr Neurol Neurosci Rep. 2010 Jul;10(4):319-26 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20446062
- ↑ Arain AM, Abou-Khalil BW. Management of new-onset epilepsy in the elderly. Nat Rev Neurol. 2009 Jul;5(7):363-71 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19578343
- ↑ 8.0 8.1 8.2 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 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/24730690 <Internet> http://onlinelibrary.wiley.com/doi/10.1111/epi.12550/full - ↑ 9.0 9.1 9.2 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
- ↑ Gaffield ME, Culwell KR, Lee CR. The use of hormonal contraception among women taking anticonvulsant therapy. Contraception. 2011 Jan;83(1):16-29 PMID: https://www.ncbi.nlm.nih.gov/pubmed/21134499
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/23350722
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/22895589
- ↑ Maschio M, Dinapoli L. Patients with brain tumor-related epilepsy. J Neurooncol. 2012 Aug;109(1):1-6 PMID: https://www.ncbi.nlm.nih.gov/pubmed/22528794
- ↑ 14.0 14.1 14.2 14.3 14.4 Kao A Medscape: Quiz: January 12, 2016 Test Your Knowledge of Epilepsy and Seizure-related Conditions. http://reference.medscape.com/viewarticle/856770_2
- ↑ Ko DY, Benbadis SR Medscape: Epilepsy and Seizures. http://emedicine.medscape.com/article/1184846-overview
- ↑ 16.0 16.1 16.2 Scheffer IE et al ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology. Epilepsia. March 8, 2017 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/28276062 <Internet> 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 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/28276064 <Internet> 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 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/28276060 <Internet> http://onlinelibrary.wiley.com/doi/10.1111/epi.13670/full
Zuberi SM, Perucca E. A new classification is born. Epilepsia. March 8, 2017 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/28276059 <Internet> http://onlinelibrary.wiley.com/doi/10.1111/epi.13694/full - ↑ 17.0 17.1 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. - ↑ 18.0 18.1 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
- ↑ 19.0 19.1 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: https://www.ncbi.nlm.nih.gov/pubmed/29444968 - ↑ 20.0 20.1 20.2 Gorton HC, Webb RT, Carr MJ et al Risk of Unnatural Mortality in People With Epilepsy. JAMA Neurol. Published online April 9, 2018. PMID: https://www.ncbi.nlm.nih.gov/pubmed/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: https://www.ncbi.nlm.nih.gov/pubmed/29630704 https://jamanetwork.com/journals/jamaneurology/fullarticle/2677846 - ↑ 21.0 21.1 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
- ↑ 22.0 22.1 22.2 22.3 22.4 22.5 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: https://www.ncbi.nlm.nih.gov/pubmed/30039175 https://jamanetwork.com/journals/jamaneurology/fullarticle/2688301
- ↑ 23.0 23.1 23.2 23.3 23.4 23.5 Choogin Wisely. Five Things Physicians and Patients Should Question. American Epilepsy Society. Aug 15, 2018 http://www.choosingwisely.org/societies/american-epilepsy-society/
- ↑ Dobrin S. Seizures and epilepsy in adolescents and adults. Dis Mon. 2012 Dec;58(12):708-29. PMID: https://www.ncbi.nlm.nih.gov/pubmed/23149523
- ↑ 25.0 25.1 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: https://www.ncbi.nlm.nih.gov/pubmed/34046890 https://onlinelibrary.wiley.com/doi/10.1111/epi.16930 - ↑ 26.0 26.1 Li J, Shlobin NA, Thijs RD et al Antiseizure Medications and Cardiovascular Events in Older People With Epilepsy. JAMA Neurol. 2024 Sep 30. PMID: https://www.ncbi.nlm.nih.gov/pubmed/39348143 https://jamanetwork.com/journals/jamaneurology/fullarticle/2824203
- ↑ The Human Epilepsy Project http://www.humanepilepsyproject.org/
- ↑ NINDS Epilepsy Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Epilepsy-Information-Page
- ↑ Centers of Disease Control and Prevention (CDC) Epilepsy http://www.cdc.gov/epilepsy/index.html
- ↑ Epilepsy Foundation: State Driving Laws Database http://www.epilepsy.com/driving-laws