Status epilepticus
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{{Infobox_Disease |
Name = {{PAGENAME}} |
Image = |
Caption = |
DiseasesDB = |
ICD10 = {{ICD10|G|41||g|40}} |
ICD9 = {{ICD9|345.3}} |
ICDO = |
OMIM = |
MedlinePlus = |
eMedicineSubj = emerg |
eMedicineTopic = 554 |
MeshID = D013226 |
}}
'''Status epilepticus''' (SE) refers to a life-threatening condition in which the [[brain]] is in a state of persistent [[seizure]]. Definitions vary, but traditionally it is defined as one continuous unremitting seizure lasting longer than 5-10 minutes, or recurrent seizures without regaining consciousness between seizures for greater than 30 minutes. There is some evidence that 5 minutes is sufficient to damage neurons and that seizures are unlikely to self-terminate by that time{{Fact|date=February 2008}}.
In known epileptics, this condition is associated with poor [[compliance (medicine)|compliance]] (adherence to medication regimen), [[alcohol]] withdrawal, and [[metabolism|metabolic]] disturbances. As a primary presentation it normally indicates a [[tumour]] or [[abscess]].
It can also be induced by [[nerve agent]]s such as [[soman]].<ref name="soman">{{cite journal | first = John H. | last = McDonough | coauthors = A. Benjamin, Joseph D. McMonagle, Thomas Rowland, Shih Tsung-Ming | month = February | year = 2004 | title = Effects of fosphenytoin on nerve agent-induced status epilepticus | journal = Drug and Chemical Toxicology | volume = 27 | issue = 1 | pages = 27–39 | pmid = 15038246 | url = http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&list_uids=15038246&dopt=ExternalLink | doi = 10.1081/DCT-120027895}}</ref>
==Variants==
Status epilepticus can be divided into two categories—convulsive and nonconvulsive, the latter of which is underdiagnosed.
===Convulsive===
[[Epilepsia partialis continua]] is a variant involving [[hour]], [[day]], or even [[week]]-long jerking. It is a consequence of [[vascular disease]], [[tumour]]s, or [[encephalitis]], and is drug-resistant.
Generalized [[myoclonus]] is commonly seen in [[coma]]tose patients following [[CPR]] and is seen by some as an indication of catastrophic damage to the [[neocortex]].<ref name="gen-myo-se">{{cite journal | first = Eelco F. M. | last = Wijdicks | coauthors = Parisi JE, Sharbrough FW | month = February | year = 1994 | title = Prognostic value of myoclonus status in comatose survivors of cardiac arrest | journal = Annals of Neurology | volume = 35 | issue = 2 | pages = 239–43 | pmid =8109907 | doi = 10.1002/ana.410350219}}</ref>
===Nonconvulsive===
[[Complex partial status epilepticus]], or CPSE, and [[absence status epilepticus]] are rare forms of the condition which are marked by nonconvulsive seizures. In the case of CPSE, the seizure is confined to a small area of the brain, normally the temporal lobe. But the latter, absence status epilepticus, is marked by a generalised seizure affecting the whole brain, and an [[Electroencephalogram|EEG]] is needed to differentiate between the two conditions. This results in episodes characterized by a long-lasting stupor, staring and unresponsiveness.
==Treatments==
===Benzodiazepines===
Shortly after it was introduced in 1963, [[diazepam]] became the first choice for SE. Even though other [[benzodiazepine]]s such as [[clonazepam]] were useful, diazepam was relied upon almost exclusively. This began to change in 1975 with a preliminary study on [[lorazepam]] conducted by
Waltregny and Dargent, who found that its pharmacological effects were longer lasting than those of an equal dose of diazepam.<ref name="waltdar">{{cite journal | first = Alain | last = Waltregny | coauthors = Jérôme Dargent | month = September/October | year = 1975 | title = Preliminary study of parenteral lorazepam in status epilepticus | journal = Acta Neurologica Belgica | volume = 75 | issue = 5 | pages = 219–29 | pmid = 3939}}</ref> This meant it did not have to be repeatedly injected like diazepam,<ref name="lorazepam1979">{{cite journal | first = JE | last = Walker | coauthors = RW Homan, MR Vasko, IL Crawford, RD Bell, WG Tasker | month = September | year = 1979 | title = Lorazepam in status epilepticus | journal = Annals of Neurology | volume = 6 | issue = 3 | pages = 207–13 | pmid = 43112 | doi = 10.1002/ana.410060305}}</ref> the effects of which would wear off 5–15 minutes later in spite of its 30-hour [[half-life]] (due to extensive redistribution of diazepam outside the vascular compartment as diazepam is highly lipid soluble). It has also been found that patients who were first tried on diazepam were much more likely to require [[tracheotomy|endotracheal tubing]] than patients who were first tried on [[phenobarbital]], [[phenytoin]],<ref name="vspbandpht">{{cite journal | first = Richard A. | last = Orr | coauthors = Robert J. Dimand, Shekhar T. Venkataraman, Valerie A. Karr, Kathleen J. Kennedy | month = September | year = 1991 | title = Diazepam and intubation in emergency treatment of seizures in children | journal = Annals of Emergency Medicine | volume = 20 | issue = 9 | pages = 1009–13 | pmid = 1877765 | doi = 10.1016/S0196-0644(05)82981-6 | url = http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6WB0-4G82KTB-G&_coverDate=09%2F30%2F1991&_alid=434858892&_rdoc=1&_fmt=&_orig=search&_qd=1&_cdi=6696&_sort=d&view=c&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=fab697f11c8e05b7fe0405b1d34a6f7d}}</ref> or lorazepam.<ref name="vslorazepam">{{cite journal | first = Richard | last = Appleton | coauthors = A. Sweeney, Imti Choonara, Joan Robson, Elizabeth Molyneux. | month = August | year = 1995 | title = Lorazepam versus diazepam in the acute treatment of epileptic seizures and status epilepticus | journal = Developmental Medicine and Child Neurology | volume = 37 | issue = 8 | pages = 682–8 | pmid = 7672465}}</ref>
Today, the benzodiazepine of choice is [[lorazepam]] for initial treatment due to its long (2–8 hour) duration of action and rapid onset of action, thought to be due to its high affinity for GABA receptors and to its low lipid solubility which causes it to remain in the vascular compartment. If lorazepam is not available, then diazepam should be given.<ref name="benzo-of-choice">{{cite journal | first = Trudy | last = Pang | coauthors = Lawrence J. Hirsch | month = July | year = 2005 | title = Treatment of Convulsive and Nonconvulsive Status Epilepticus | journal = Current Treatment Options in Neurology | volume = 7 | issue = 4 | pages = 247–259 | pmid = 15967088 | url = http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&list_uids=15967088&dopt=ExternalLink | doi = 10.1007/s11940-005-0035-x}}</ref> Sometimes, the failure of lorazepam alone is considered to be enough to classify a case of SE as [[Wiktionary:refractory|refractory]].
===Phenytoin and fosphenytoin===
Phenytoin was once another first-line therapy, although the [[prodrug]] [[fosphenytoin]] can be administered three times as fast and with far fewer injection site reactions. If these or any other [[hydantoin|hydantoin derivative]]s are used, then cardiac monitoring is a must if they are administered intravenously. Because the hydantoins take 15–30 minutes to work, a benzodiazepine or barbiturate is often co-administered. Because of diazepam's short duration of action, they were often administered together anyway.
===Barbiturates===
Before the benzodiazepines were invented, there were the barbiturates, which are still used today if benzodiazepines or the hydantoins are not an option. These are used to induce a [[barbiturate|barbituric]] coma. The barbiturate most commonly used for this is phenobarbital. [[Thiopental]] or [[pentobarbital]] may also be used for that purpose if the seizures have to be stopped immediately or if the patient has already been compromised by the underlying illness or toxic/metabolic-induced seizures; however, in those situations, thiopental is the agent of choice.
The failure of phenobarbital therapy does not preclude the success of a lengthy comatose state induced by a stronger barbiturate such as [[secobarbital]]. Such was the case for Ohori, Fujioka, and Ohta ca. 1998, when they induced a 10-month long coma (or "anesthesia" as they called it) in a 26-year-old woman suffering from refractory status epilepticus secondary to [[encephalitis|viral encephalitis]] and then tapered her off the secobarbital very slowly while using [[zonisamide]] at the same time.<ref name="secobarbitalofo">{{cite journal | first = Nobuhira | last = Ohori | coauthors = Fujioka Y, Ohta M. | month = May | year = 1998 | title = [Experience in managing refractory status epilepticus caused by viral encephalitis under long-term anesthesia with barbiturate: a case report] | journal = Rinsho Shinkeigaku | volume = 38 | issue = 5 | pages = 474–7 | pmid = 9806000}} (Japanese)</ref>
===General anesthetics===
If this proves ineffective or if barbiturates cannot be used for some reason, then a [[general anesthetic]] such as [[propofol]]<ref name="propofol">{{cite journal | first = X | last = Pourrat | coauthors = JM Serekian, D Antier, J. Grassin | title = [Generalized tonic-clonic status epilepticus: therapeutic strategy] | journal = Presse Médicale | volume = 30 | issue = | date = June 9, 2001 | pages = 1031–6 | pmid = 11433696}} (French).</ref> is tried; sometimes it is used second after the failure of [[lorazepam]].<ref name="propofol2">{{cite journal | first = Paul E. | last = Marik | coauthors = Joseph Varon | title = The management of status epilepticus | journal = Chest | year = 2004 | volume = 126 | issue = 2 | pages = 582–91 | pmid = 15302747 | url = http://www.chestjournal.org/cgi/content/full/126/2/582 | doi = 10.1378/chest.126.2.582}}</ref> This also means putting the patient on artificial respiration. Propofol has been shown to be effective in suppressing the jerks seen in myoclonus status epilepticus, but [[as of 2002]], there have been no cases of anyone going into myoclonus status epilepticus, undergoing propofol treatment, and then not dying anyway.<ref name="propofolmse">{{cite journal | first = Eelco F.M. | last = Wijdicks | year = 2002 | month = July | title = Propofol in myoclonus status epilepticus in comatose patients following cardiac resuscitation | journal = Journal of Neurology Neurosurgery and Psychiatry | volume = 73 | issue = 1 | pages = 94–5 | pmid = 12082068 | url = http://jnnp.bmjjournals.com/cgi/content/full/73/1/94 | doi = 10.1136/jnnp.73.1.94}}</ref>
===Lidocaine===
The use of [[lidocaine]] in status epilepticus was first reported in [[1955]] by Bernhard, Boem and Hojeberg.<ref name="lidocaine1955">{{cite journal | first = CG | last = Bernhard | coauthors = Bohm E, Hojeberg S | month = August | year = 1955 | title = A new treatment of status epilepticus; intravenous injections of a local anesthetic (lidocaine) | journal = AMA Archives of Neurology and Psychiatry | volume = 74 | issue = 2 | pmid = 14397899 | pages = 208–14}}</ref> Since then, it has been used in cases refractory to phenobarbital, diazepam, and phenytoin, and has been studied as an alternative to barbiturates and general anesthetics.<ref name="lidocainepro">{{cite journal | first = Praveen | last = Aggarwal | coauthors = Jyoti Prakash Wali | month = May | year = 1993 | title = Lidocaine in refractory status epilepticus: a forgotten drug in the emergency department | journal = American Journal of Emergency Medicine | volume = 11 | issue = 3 | pages = 243–4 | pmid = 93257009 | doi = 10.1016/0735-6757(93)90135-X | url = http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&list_uids=8489668&dopt=ExternalLink}}</ref><ref name="lidocainepro2">{{cite journal | first = N | last = Sugiyama | coauthors = Hamano S, Mochizuki M, Tanaka M, Eto Y | month = November | year = 2004 | title = [Efficacy of lidocaine on seizures by intravenous and intravenous-drip infusion] | journal = No To Hattatsu | volume = 36 | issue = 6 | pages = 451–4 | pmid = 15560386}} (Japanese)</ref> Lidocaine is a [[sodium channel]] blocker and has been used where sodium channel dysfunction was suspected.<ref name="sodium-channel-dysfunction">{{cite journal | author = Sawaishi Yukio | coauthors = Yano Tamami, Enoki Masamichi, and Takada Goro | month = February | year = 2002 | title = Lidocaine-dependent early infantile status epilepticus with highly suppressed EEG | journal = Epilepsia | volume = 43 | issue = 2 | pages = 201–4 | pmid = 11903470 | doi = 10.1046/j.1528-1157.2002.25301.x}}</ref> However, in some studies, it was either ineffective or even harmful for most patients.<ref name="lidocainecon">{{cite journal | author = Tanabe Takuya | coauthors = Suzuki Shuuhei, Shimakawa Shuichi, Yamashiro Kuniteru<!--The original kanji for "Kuniteru(?)" were 國暉. Please find the actual pronunciation if that is incorrect!-->, Tamai Hiroshi | month = January | year = 1999 | title = Problems of intravenous lidocaine treatment in status epilepticus or clustering seizures in childhood | journal = [http://www.meteo-intergate.com/journal/journal-archive_cl1nohat.html No To Hattatsu] | volume = 31 | issue = 1 | pages = 14–20 | pmid = 10025129}} (Japanese)</ref> The last is not so surprising in light of the fact that lidocaine has been known to cause seizures in humans and laboratory animals at doses greater than 15 µg/mL<ref name="pro-and-anticonvulsant">{{cite journal | first = John C. | last = DeToledo | month = June | year = 2000 | title = Lidocaine and Seizures | journal = Therapeutic Drug Monitoring | volume = 22 | issue = 3 | pages = 320–322 | pmid = 10850400 | doi = 10.1097/00007691-200006000-00014}}</ref> or 2–3 mg/kg.<ref name="pro-and-anticonvulsant2">{{cite web | author=Steven C. Schachter | title=Lidocaine | url=http://professionals.epilepsy.com/page/local_lidocaine.html | publisher=epilepsy.com/professionals}} Adapted from: {{cite book | author=Najjar S, Devinsky O, Rosenberg AD, et al | editor=ed. Ettinger AB and Devinsky O | title=Managing epilepsy and co-existing disorders | chapter=Procedures in epilepsy patients | year=2002 | pages=499–513 | publisher=Butterworth-Heinemann | location=Boston | isbn=0-7506-7241-2}}</ref>
== Notable Instances ==
On February 20, 2008, a fourteen year old girl who had been seeing a neurologist, but had not been diagnosed with epilepsy had a convulsive seizure at her school that lasted less than five minutes, and an ambulance was called because nobody present was able to properly handle the situation. She was transported to the hospital, but after an abnormal amount of time she still had not awakened. After EEG electrodes were attached to her head, it was discovered that she was suffering from status epilepticus. Her doctor went through the routine normally used to stop an episode of status epilepticus, but the seizure would not stop. In the end she had to be given such a large amount of anti-convulsive medications that her breathing system shut down and she went into kidney failure. The in-head seizure had lasted approximately four and a half hours. She went into a two day long coma, was put on a ventilator, and received treatments for her kidney failure. She was disoriented for her first five days in the hospital, and it was unknown how long it would take for her to recover, but it was estimated that it would take about a month and a half. Very surprisingly, she recovered in only 10 days and was able to attend school the day after she was released from the hospital. <ref>Tate, Elizabeth S. Personal interview. 4 Mar. 2008.</ref>
==References==
{{Reflist}}
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{{Diseases of the nervous system}}
==External links==
* [http://www.epilepsyfoundation.org/about/types/types/statusepilepticus.cfm Status Epilepticus Information from the Epilepsy Foundation]
* [http://www.epilepsyfoundation.org/about/types/ Information about Seizures and Syndromes: Epilepsy Foundation]
* [http://www.epilepsyfoundation.org/about/types/types/index.cfm Seizure Categorization: Epilepsy Foundation]
[[Category:Neurology]]
[[Category:Medical emergencies]]
[[Category:Epilepsy]]
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