{"id":9641,"date":"2022-03-15T18:47:07","date_gmt":"2022-03-15T18:47:07","guid":{"rendered":"https:\/\/medicalcriteria.com\/web\/?p=9641"},"modified":"2025-12-30T20:17:52","modified_gmt":"2025-12-30T20:17:52","slug":"spasms-and-west","status":"publish","type":"post","link":"https:\/\/medicalcriteria.com\/web\/spasms-and-west\/","title":{"rendered":"Diagnostic Criteria for Infantile Spasms and West Syndrome"},"content":{"rendered":"<div class=\"99c380e4b4a7b96c35d7ddf7dcb434e8\" data-index=\"1\" style=\"float: none; margin:0px 0 0px 0; text-align:center;\">\n<script async src=\"https:\/\/pagead2.googlesyndication.com\/pagead\/js\/adsbygoogle.js\"><\/script>\r\n<!-- MC 2019- Horizontal -->\r\n<ins class=\"adsbygoogle\"\r\n     style=\"display:block\"\r\n     data-ad-client=\"ca-pub-0127150553352455\"\r\n     data-ad-slot=\"3806776041\"\r\n     data-ad-format=\"auto\"\r\n     data-full-width-responsive=\"true\"><\/ins>\r\n<script>\r\n     (adsbygoogle = window.adsbygoogle || []).push({});\r\n<\/script>\n<\/div>\n<p>West syndrome is characterized by a specific type of seizure ( infantile spasms) and developmental regression.<\/p>\n<p>The gold standard method of diagnosing infantile spasms is to capture them on video-EEG to confirm the ictal correlate of the seizure.<!--more--><\/p>\n<p><strong>Guidance for the diagnosis of infantile spasms<\/strong><\/p>\n<p>Required either 1 of:<\/p>\n<ul>\n<li>Video-EEG confirmed spasms with typical ictal correlate<\/li>\n<li>Either home video of spasms with typical features or definite typical clustering spasms by history, PLUS probable or definite epileptic encephalopathy on EEG<\/li>\n<\/ul>\n<p>The 2021 BASED score*<\/p>\n<table style=\"border-collapse: collapse; width: 100%;\" border=\"1\">\n<tbody>\n<tr style=\"height: 24px;\">\n<td style=\"width: 23.6264%; height: 24px; text-align: center;\"><strong>BASED score<\/strong><\/td>\n<td style=\"width: 76.3736%; height: 24px;\"><strong>Description<\/strong><\/td>\n<\/tr>\n<tr style=\"height: 24px;\">\n<td style=\"width: 23.6264%; height: 24px; text-align: center;\">0<\/td>\n<td style=\"width: 76.3736%; height: 24px;\">Normal<\/td>\n<\/tr>\n<tr style=\"height: 24px;\">\n<td style=\"width: 23.6264%; height: 24px; text-align: center;\">1<\/td>\n<td style=\"width: 76.3736%; height: 24px;\">Any definite nonepileptiform abnormality<\/td>\n<\/tr>\n<tr style=\"height: 24px;\">\n<td style=\"width: 23.6264%; height: 24px; text-align: center;\">2<\/td>\n<td style=\"width: 76.3736%; height: 24px;\">&lt;3 spike foci AND no channel with abnormal high amplitude<\/td>\n<\/tr>\n<tr style=\"height: 48px;\">\n<td style=\"width: 23.6264%; height: 48px; text-align: center;\">3<\/td>\n<td style=\"width: 76.3736%; height: 48px;\">\u22653 spike foci &lt;50% of one second bins AND no channel with abnormal high amplitude, OR &lt;3 spike foci but \u22651 channel with abnormal high amplitude<\/td>\n<\/tr>\n<tr style=\"height: 48px;\">\n<td style=\"width: 23.6264%; height: 48px; text-align: center;\">4 (Probable EE)<\/td>\n<td style=\"width: 76.3736%; height: 48px;\">\u22653 spike foci &lt;50% of one second bins AND \u22651 channel with abnormal high amplitude, OR Not meeting criteria for 5 but includes GMFS or paroxysmal voltage attenuations<\/td>\n<\/tr>\n<tr style=\"height: 24px;\">\n<td style=\"width: 23.6264%; height: 24px; text-align: center;\">5 (Definite EE)<\/td>\n<td style=\"width: 76.3736%; height: 24px;\">\u22653 spike foci that are \u226550% of one second bins<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<p>BASED: Burden of AmplitudeS and Epileptiform Discharges, GMFS: grouped multifocal spikes, EE: epileptic encephalopathy<br \/>\nBASED Score Rules: Apply score 3-5 to the most epileptic 5 min epoch; if no score reached, apply score 0-2 to<br \/>\nthe remainder of the study<br \/>\n\u22653 Spike Foci Rules:<\/p>\n<ol>\n<li>May be at least one from each hemisphere OR all from one hemisphere (may include midline)<\/li>\n<li>If \u22653 spike foci in entire study but no 3 spike foci within 5 min, and no channel with abnormal high amplitude, BASED score is 2 + uncommon multifocal spikes<\/li>\n<\/ol>\n<p>Spike Burden Rules:<\/p>\n<ol>\n<li>\u00a0% one second bins that include 1 or more spikes in the most epileptic 5 min epoch<\/li>\n<li>Calculate \u226550% \u22653 spike foci by determining if 10 or more 15 s pages in a 5 min epoch include \u22658\/15 one second bins with a spike<\/li>\n<\/ol>\n<p>Amplitude Rules:<\/p>\n<ol>\n<li>Peak-to-peak amplitude on a longitudinal bipolar montage, refers to background waves and excludes 1) the slow wave of a preceding spike and the field of these waves in other channels, 2) hypnagogic patterns, and 3) arousal rhythms<\/li>\n<li>Waves must be common: present at least once in 10 or more 15 s pages in a 5 min epoch<\/li>\n<li>Abnormal high amplitude:<br \/>\na. \u2265200 mv: Fp1-F7, F7-T3, Fp1-F3, F3-C3, C3-P3, Fp2-F4, F4-C4, C4-P4, C4-P4, Fp2-F8, F8-T4<br \/>\nb. \u2265300 mv: T3-T5, T4-T6<br \/>\nc. Excluded: Fz-Cz, Cz-Pz, T5-O1, P3-O1, P4-O2, T6-O2<\/li>\n<\/ol>\n<p>Grouped Multifocal Spikes Definition:<\/p>\n<ol>\n<li>At least 2 different spike foci in each hemisphere within a fairly well delineated group (may include midline)<\/li>\n<li>For hemispheric grouping, at least 3 different spike foci in one hemisphere (may include midline) within a fairly well delineated group<\/li>\n<\/ol>\n<p>Paroxysmal Voltage Attenuation Definition:<\/p>\n<ol>\n<li>Definite sudden change from ongoing background activities appearing as a relative attenuation most often lasting one second (but may last several seconds), often occurring after an epileptic discharge, and usually diffuse (but may be present in only one hemisphere)<\/li>\n<\/ol>\n<p>Remission Rules:<\/p>\n<ol>\n<li>Pretreatment score of 4 or 5, must improve to \u22643<\/li>\n<li>Pretreatment score of 3, must improve to \u22642<\/li>\n<\/ol>\n<p>&nbsp;<\/p>\n<p><strong>Guidance for the diagnosis of West syndrome<\/strong><\/p>\n<table style=\"border-collapse: collapse; width: 100%;\" border=\"1\">\n<tbody>\n<tr>\n<td style=\"width: 23.3211%;\">Required Features:<\/td>\n<td style=\"width: 38.0952%;\">Required 1 of:<\/td>\n<td style=\"width: 38.5836%;\">Exclusionary Features:<\/td>\n<\/tr>\n<tr>\n<td style=\"width: 23.3211%;\" rowspan=\"2\">\n<ul>\n<li><span style=\"font-family: inherit; font-size: inherit;\">Infantile Spasms<\/span><\/li>\n<li>Age 2 months to 2 years<\/li>\n<\/ul>\n<\/td>\n<td style=\"width: 38.0952%;\" rowspan=\"2\">\n<ul>\n<li><span style=\"font-family: inherit; font-size: inherit;\">Developmental abnormalities with definitively epileptic EEG<\/span><\/li>\n<li>No developmental abnormalities but with Probable or Definite EE<\/li>\n<\/ul>\n<\/td>\n<td style=\"width: 38.5836%;\" rowspan=\"2\">Presence of alternative age-dependent DEE such as:<\/p>\n<ul>\n<li>Early Infantile DEE<\/li>\n<li>Late Infantile DEE<\/li>\n<li>Lennox-Gastaut syndrome<\/li>\n<\/ul>\n<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<p>EE, epileptic encephalopathy; DEE, developmental and epileptic encephalopathy<\/p>\n<p>&nbsp;<\/p>\n<p><strong>References:<\/strong><\/p>\n<ol>\n<li>Mytinger JR. Definitions and Diagnostic Criteria for Infantile Spasms and West Syndrome &#8211; Historical Perspectives and Practical Considerations. Semin Pediatr Neurol. 2021 Jul;38:100893. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/34183140\/\" target=\"_blank\" rel=\"noopener\">[Medline]<\/a><\/li>\n<li>Chopra SS. Infantile Spasms and West Syndrome &#8211; A Clinician&#8217;s Perspective. Indian J Pediatr. 2020 Dec;87(12):1040-1046. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/32557136\/\" target=\"_blank\" rel=\"noopener\">[Medline]<\/a><\/li>\n<\/ol>\n<p>&nbsp;<\/p>\n<p>Created Jan 26, 2022.<\/p>\n\n<div style=\"font-size: 0px; height: 0px; line-height: 0px; margin: 0; padding: 0; clear: both;\"><\/div>","protected":false},"excerpt":{"rendered":"<p>Sorry, this entry is only available in Espa\u00f1ol.<\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_lmt_disableupdate":"no","_lmt_disable":"no","_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"footnotes":""},"categories":[441],"tags":[16,15,14,13,2474,2472,2475,5,2473,4,653],"class_list":["post-9641","post","type-post","status-publish","format-standard","hentry","category-pediatrics","tag-criteria","tag-criterios","tag-diagnostic","tag-diagnostico","tag-espasmos","tag-infantile","tag-infantiles","tag-sindrome","tag-spasms","tag-syndrome","tag-west"],"modified_by":"Guillermo Firman","_links":{"self":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/9641","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/comments?post=9641"}],"version-history":[{"count":12,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/9641\/revisions"}],"predecessor-version":[{"id":9663,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/9641\/revisions\/9663"}],"wp:attachment":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/media?parent=9641"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/categories?post=9641"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/tags?post=9641"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}