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<ul id="toc-Signs_and_symptoms-sublist" class="vector-toc-list"> <li id="toc-Epileptic_spasms" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Epileptic_spasms"> <div class="vector-toc-text"> <span class="vector-toc-numb">1.1</span> <span>Epileptic spasms</span> </div> </a> <ul id="toc-Epileptic_spasms-sublist" class="vector-toc-list"> </ul> </li> <li id="toc-Developmental" class="vector-toc-list-item vector-toc-level-2"> <a class="vector-toc-link" href="#Developmental"> <div class="vector-toc-text"> <span class="vector-toc-numb">1.2</span> <span>Developmental</span> </div> </a> <ul id="toc-Developmental-sublist" class="vector-toc-list"> </ul> </li> </ul> </li> <li id="toc-Causes" class="vector-toc-list-item vector-toc-level-1 vector-toc-list-item-expanded"> <a class="vector-toc-link" href="#Causes"> <div class="vector-toc-text"> <span class="vector-toc-numb">2</span> <span>Causes</span> </div> </a> <button aria-controls="toc-Causes-sublist" 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class="vector-icon mw-ui-icon-listBullet mw-ui-icon-wikimedia-listBullet"></span> <span class="vector-dropdown-label-text">Toggle the table of contents</span> </label> <div class="vector-dropdown-content"> <div id="vector-page-titlebar-toc-unpinned-container" class="vector-unpinned-container"> </div> </div> </div> </nav> <h1 id="firstHeading" class="firstHeading mw-first-heading"><span class="mw-page-title-main">Infantile epileptic spasms syndrome</span></h1> <div id="p-lang-btn" class="vector-dropdown mw-portlet mw-portlet-lang" > <input type="checkbox" id="p-lang-btn-checkbox" role="button" aria-haspopup="true" data-event-name="ui.dropdown-p-lang-btn" class="vector-dropdown-checkbox mw-interlanguage-selector" aria-label="Go to an article in another language. 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href="https://az.wikipedia.org/wiki/Vest_sindromu" title="Vest sindromu – Azerbaijani" lang="az" hreflang="az" data-title="Vest sindromu" data-language-autonym="Azərbaycanca" data-language-local-name="Azerbaijani" class="interlanguage-link-target"><span>Azərbaycanca</span></a></li><li class="interlanguage-link interwiki-ca mw-list-item"><a href="https://ca.wikipedia.org/wiki/S%C3%ADndrome_de_West" title="Síndrome de West – Catalan" lang="ca" hreflang="ca" data-title="Síndrome de West" data-language-autonym="Català" data-language-local-name="Catalan" class="interlanguage-link-target"><span>Català</span></a></li><li class="interlanguage-link interwiki-cs mw-list-item"><a href="https://cs.wikipedia.org/wiki/West%C5%AFv_syndrom" title="Westův syndrom – Czech" lang="cs" hreflang="cs" data-title="Westův syndrom" data-language-autonym="Čeština" data-language-local-name="Czech" class="interlanguage-link-target"><span>Čeština</span></a></li><li class="interlanguage-link interwiki-de mw-list-item"><a href="https://de.wikipedia.org/wiki/West-Syndrom" title="West-Syndrom – German" lang="de" hreflang="de" data-title="West-Syndrom" data-language-autonym="Deutsch" data-language-local-name="German" class="interlanguage-link-target"><span>Deutsch</span></a></li><li class="interlanguage-link interwiki-es mw-list-item"><a href="https://es.wikipedia.org/wiki/S%C3%ADndrome_de_West" title="Síndrome de West – Spanish" lang="es" hreflang="es" data-title="Síndrome de West" data-language-autonym="Español" data-language-local-name="Spanish" class="interlanguage-link-target"><span>Español</span></a></li><li class="interlanguage-link interwiki-fr mw-list-item"><a href="https://fr.wikipedia.org/wiki/Syndrome_de_West" title="Syndrome de West – French" lang="fr" hreflang="fr" data-title="Syndrome de West" data-language-autonym="Français" data-language-local-name="French" class="interlanguage-link-target"><span>Français</span></a></li><li class="interlanguage-link interwiki-it mw-list-item"><a href="https://it.wikipedia.org/wiki/Sindrome_di_West" title="Sindrome di West – Italian" lang="it" hreflang="it" data-title="Sindrome di West" data-language-autonym="Italiano" data-language-local-name="Italian" class="interlanguage-link-target"><span>Italiano</span></a></li><li class="interlanguage-link interwiki-he mw-list-item"><a href="https://he.wikipedia.org/wiki/%D7%AA%D7%A1%D7%9E%D7%95%D7%A0%D7%AA_%D7%95%D7%A1%D7%98" title="תסמונת וסט – Hebrew" lang="he" hreflang="he" data-title="תסמונת וסט" data-language-autonym="עברית" data-language-local-name="Hebrew" class="interlanguage-link-target"><span>עברית</span></a></li><li class="interlanguage-link interwiki-hu mw-list-item"><a href="https://hu.wikipedia.org/wiki/West-szindr%C3%B3ma" title="West-szindróma – Hungarian" lang="hu" hreflang="hu" data-title="West-szindróma" data-language-autonym="Magyar" data-language-local-name="Hungarian" class="interlanguage-link-target"><span>Magyar</span></a></li><li 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data-language-local-name="Japanese" class="interlanguage-link-target"><span>日本語</span></a></li><li class="interlanguage-link interwiki-pl mw-list-item"><a href="https://pl.wikipedia.org/wiki/Zesp%C3%B3%C5%82_Westa" title="Zespół Westa – Polish" lang="pl" hreflang="pl" data-title="Zespół Westa" data-language-autonym="Polski" data-language-local-name="Polish" class="interlanguage-link-target"><span>Polski</span></a></li><li class="interlanguage-link interwiki-pt mw-list-item"><a href="https://pt.wikipedia.org/wiki/S%C3%ADndrome_de_West" title="Síndrome de West – Portuguese" lang="pt" hreflang="pt" data-title="Síndrome de West" data-language-autonym="Português" data-language-local-name="Portuguese" class="interlanguage-link-target"><span>Português</span></a></li><li class="interlanguage-link interwiki-zh mw-list-item"><a href="https://zh.wikipedia.org/wiki/%E9%9F%8B%E6%96%AF%E7%89%B9%E7%B6%9C%E5%90%88%E7%97%87" title="韋斯特綜合症 – Chinese" lang="zh" hreflang="zh" data-title="韋斯特綜合症" 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.infobox .navbar{font-size:100%}@media screen{html.skin-theme-clientpref-night .mw-parser-output .infobox-full-data:not(.notheme)>div:not(.notheme)[style]{background:#1f1f23!important;color:#f8f9fa}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .infobox-full-data:not(.notheme) div:not(.notheme){background:#1f1f23!important;color:#f8f9fa}}@media(min-width:640px){body.skin--responsive .mw-parser-output .infobox-table{display:table!important}body.skin--responsive .mw-parser-output .infobox-table>caption{display:table-caption!important}body.skin--responsive .mw-parser-output .infobox-table>tbody{display:table-row-group}body.skin--responsive .mw-parser-output .infobox-table tr{display:table-row!important}body.skin--responsive .mw-parser-output .infobox-table th,body.skin--responsive .mw-parser-output .infobox-table td{padding-left:inherit;padding-right:inherit}}</style><table class="infobox ib-medical-condition"><tbody><tr><th colspan="2" class="infobox-above" style="background:#ccc">Infantile epileptic spasms syndrome</th></tr><tr><th scope="row" class="infobox-label"><a href="/wiki/Medical_specialty" title="Medical specialty">Specialty</a></th><td class="infobox-data"><a href="/wiki/Neurology" title="Neurology">Neurology</a> <span class="penicon autoconfirmed-show"><span class="mw-valign-text-top" typeof="mw:File/Frameless"><a href="https://www.wikidata.org/wiki/Q1041258?uselang=en#P1995" title="Edit this on Wikidata"><img alt="Edit this on Wikidata" src="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/10px-OOjs_UI_icon_edit-ltr-progressive.svg.png" decoding="async" width="10" height="10" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/15px-OOjs_UI_icon_edit-ltr-progressive.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/20px-OOjs_UI_icon_edit-ltr-progressive.svg.png 2x" data-file-width="20" data-file-height="20" /></a></span></span></td></tr></tbody></table> <p><b>Infantile epileptic spasms syndrome</b> <b>(IESS)</b> previously known as <b>West syndrome</b> needs the inclusion of <b>epileptic spasms</b> for diagnosis.<sup id="cite_ref-Epilepsy_action_1-0" class="reference"><a href="#cite_note-Epilepsy_action-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup> Epileptic spasms (also known as infantile spasms) may also occur outside of a syndrome (that is, in the absence of <a href="/wiki/Hypsarrhythmia" title="Hypsarrhythmia">hypsarrhythmia</a> and cognitive regression) - notably in association with severe brain disorders (e.g. <a href="/wiki/Lissencephaly" title="Lissencephaly">lissencephaly</a>).<sup id="cite_ref-Oxford_University_Press-2013_2-0" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> </p><p>IESS is an epileptic <a href="/wiki/Encephalopathy" title="Encephalopathy">encephalopathy</a>, a childhood epilepsy syndrome arising during <a href="/wiki/Infant" title="Infant">infancy</a>.<sup id="cite_ref-ILAE2024_3-0" class="reference"><a href="#cite_note-ILAE2024-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> It can often arise as a complication of various other medical conditions.<sup id="cite_ref-Oxford_University_Press-2013_2-1" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-rarediseases.org_4-0" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> It is clinically defined by the occurrence of the characteristic epileptic spasms, episodes of clusters of tonic spasms of the axial and limb musculature.<sup id="cite_ref-Panayiotopoulos-2007_5-0" class="reference"><a href="#cite_note-Panayiotopoulos-2007-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> Such spasms are found in association with characteristic abnormal <a href="/wiki/EEG" class="mw-redirect" title="EEG">EEG</a> pattern findings (<a href="/wiki/Hypsarrhythmia" title="Hypsarrhythmia">hypsarrhythmia</a>), and cognitive delay or deterioration.<sup id="cite_ref-Oxford_University_Press-2013_2-2" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-rarediseases.org_4-1" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> The peak age of onset is 4-6 months of age, with 90% of cases presenting during the first year of life. The spasms are usually resistant to conventional <a href="/wiki/Antiepileptic" class="mw-redirect" title="Antiepileptic">antiepileptics</a>. They may persist beyond infancy, or, rarely, commence only later in childhood. Many individuals with the syndrome go on to develop other forms of epilepsy later in life (notably <a href="/wiki/Lennox%E2%80%93Gastaut_syndrome" title="Lennox–Gastaut syndrome">Lennox–Gastaut syndrome</a>), and persisting neurodevelopmental deficits are common;<sup id="cite_ref-Oxford_University_Press-2013_2-3" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> notably, up to about a third of children are subsequently diagnosed with <a href="/wiki/Autism" title="Autism">autism</a>.<sup id="cite_ref-Coleman-2012_6-0" class="reference"><a href="#cite_note-Coleman-2012-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> <a href="/wiki/Pharmacotherapy" title="Pharmacotherapy">Pharmacotherapy</a> consists of either <a href="/wiki/Adrenocorticotropic_hormone" title="Adrenocorticotropic hormone">adrenocorticotropic hormone (ACTH)</a> or <a href="/wiki/Glucocorticoid" title="Glucocorticoid">glucocorticoids</a> (<a href="/wiki/Prednisone" title="Prednisone">prednisone</a>), or <a href="/wiki/Vigabatrin" title="Vigabatrin">vigabatrin</a>. <a href="/wiki/Ketogenic_diet" title="Ketogenic diet">Ketogenic diet</a> may be effective as second-line therapy for treatment-resistant cases. Neurosurgery may be indicated in certain cases.<sup id="cite_ref-Oxford_University_Press-2013_2-4" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-7" class="reference"><a href="#cite_note-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> </p><p>Epileptic spasms are commonly classified as symptomatic when a potential cause can be identified, or as cryptogenic if not (though these designations are used inconsistently).<sup id="cite_ref-Oxford_University_Press-2013_2-5" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> A specific cause can be identified in ~70-75%. Any condition that may cause cerebral insult may give rise to IESS. Causes range from genetic disorders, infections, congenital malformations, malnutrition, to brain trauma. The most commonly identified common cause is <a href="/wiki/Tuberous_sclerosis" title="Tuberous sclerosis">tuberous sclerosis complex</a>. Cryptogenic cases entail a more favourable prognosis overall.<sup id="cite_ref-rarediseases.org_4-2" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> </p><p>West syndrome is named for the English physician <a href="/wiki/William_James_West" title="William James West">William James West</a> who was first to describe the condition in an article in <i><a href="/wiki/The_Lancet" title="The Lancet">The Lancet</a></i> in 1841 based on observations of the condition in his son.<sup id="cite_ref-Epilepsy_action_1-1" class="reference"><a href="#cite_note-Epilepsy_action-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup> </p> <meta property="mw:PageProp/toc" /> <div class="mw-heading mw-heading2"><h2 id="Signs_and_symptoms">Signs and symptoms</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Infantile_epileptic_spasms_syndrome&action=edit&section=1" title="Edit section: Signs and symptoms"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <div class="mw-heading mw-heading3"><h3 id="Epileptic_spasms">Epileptic spasms</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Infantile_epileptic_spasms_syndrome&action=edit&section=2" title="Edit section: Epileptic spasms"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Epileptic spasms are a seizure type characteristic for the first year of life. The spasms are typically resistant to conventional pharmacotherapy. There are many episodes per day.<sup id="cite_ref-Oxford_University_Press-2013_2-6" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> Episodes may take place after waking or feeding,<sup id="cite_ref-rarediseases.org_4-3" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> or less often before falling asleep.<sup id="cite_ref-MSD_Manual_Professional_Edition_8-0" class="reference"><a href="#cite_note-MSD_Manual_Professional_Edition-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> Episode duration,<sup id="cite_ref-Oxford_University_Press-2013_2-7" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-rarediseases.org_4-4" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> intensity, and muscle groups affected are variable.<sup id="cite_ref-rarediseases.org_4-5" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> Mild spasms may involve mere nodding,<sup id="cite_ref-MSD_Manual_Professional_Edition_8-1" class="reference"><a href="#cite_note-MSD_Manual_Professional_Edition-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> muscle twitching or eye movements, whereas powerful spasms may result in the infant's body violently bending over (the so-called "<a href="/wiki/Salaam" class="mw-redirect" title="Salaam">salaam</a>" or "<a href="/wiki/Jackknife_(exercise)" title="Jackknife (exercise)">jackknife</a>" movements). Individual spasms typically last only seconds, but episodes may last over 20 minutes.<sup id="cite_ref-rarediseases.org_4-6" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> An episode is typically followed by exhaustion;<sup id="cite_ref-Oxford_University_Press-2013_2-8" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Panayiotopoulos-2007_5-1" class="reference"><a href="#cite_note-Panayiotopoulos-2007-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> episodes are typically followed by over a minute of motionaless and diminished responsiveness.<sup id="cite_ref-Panayiotopoulos-2007_5-2" class="reference"><a href="#cite_note-Panayiotopoulos-2007-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> </p><p>The spasms present as episodes of brisk (0.2-2s<sup id="cite_ref-Panayiotopoulos-2007_5-3" class="reference"><a href="#cite_note-Panayiotopoulos-2007-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup>) neck flexions-extensions and upper limp abductions-adductions,<sup id="cite_ref-Oxford_University_Press-2013_2-9" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> lower limb extension, and trunk musculature contractions,<sup id="cite_ref-rarediseases.org_4-7" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> accompanied by upward deviation of the eyes.<sup id="cite_ref-Oxford_University_Press-2013_2-10" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> Nevertheless, individual muscle groups (abdominal, shoulder, neck) may be involved.<sup id="cite_ref-Panayiotopoulos-2007_5-4" class="reference"><a href="#cite_note-Panayiotopoulos-2007-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> Most often, there is simultaneous contraction of both flexors and extensors, followed by flexor spasms, and the least frequent extensor spasms.<sup id="cite_ref-Panayiotopoulos-2007_5-5" class="reference"><a href="#cite_note-Panayiotopoulos-2007-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-MSD_Manual_Professional_Edition_8-2" class="reference"><a href="#cite_note-MSD_Manual_Professional_Edition-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> Spasms are usually symmetrical, but up to 30% of cases may exhibit varying degrees of lateralisation.<sup id="cite_ref-Panayiotopoulos-2007_5-6" class="reference"><a href="#cite_note-Panayiotopoulos-2007-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> Unilateral brain lesions often (but not always) result in asymmetric spasms; unilateral spasms may progress to generalised spasms.<sup id="cite_ref-Oxford_University_Press-2013_2-11" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> Drop attacks may be the initial presentation of West syndrome of later onset. Altered or absent breathing is common during episodes.<sup id="cite_ref-Panayiotopoulos-2007_5-7" class="reference"><a href="#cite_note-Panayiotopoulos-2007-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> </p><p>When spontaneous remissions occurs, it is typically gradual. Remission by the age of three is 50%, rising to 90% by the age of five.<sup id="cite_ref-Oxford_University_Press-2013_2-12" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Developmental">Developmental</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Infantile_epileptic_spasms_syndrome&action=edit&section=3" title="Edit section: Developmental"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The onset of epileptic spasms is often associated with developmental regression: autistic withdrawal, and loss of social smiling and of visual attention.<sup id="cite_ref-Oxford_University_Press-2013_2-13" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> A majority of individuals with West syndrome exhibit regression of psychomotor skills.<sup id="cite_ref-rarediseases.org_4-8" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> However, developmental delay is noted in up to about two-thirds of infants with West syndrome already before the onset of spasms,<sup id="cite_ref-Oxford_University_Press-2013_2-14" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Panayiotopoulos-2007_5-8" class="reference"><a href="#cite_note-Panayiotopoulos-2007-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> whereas only about a third had exhibited normal development prior to spasm onset.<sup id="cite_ref-Panayiotopoulos-2007_5-9" class="reference"><a href="#cite_note-Panayiotopoulos-2007-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Causes">Causes</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Infantile_epileptic_spasms_syndrome&action=edit&section=4" title="Edit section: Causes"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Based on etiology, cases of IESS are commonly classified as either symptomatic or <a href="/wiki/Cryptogenic_disease" class="mw-redirect" title="Cryptogenic disease">cryptogenic</a> - although these terms have not been used consistently. Symptomatic cases are most often defined as those in which a clear cause can be identified, though some investigators also use the designation in cases in which there was previous clinical or imaging evidence of brain lesions and/or abnormal development was noted prior to the onset of the syndrome. Cryptogenic cases are thus contrastingly defined as those in which no specific cause can be identified, or where no such lesions or abnormalities were noted prior to syndrome onset.<sup id="cite_ref-Oxford_University_Press-2013_2-15" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> </p> <ul><li>Brain malformations <ul><li><a href="/wiki/Microcephaly" title="Microcephaly">Microcephaly</a><sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (December 2020)">citation needed</span></a></i>]</sup></li> <li><a href="/wiki/Hemimegalencephaly" title="Hemimegalencephaly">Hemimegalencephaly</a><sup id="cite_ref-rarediseases.org_4-9" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Panayiotopoulos-2007_5-10" class="reference"><a href="#cite_note-Panayiotopoulos-2007-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Cortical_dysplasia" class="mw-redirect" title="Cortical dysplasia">Cortical dysplasia</a><sup id="cite_ref-rarediseases.org_4-10" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Cerebral_atrophy" title="Cerebral atrophy">Cerebral atrophy</a><sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (December 2020)">citation needed</span></a></i>]</sup></li> <li><a href="/wiki/Focal_cortical_dysplasia" title="Focal cortical dysplasia">Focal cortical dysplasia</a><sup id="cite_ref-Panayiotopoulos-2007_5-11" class="reference"><a href="#cite_note-Panayiotopoulos-2007-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Lissencephaly" title="Lissencephaly">Lissencephaly</a> (may be of heritable aetiology)<sup id="cite_ref-Oxford_University_Press-2013_2-16" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Porencephaly" title="Porencephaly">Porencephaly</a><sup id="cite_ref-Panayiotopoulos-2007_5-12" class="reference"><a href="#cite_note-Panayiotopoulos-2007-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Pachygyria" title="Pachygyria">Pachygyria</a><sup id="cite_ref-Panayiotopoulos-2007_5-13" class="reference"><a href="#cite_note-Panayiotopoulos-2007-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Polymicrogyria" title="Polymicrogyria">Peri-sylvian polymicrogyria</a><sup id="cite_ref-Panayiotopoulos-2007_5-14" class="reference"><a href="#cite_note-Panayiotopoulos-2007-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Gray_matter_heterotopia" title="Gray matter heterotopia">Gray matter heterotopia</a><sup id="cite_ref-Panayiotopoulos-2007_5-15" class="reference"><a href="#cite_note-Panayiotopoulos-2007-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Aicardi_syndrome" title="Aicardi syndrome">Aicardi syndrome</a><sup id="cite_ref-Panayiotopoulos-2007_5-16" class="reference"><a href="#cite_note-Panayiotopoulos-2007-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup></li> <li>Cerebrovascular malformations<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (December 2020)">citation needed</span></a></i>]</sup></li></ul></li> <li>Genetic disorders <ul><li><a href="/wiki/Phakomatosis" title="Phakomatosis">Phakomatoses</a> (e.g. <a href="/wiki/Tuberous_sclerosis" title="Tuberous sclerosis">tuberous sclerosis</a>)<sup id="cite_ref-rarediseases.org_4-11" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Incontinentia_pigmenti" title="Incontinentia pigmenti">lncontinentia pigmenti</a><sup id="cite_ref-rarediseases.org_4-12" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Foix%E2%80%93Chavany%E2%80%93Marie_syndrome" title="Foix–Chavany–Marie syndrome">Foix–Chavany–Marie syndrome</a><sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (December 2020)">citation needed</span></a></i>]</sup></li> <li><a href="/wiki/Down_syndrome" title="Down syndrome">Down syndrome (trisomy 21)</a><sup id="cite_ref-rarediseases.org_4-13" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Patau_syndrome" title="Patau syndrome">Patau syndrome (trisomy 13)</a><sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (December 2020)">citation needed</span></a></i>]</sup></li> <li><a href="/wiki/Sturge%E2%80%93Weber_syndrome" title="Sturge–Weber syndrome">Sturge–Weber syndrome</a><sup id="cite_ref-rarediseases.org_4-14" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Maple_syrup_urine_disease" title="Maple syrup urine disease">Maple syrup urine disease</a><sup id="cite_ref-rarediseases.org_4-15" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Phenylketonuria" title="Phenylketonuria">Phenylketonuria</a><sup id="cite_ref-rarediseases.org_4-16" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Biotinidase_deficiency" title="Biotinidase deficiency">Biotinidase deficiency</a><sup id="cite_ref-rarediseases.org_4-17" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Ohtahara_syndrome" title="Ohtahara syndrome">Ohtahara syndrome</a><sup id="cite_ref-rarediseases.org_4-18" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/PEHO_syndrome" title="PEHO syndrome">PEHO syndrome</a><sup id="cite_ref-Oxford_University_Press-2013_2-17" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Leukodystrophy" title="Leukodystrophy">Leukodystrophy</a><sup id="cite_ref-Oxford_University_Press-2013_2-18" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Mitochondrial_disease" title="Mitochondrial disease">Mitochondrial diseases</a><sup id="cite_ref-Oxford_University_Press-2013_2-19" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-rarediseases.org_4-19" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup></li> <li>Mutations of the <a href="/wiki/ARX_(gene)" class="mw-redirect" title="ARX (gene)">ARX</a> gene, or <a href="/wiki/CDKL5" title="CDKL5">CDKL5</a> gene<sup id="cite_ref-rarediseases.org_4-20" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Oxford_University_Press-2013_2-20" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Chromosomal_deletion" class="mw-redirect" title="Chromosomal deletion">Chromosomal deletions</a> (e.g. affecting <a href="/wiki/MAGI2" title="MAGI2">MAGI2</a> gene that mediates <a href="/wiki/Glutamate_receptor" title="Glutamate receptor">glutamate receptor</a> functioning)<sup id="cite_ref-Oxford_University_Press-2013_2-21" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup></li></ul></li> <li>Infectious - may be either congenital or acquired. Infantile spasm outcomes tend to be especially poor when secondary to brain infection. Infectious agents include viral (cytomegalovirus, HSV, rubella, pertussis, adenovirus, enterovirus), bacterial (<a href="/wiki/Neisseria_meningitidis" title="Neisseria meningitidis">N. meningitidis</a>, <a href="/wiki/Streptococcus_pneumoniae" title="Streptococcus pneumoniae">S. pneumoniae</a>), protozoal (<a href="/wiki/Toxoplasmosis" title="Toxoplasmosis">toxoplasmosis</a>), and others.<sup id="cite_ref-Panayiotopoulos-2007_5-17" class="reference"><a href="#cite_note-Panayiotopoulos-2007-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup></li> <li>Metabolic <ul><li><a href="/wiki/Hypoglycemia" title="Hypoglycemia">Hypoglycemia</a><sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (December 2020)">citation needed</span></a></i>]</sup></li> <li>Non-<a href="/wiki/Ketoacidosis" title="Ketoacidosis">ketotic</a> <a href="/wiki/Hyperglycemia" title="Hyperglycemia">hyperglycemia</a><sup id="cite_ref-rarediseases.org_4-21" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Vitamin_B6_deficiency" class="mw-redirect" title="Vitamin B6 deficiency">Vitamin B6 deficiency</a><sup id="cite_ref-rarediseases.org_4-22" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup></li></ul></li> <li><a href="/wiki/Periventricular_leukomalacia" title="Periventricular leukomalacia">Periventricular leukomalacia</a></li> <li><a href="/wiki/Stroke" title="Stroke">Cerebrovascular accidents</a><sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (December 2020)">citation needed</span></a></i>]</sup> <ul><li><a href="/wiki/Cephalhematoma" class="mw-redirect" title="Cephalhematoma">Cephalhematoma</a><sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (December 2020)">citation needed</span></a></i>]</sup></li></ul></li> <li><a href="/wiki/Traumatic_brain_injury" title="Traumatic brain injury">Traumatic brain injury</a><sup id="cite_ref-rarediseases.org_4-23" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup></li> <li><a href="/wiki/Hypoxic_Ischaemic_Encephalopathy" class="mw-redirect" title="Hypoxic Ischaemic Encephalopathy">Hypoxic-ischaemic encephalopathy</a><sup id="cite_ref-Oxford_University_Press-2013_2-22" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> (e.g. <a href="/wiki/Perinatal_asphyxia" title="Perinatal asphyxia">perinatal asphyxia</a>) - likely the most common aetiology<sup id="cite_ref-Panayiotopoulos-2007_5-18" class="reference"><a href="#cite_note-Panayiotopoulos-2007-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup></li></ul> <div class="mw-heading mw-heading3"><h3 id="Down_syndrome">Down syndrome</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Infantile_epileptic_spasms_syndrome&action=edit&section=5" title="Edit section: Down syndrome"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Infantile epileptic spasms syndrome appears in 1% to 5% of infants with <a href="/wiki/Down_syndrome" title="Down syndrome">Down syndrome</a>. IESS in those with Down syndrome is milder, more responsive to treatment (due to unknown reasons), and less likely to evolve into <a href="/wiki/Lennox-Gastaut_syndrome" class="mw-redirect" title="Lennox-Gastaut syndrome">Lennox-Gastaut syndrome</a> or other forms of epilepsy.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (December 2020)">citation needed</span></a></i>]</sup> A child with Down syndrome presenting with seizures that are difficult to control should be assessed for <a href="/wiki/Autistic_spectrum_disorder" class="mw-redirect" title="Autistic spectrum disorder">autistic spectrum disorder</a>.<sup id="cite_ref-9" class="reference"><a href="#cite_note-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup><sup class="noprint Inline-Template" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Verifiability" title="Wikipedia:Verifiability"><span title="The material near this tag needs to be fact-checked with the cited source(s). (September 2024)">verification needed</span></a></i>]</sup> </p> <div class="mw-heading mw-heading3"><h3 id="Genetic">Genetic</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Infantile_epileptic_spasms_syndrome&action=edit&section=6" title="Edit section: Genetic"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Mutations in several genes have been associated with IESS. These include the Aristaless related homeobox (<a href="/wiki/Aristaless_related_homeobox" title="Aristaless related homeobox">ARX</a>) and cyclin dependent kinase like 5 (<a href="/wiki/CDKL5" title="CDKL5">CDKL5</a>) genes.<sup id="cite_ref-Bahi-Buisson2012_10-0" class="reference"><a href="#cite_note-Bahi-Buisson2012-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> The ARX gene in particular seems to be responsible for at least some of the X linked cases.<sup id="cite_ref-Sherr2003_11-0" class="reference"><a href="#cite_note-Sherr2003-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup> Variants in the <a href="/wiki/KCNT1" title="KCNT1">KCNT1</a> gene can also in rare cases result in West syndrome.<sup id="cite_ref-urlKCNT1-Related_Epilepsy_-_GeneReviews®_-_NCBI_Bookshelf_12-0" class="reference"><a href="#cite_note-urlKCNT1-Related_Epilepsy_-_GeneReviews®_-_NCBI_Bookshelf-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Diagnosis">Diagnosis</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Infantile_epileptic_spasms_syndrome&action=edit&section=7" title="Edit section: Diagnosis"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Infantile spasms can be misdiagnosed as non-epileptic, non-pathological movements such as <a href="/wiki/Infantile_colic" class="mw-redirect" title="Infantile colic">infantile colic</a>, <a href="/wiki/Startle_response" title="Startle response">startle response</a>, or <a href="/wiki/Moro_reflex" title="Moro reflex">Moro reflex</a>.<sup id="cite_ref-Oxford_University_Press-2013_2-23" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Treatment">Treatment</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Infantile_epileptic_spasms_syndrome&action=edit&section=8" title="Edit section: Treatment"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <div class="mw-heading mw-heading3"><h3 id="Pharmacotherapy">Pharmacotherapy</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Infantile_epileptic_spasms_syndrome&action=edit&section=9" title="Edit section: Pharmacotherapy"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>There is limited evidence as to which pharmacotherapy approach is optimal.<sup id="cite_ref-Wilmshurst2017rev_13-0" class="reference"><a href="#cite_note-Wilmshurst2017rev-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Oxford_University_Press-2013_2-24" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> Hormones therapy with either <a href="/wiki/Adrenocorticotropic_hormone_(medication)" title="Adrenocorticotropic hormone (medication)">adrenocorticotrophic hormone</a> (ACTH) or oral <a href="/wiki/Prednisone" title="Prednisone">prednisone</a> is the standard of care (with the two treatments apparently producing equivalent outcomes). ACTH therapy is cost-prohibitive in the US.<sup id="cite_ref-Wilmshurst2017rev_13-1" class="reference"><a href="#cite_note-Wilmshurst2017rev-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup> ACTH therapy produces improvement in spasms within days whereas neurodevelopmental improvements take weeks. ACTH therapy is associated with increased risk of infections (which account for the majority of deaths).<sup id="cite_ref-Oxford_University_Press-2013_2-25" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> Therapy with <a href="/wiki/Vigabatrin" title="Vigabatrin">vigabatrin</a> is also commonly undertaken (though long-term use is associated with a risk of <a href="/wiki/Visual_field#Visual_field_loss" title="Visual field">visual field loss</a>);<sup id="cite_ref-Wilmshurst2017rev_13-2" class="reference"><a href="#cite_note-Wilmshurst2017rev-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup> vigabatrin is considered the treatment of choice for infantile spasms associated with tuberous sclerosis complex, and is also favoured in those with serious brain lesions or malformations.<sup id="cite_ref-MSD_Manual_Professional_Edition_8-3" class="reference"><a href="#cite_note-MSD_Manual_Professional_Edition-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Neurosurgery">Neurosurgery</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Infantile_epileptic_spasms_syndrome&action=edit&section=10" title="Edit section: Neurosurgery"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Prompt neurosurgery may be indicated in treatment-resistant cases with a demonstrated localised epileptic focus. Some 60% of persons having undergone neurosurgery are subsequently seizure-free. More specifically, these neurosurgical procedures include: hemispherectomy/hemispherotomy and non-hemispheric surgery.<sup id="cite_ref-14" class="reference"><a href="#cite_note-14"><span class="cite-bracket">[</span>14<span class="cite-bracket">]</span></a></sup> Small epileptic foci augur a favourable outcome, however, in most cases, resection of extensive multilobar cortical dysplasias is called for, resulting in limited cognitive improvement.<sup id="cite_ref-Oxford_University_Press-2013_2-26" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Ketogenic_diet">Ketogenic diet</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Infantile_epileptic_spasms_syndrome&action=edit&section=11" title="Edit section: Ketogenic diet"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>There is some evidence for the use of the ketogenic diet in cases which have failed to respond to pharmacotherapy.<sup id="cite_ref-Oxford_University_Press-2013_2-27" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Prognosis">Prognosis</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Infantile_epileptic_spasms_syndrome&action=edit&section=12" title="Edit section: Prognosis"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Prognosis of epileptic spasms and IESS depends predominately upon aetiology, and less so on treatment. Unfavourable prognostic factors include: symptomatic aetiology, early onset (prior to 3 months), presence of other seizure types prior to onset of infantile spasms, poor treatment response, EEG asymmetry, absence of typical hypsarrhythmia, and (prolongued) developmental regression.<sup id="cite_ref-Oxford_University_Press-2013_2-28" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> Premature mortality rates range from 5% to 31%, and depend upon the underlying aetiology of the infantile spasms.<sup id="cite_ref-MSD_Manual_Professional_Edition_8-4" class="reference"><a href="#cite_note-MSD_Manual_Professional_Edition-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> </p><p>Whereas some 80% of all individuals with IESS will exhibit residual neurodevelopmental impairment, the figure falls to only a third for cryptogenic cases.<sup id="cite_ref-Oxford_University_Press-2013_2-29" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> Brisk initiation of therapy appears to be associated with more favourable neurodevelopmental outcomes - especially in cryptogenic cases.<sup id="cite_ref-MSD_Manual_Professional_Edition_8-5" class="reference"><a href="#cite_note-MSD_Manual_Professional_Edition-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Seizures">Seizures</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Infantile_epileptic_spasms_syndrome&action=edit&section=13" title="Edit section: Seizures"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>In about one quarter to one third of children with IESS, seizures will subside completely with time; such resolution is more common when the cause is cryptogenic. In another third, the characteristic epilepstic spasmswill persist in later life. Finally, a third will experience a deterioration with the appearance of additional recalcitrant seizure types - often evolving into <a href="/wiki/Lennox%E2%80%93Gastaut_syndrome" title="Lennox–Gastaut syndrome">Lennox–Gastaut syndrome</a>.<sup id="cite_ref-rarediseases.org_4-24" class="reference"><a href="#cite_note-rarediseases.org-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> About 50% of cases will exhibit other types of epilepsy later in life.<sup id="cite_ref-Oxford_University_Press-2013_2-30" class="reference"><a href="#cite_note-Oxford_University_Press-2013-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Autism">Autism</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Infantile_epileptic_spasms_syndrome&action=edit&section=14" title="Edit section: Autism"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>From 10% to 35% of children with infantile spasms are eventually recognised as autistic. Autism may arise more frequently in those with bilateral temporal lobe epileptic foci. The aetiology of infantile spasms-associated autism may be idiopathic, or an additional comorbidity that itself better explains the autism may be identified. It is believed that early aggressive treatment of infantile spasms can often prevent the later development of autistic features, or lessen their severity.<sup id="cite_ref-Coleman-2012_6-1" class="reference"><a href="#cite_note-Coleman-2012-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Epidemiology">Epidemiology</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Infantile_epileptic_spasms_syndrome&action=edit&section=15" title="Edit section: Epidemiology"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Incidence is around 1:3200 to 1:3500 of live births. Statistically, boys are more likely to be affected than girls at a ratio of around 3:2.<sup id="cite_ref-15" class="reference"><a href="#cite_note-15"><span class="cite-bracket">[</span>15<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="See_also">See also</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Infantile_epileptic_spasms_syndrome&action=edit&section=16" title="Edit section: See also"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <ul><li><a href="/wiki/Epilepsy_Phenome/Genome_Project" title="Epilepsy Phenome/Genome Project">Epilepsy Phenome/Genome Project</a></li></ul> <div class="mw-heading mw-heading2"><h2 id="References">References</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Infantile_epileptic_spasms_syndrome&action=edit&section=17" title="Edit section: References"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <style data-mw-deduplicate="TemplateStyles:r1239543626">.mw-parser-output .reflist{margin-bottom:0.5em;list-style-type:decimal}@media screen{.mw-parser-output .reflist{font-size:90%}}.mw-parser-output .reflist .references{font-size:100%;margin-bottom:0;list-style-type:inherit}.mw-parser-output .reflist-columns-2{column-width:30em}.mw-parser-output .reflist-columns-3{column-width:25em}.mw-parser-output .reflist-columns{margin-top:0.3em}.mw-parser-output .reflist-columns ol{margin-top:0}.mw-parser-output .reflist-columns li{page-break-inside:avoid;break-inside:avoid-column}.mw-parser-output .reflist-upper-alpha{list-style-type:upper-alpha}.mw-parser-output .reflist-upper-roman{list-style-type:upper-roman}.mw-parser-output .reflist-lower-alpha{list-style-type:lower-alpha}.mw-parser-output .reflist-lower-greek{list-style-type:lower-greek}.mw-parser-output .reflist-lower-roman{list-style-type:lower-roman}</style><div class="reflist"> <div class="mw-references-wrap mw-references-columns"><ol class="references"> <li id="cite_note-Epilepsy_action-1"><span class="mw-cite-backlink">^ <a href="#cite_ref-Epilepsy_action_1-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-Epilepsy_action_1-1"><sup><i><b>b</b></i></sup></a></span> <span class="reference-text"><style data-mw-deduplicate="TemplateStyles:r1238218222">.mw-parser-output cite.citation{font-style:inherit;word-wrap:break-word}.mw-parser-output .citation q{quotes:"\"""\"""'""'"}.mw-parser-output .citation:target{background-color:rgba(0,127,255,0.133)}.mw-parser-output .id-lock-free.id-lock-free a{background:url("//upload.wikimedia.org/wikipedia/commons/6/65/Lock-green.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-limited.id-lock-limited a,.mw-parser-output .id-lock-registration.id-lock-registration a{background:url("//upload.wikimedia.org/wikipedia/commons/d/d6/Lock-gray-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-subscription.id-lock-subscription a{background:url("//upload.wikimedia.org/wikipedia/commons/a/aa/Lock-red-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .cs1-ws-icon a{background:url("//upload.wikimedia.org/wikipedia/commons/4/4c/Wikisource-logo.svg")right 0.1em center/12px no-repeat}body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-free a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-limited a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-registration a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-subscription a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .cs1-ws-icon a{background-size:contain;padding:0 1em 0 0}.mw-parser-output .cs1-code{color:inherit;background:inherit;border:none;padding:inherit}.mw-parser-output .cs1-hidden-error{display:none;color:var(--color-error,#d33)}.mw-parser-output .cs1-visible-error{color:var(--color-error,#d33)}.mw-parser-output .cs1-maint{display:none;color:#085;margin-left:0.3em}.mw-parser-output .cs1-kern-left{padding-left:0.2em}.mw-parser-output .cs1-kern-right{padding-right:0.2em}.mw-parser-output .citation .mw-selflink{font-weight:inherit}@media screen{.mw-parser-output .cs1-format{font-size:95%}html.skin-theme-clientpref-night .mw-parser-output .cs1-maint{color:#18911f}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .cs1-maint{color:#18911f}}</style><cite class="citation web cs1"><a rel="nofollow" class="external text" href="https://www.epilepsy.org.uk/info/syndromes/west-syndrome-infantile-spasms">"Infantile epileptic spasms syndrome"</a>. <i>Epilepsy Action</i>.</cite><span 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D.; Guerrini, Renzo; Cook, Mark; Lhatoo, Samden D., eds. (2013). <a rel="nofollow" class="external text" href="https://www.worldcat.org/title/806014746"><i>Oxford Textbook of Epilepsy and Epileptic Seizures</i></a>. Oxford Textbooks in Clinical Neurology. 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Retrieved <span class="nowrap">2024-09-28</span></span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=rarediseases.org&rft.atitle=West+Syndrome+-+Symptoms%2C+Causes%2C+Treatment+%7C+NORD&rft_id=https%3A%2F%2Frarediseases.org%2Frare-diseases%2Fwest-syndrome%2F&rfr_id=info%3Asid%2Fen.wikipedia.org%3AInfantile+epileptic+spasms+syndrome" class="Z3988"></span></span> </li> <li id="cite_note-Panayiotopoulos-2007-5"><span class="mw-cite-backlink">^ <a href="#cite_ref-Panayiotopoulos-2007_5-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-Panayiotopoulos-2007_5-1"><sup><i><b>b</b></i></sup></a> <a href="#cite_ref-Panayiotopoulos-2007_5-2"><sup><i><b>c</b></i></sup></a> <a href="#cite_ref-Panayiotopoulos-2007_5-3"><sup><i><b>d</b></i></sup></a> <a href="#cite_ref-Panayiotopoulos-2007_5-4"><sup><i><b>e</b></i></sup></a> <a href="#cite_ref-Panayiotopoulos-2007_5-5"><sup><i><b>f</b></i></sup></a> <a href="#cite_ref-Panayiotopoulos-2007_5-6"><sup><i><b>g</b></i></sup></a> <a href="#cite_ref-Panayiotopoulos-2007_5-7"><sup><i><b>h</b></i></sup></a> <a href="#cite_ref-Panayiotopoulos-2007_5-8"><sup><i><b>i</b></i></sup></a> <a href="#cite_ref-Panayiotopoulos-2007_5-9"><sup><i><b>j</b></i></sup></a> <a href="#cite_ref-Panayiotopoulos-2007_5-10"><sup><i><b>k</b></i></sup></a> <a href="#cite_ref-Panayiotopoulos-2007_5-11"><sup><i><b>l</b></i></sup></a> <a href="#cite_ref-Panayiotopoulos-2007_5-12"><sup><i><b>m</b></i></sup></a> <a href="#cite_ref-Panayiotopoulos-2007_5-13"><sup><i><b>n</b></i></sup></a> <a href="#cite_ref-Panayiotopoulos-2007_5-14"><sup><i><b>o</b></i></sup></a> <a href="#cite_ref-Panayiotopoulos-2007_5-15"><sup><i><b>p</b></i></sup></a> <a href="#cite_ref-Panayiotopoulos-2007_5-16"><sup><i><b>q</b></i></sup></a> <a href="#cite_ref-Panayiotopoulos-2007_5-17"><sup><i><b>r</b></i></sup></a> <a href="#cite_ref-Panayiotopoulos-2007_5-18"><sup><i><b>s</b></i></sup></a></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFPanayiotopoulos2007" class="citation book cs1">Panayiotopoulos, C. 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(1993). <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/books/NBK525917/">"KCNT1-Related Epilepsy"</a>. <i>KCNT1-Related Epilepsy - GeneReviews® - NCBI Bookshelf</i>. 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Michelle; Venkataramani, Maya; Lerman, Jennifer L.; Zhang, Allen; Sharma, Ritu; Bass, Eric B. (2022). <a rel="nofollow" class="external text" href="https://effectivehealthcare.ahrq.gov/products/outcomes-nutrition/research">"Maternal and Child Outcomes Associated With the Special Supplemental Nutrition Program for Women, Infants, and Children (WIC)"</a>. <i>effectivehealthcare.ahrq.gov</i>. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.23970%2Fahrqepccer253">10.23970/ahrqepccer253</a>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/35503870">35503870</a><span class="reference-accessdate">. Retrieved <span class="nowrap">2024-10-21</span></span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=effectivehealthcare.ahrq.gov&rft.atitle=Maternal+and+Child+Outcomes+Associated+With+the+Special+Supplemental+Nutrition+Program+for+Women%2C+Infants%2C+and+Children+%28WIC%29&rft.date=2022&rft_id=info%3Adoi%2F10.23970%2Fahrqepccer253&rft_id=info%3Apmid%2F35503870&rft.aulast=Caulfield&rft.aufirst=Laura+E.&rft.au=Bennett%2C+Wendy+L.&rft.au=Gross%2C+Susan+M.&rft.au=Hurley%2C+Kristen+M.&rft.au=Ogunwole%2C+S.+Michelle&rft.au=Venkataramani%2C+Maya&rft.au=Lerman%2C+Jennifer+L.&rft.au=Zhang%2C+Allen&rft.au=Sharma%2C+Ritu&rft.au=Bass%2C+Eric+B.&rft_id=https%3A%2F%2Feffectivehealthcare.ahrq.gov%2Fproducts%2Foutcomes-nutrition%2Fresearch&rfr_id=info%3Asid%2Fen.wikipedia.org%3AInfantile+epileptic+spasms+syndrome" class="Z3988"></span></span> </li> <li id="cite_note-15"><span class="mw-cite-backlink"><b><a href="#cite_ref-15">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFJohnstonAdamsFatemi2016" class="citation book cs1">Johnston, Michael V.; Adams, Harold P.; Fatemi, Ali (2016-08-18). <a rel="nofollow" class="external text" href="https://books.google.com/books?id=SdTeDAAAQBAJ&q=West+syndrome+boys+girls+ratio&pg=PT1346"><i>Neurobiology of Disease</i></a>. Oxford University Press. <a href="/wiki/ISBN_(identifier)" class="mw-redirect" title="ISBN (identifier)">ISBN</a> <a href="/wiki/Special:BookSources/9780190219086" title="Special:BookSources/9780190219086"><bdi>9780190219086</bdi></a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Abook&rft.genre=book&rft.btitle=Neurobiology+of+Disease&rft.pub=Oxford+University+Press&rft.date=2016-08-18&rft.isbn=9780190219086&rft.aulast=Johnston&rft.aufirst=Michael+V.&rft.au=Adams%2C+Harold+P.&rft.au=Fatemi%2C+Ali&rft_id=https%3A%2F%2Fbooks.google.com%2Fbooks%3Fid%3DSdTeDAAAQBAJ%26q%3DWest%2Bsyndrome%2Bboys%2Bgirls%2Bratio%26pg%3DPT1346&rfr_id=info%3Asid%2Fen.wikipedia.org%3AInfantile+epileptic+spasms+syndrome" class="Z3988"></span></span> </li> </ol></div></div> <div class="mw-heading mw-heading2"><h2 id="External_links">External links</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a 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class="nowraplinks navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="row" class="navbox-group" style="width:1%;background: #EAECF0;color:black;">Classification</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"><div style="position:relative; float:right; font-size:0.8em;"><a href="https://www.wikidata.org/wiki/Q1041258" class="extiw" title="d:Q1041258">D</a></div><div class="hlist" style="text-align:left;"><ul><li><b><a href="/wiki/International_Statistical_Classification_of_Diseases_and_Related_Health_Problems" class="mw-redirect" title="International Statistical Classification of Diseases and Related Health Problems">ICD</a>-<a href="/wiki/ICD-10" title="ICD-10">10</a></b>: <a rel="nofollow" class="external text" href="https://icd.who.int/browse10/2019/en#/G40.4">G40.4</a></li><li><b><a href="/wiki/International_Statistical_Classification_of_Diseases_and_Related_Health_Problems" class="mw-redirect" title="International Statistical Classification of Diseases and Related Health Problems">ICD</a>-<a href="/wiki/List_of_ICD-9_codes" title="List of ICD-9 codes">9-CM</a></b>: <a rel="nofollow" class="external text" href="http://www.icd9data.com/getICD9Code.ashx?icd9=345.6">345.6</a></li><li><b><a href="/wiki/Online_Mendelian_Inheritance_in_Man" title="Online Mendelian Inheritance in Man">OMIM</a></b>: <a rel="nofollow" class="external text" href="https://omim.org/entry/308350">308350</a></li><li><b><a href="/wiki/Medical_Subject_Headings" title="Medical Subject Headings">MeSH</a></b>: <a rel="nofollow" class="external text" href="https://meshb.nlm.nih.gov/record/ui?ui=D013036">D013036</a></li><li><b><a href="/wiki/Diseases_Database" title="Diseases Database">DiseasesDB</a></b>: <a rel="nofollow" class="external text" href="http://www.diseasesdatabase.com/ddb6788.htm">6788</a></li></ul></div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%;background: #EAECF0;color:black;">External resources</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"><div class="hlist" style="text-align:left;"><ul><li><b><a href="/wiki/EMedicine" title="EMedicine">eMedicine</a></b>: <a rel="nofollow" class="external text" href="https://emedicine.medscape.com/neuro/171-overview">neuro/171</a></li></ul></div></div></td></tr></tbody></table></div> <dl><dd><i>Much of this article is translated from <a href="https://de.wikipedia.org/wiki/West-Syndrom" class="extiw" title="de:West-Syndrom">the German Wikipedia article</a></i></dd></dl> <div class="navbox-styles"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236075235"></div><div role="navigation" class="navbox" aria-labelledby="Seizures_and_epilepsy161" style="padding:3px"><table class="nowraplinks mw-collapsible autocollapse navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="col" class="navbox-title" colspan="2"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><style data-mw-deduplicate="TemplateStyles:r1239400231">.mw-parser-output .navbar{display:inline;font-size:88%;font-weight:normal}.mw-parser-output .navbar-collapse{float:left;text-align:left}.mw-parser-output .navbar-boxtext{word-spacing:0}.mw-parser-output .navbar ul{display:inline-block;white-space:nowrap;line-height:inherit}.mw-parser-output .navbar-brackets::before{margin-right:-0.125em;content:"[ "}.mw-parser-output .navbar-brackets::after{margin-left:-0.125em;content:" ]"}.mw-parser-output .navbar li{word-spacing:-0.125em}.mw-parser-output .navbar a>span,.mw-parser-output .navbar a>abbr{text-decoration:inherit}.mw-parser-output .navbar-mini abbr{font-variant:small-caps;border-bottom:none;text-decoration:none;cursor:inherit}.mw-parser-output .navbar-ct-full{font-size:114%;margin:0 7em}.mw-parser-output .navbar-ct-mini{font-size:114%;margin:0 4em}html.skin-theme-clientpref-night .mw-parser-output .navbar li a abbr{color:var(--color-base)!important}@media(prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .navbar li a abbr{color:var(--color-base)!important}}@media print{.mw-parser-output .navbar{display:none!important}}</style><div class="navbar plainlinks hlist navbar-mini"><ul><li class="nv-view"><a href="/wiki/Template:Seizures_and_epilepsy" title="Template:Seizures and epilepsy"><abbr title="View this template">v</abbr></a></li><li class="nv-talk"><a href="/wiki/Template_talk:Seizures_and_epilepsy" title="Template talk:Seizures and epilepsy"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a 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title="Neonatal seizure">Neonatal seizure</a></li> <li><a href="/wiki/Epilepsy_in_children" title="Epilepsy in children">Epilepsy in children</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Management</th><td class="navbox-list-with-group navbox-list navbox-even hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Anticonvulsant" title="Anticonvulsant">Anticonvulsants</a></li> <li>Investigations <ul><li><a href="/wiki/Electroencephalography" title="Electroencephalography">Electroencephalography</a></li></ul></li> <li><a href="/wiki/Epileptologist" title="Epileptologist">Epileptologist</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Issues_for_people_with_epilepsy" title="Issues for people with epilepsy">Personal issues</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Epilepsy_and_driving" title="Epilepsy and driving">Epilepsy and driving</a></li> <li><a href="/wiki/Epilepsy_and_employment" title="Epilepsy and employment">Epilepsy and employment</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Seizure_types" title="Seizure types">Seizure types</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Focal_seizure" title="Focal seizure">Focal</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <dl><dt>Seizures</dt> <dd><a href="/wiki/Simple_partial_seizure" class="mw-redirect" title="Simple partial seizure">Simple partial</a></dd> <dd><a href="/wiki/Complex_partial_seizure" class="mw-redirect" title="Complex partial seizure">Complex partial</a></dd> <dd><a href="/wiki/Gelastic_seizure" title="Gelastic seizure">Gelastic seizure</a></dd></dl> <dl><dt>Epilepsy</dt> <dd><a href="/wiki/Temporal_lobe_epilepsy" title="Temporal lobe epilepsy">Temporal lobe epilepsy</a></dd> <dd><a href="/wiki/Frontal_lobe_epilepsy" title="Frontal lobe epilepsy">Frontal lobe epilepsy</a></dd> <dd><a href="/wiki/Rolandic_epilepsy" title="Rolandic epilepsy">Rolandic epilepsy</a></dd> <dd><a href="/wiki/Sleep-related_hypermotor_epilepsy" title="Sleep-related hypermotor epilepsy">Sleep-related hypermotor epilepsy</a></dd> <dd><a href="/wiki/Panayiotopoulos_syndrome" title="Panayiotopoulos syndrome">Panayiotopoulos syndrome</a></dd> <dd><a href="/wiki/Vertiginous_epilepsy" title="Vertiginous epilepsy">Vertiginous epilepsy</a></dd></dl> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Generalised_epilepsy" class="mw-redirect" title="Generalised epilepsy">Generalised</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Generalized_tonic%E2%80%93clonic_seizure" title="Generalized tonic–clonic seizure">Tonic–clonic</a></li> <li><a href="/wiki/Absence_seizure" title="Absence seizure">Absence seizure</a></li> <li><a href="/wiki/Atonic_seizure" title="Atonic seizure">Atonic seizure</a></li> <li><a href="/wiki/Automatism_(medicine)" title="Automatism (medicine)">Automatism</a></li> <li><a href="/wiki/Benign_familial_neonatal_seizures" title="Benign familial neonatal seizures">Benign familial neonatal seizures</a></li> <li><a href="/wiki/Lennox%E2%80%93Gastaut_syndrome" title="Lennox–Gastaut syndrome">Lennox–Gastaut syndrome</a></li> <li><a href="/wiki/Myoclonic_astatic_epilepsy" title="Myoclonic astatic epilepsy">Myoclonic astatic epilepsy</a></li> <li><a href="/wiki/Epileptic_spasms" class="mw-redirect" title="Epileptic spasms">Epileptic spasms</a></li> <li><a href="/wiki/Febrile_seizure" title="Febrile seizure">Febrile seizure</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Status_epilepticus" title="Status epilepticus">Status epilepticus</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Epilepsia_partialis_continua" title="Epilepsia partialis continua">Epilepsia partialis continua</a></li> <li><a href="/wiki/Complex_partial_status_epilepticus" title="Complex partial status epilepticus">Complex partial status epilepticus</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Myoclonic_epilepsy" title="Myoclonic epilepsy">Myoclonic epilepsy</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Progressive_myoclonus_epilepsy" title="Progressive myoclonus epilepsy">Progressive myoclonus epilepsy</a> <ul><li><a href="/wiki/Dentatorubral%E2%80%93pallidoluysian_atrophy" title="Dentatorubral–pallidoluysian atrophy">Dentatorubral–pallidoluysian atrophy</a></li> <li><a href="/wiki/Unverricht%E2%80%93Lundborg_disease" title="Unverricht–Lundborg disease">Unverricht–Lundborg disease</a></li> <li><a href="/wiki/MERRF_syndrome" title="MERRF syndrome">MERRF syndrome</a></li> <li><a href="/wiki/Lafora_disease" title="Lafora disease">Lafora disease</a></li></ul></li> <li><a href="/wiki/Early_myoclonic_encephalopathy" title="Early myoclonic encephalopathy">Early myoclonic encephalopathy</a></li> <li><a href="/wiki/Juvenile_myoclonic_epilepsy" title="Juvenile myoclonic epilepsy">Juvenile myoclonic epilepsy</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Related disorders</th><td class="navbox-list-with-group navbox-list navbox-even hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Sudden_unexpected_death_in_epilepsy" title="Sudden unexpected death in epilepsy">Sudden unexpected death in epilepsy</a></li> <li><a href="/wiki/Todd%27s_paresis" title="Todd's paresis">Todd's paresis</a></li> <li><a href="/wiki/Landau%E2%80%93Kleffner_syndrome" title="Landau–Kleffner syndrome">Landau–Kleffner syndrome</a></li> <li><a href="/wiki/Psychogenic_non-epileptic_seizure" title="Psychogenic non-epileptic seizure">Psychogenic non-epileptic seizure</a></li> <li><a href="/wiki/Epilepsy_in_animals" title="Epilepsy in animals">Epilepsy in animals</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Organizations</th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Citizens_United_for_Research_in_Epilepsy" title="Citizens United for Research in Epilepsy">Citizens United for Research in Epilepsy</a> (US)</li> <li><a href="/wiki/Epilepsy_Action" title="Epilepsy Action">Epilepsy Action</a> (UK)</li> <li><a href="/wiki/Epilepsy_Action_Australia" title="Epilepsy Action Australia">Epilepsy Action Australia</a></li> <li><a href="/wiki/Epilepsy_Foundation" title="Epilepsy Foundation">Epilepsy Foundation</a> (US)</li> <li><a href="/wiki/Epilepsy_Outlook" title="Epilepsy Outlook">Epilepsy Outlook</a> (UK)</li> <li><a href="/wiki/Epilepsy_Research_UK" class="mw-redirect" title="Epilepsy Research UK">Epilepsy Research UK</a></li> <li><a href="/wiki/Epilepsy_Society" title="Epilepsy Society">Epilepsy Society</a> (UK)</li></ul> </div></td></tr></tbody></table></div> <div class="navbox-styles"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236075235"></div><div role="navigation" class="navbox authority-control" aria-label="Navbox506" style="padding:3px"><table 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