{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T20:06:16Z","timestamp":1792267576317,"version":"4.4.0"},"reference-count":18,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2024,3,27]],"date-time":"2024-03-27T00:00:00Z","timestamp":1711497600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2024,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Pilomotor seizures are strongly associated with autoimmune encephalitis (AE), particularly anti\u2010LGI1 encephalitis. The carbonic anhydrase inhibitor acetazolamide may have special efficacy for treating AE\u2010associated pilomotor seizures. Six patients with AE (five anti\u2010LGI1, one seronegative) and temporal lobe pilomotor seizures (five with seizures inducible by hyperventilation) were treated with acetazolamide, administered in a cycling (2\u2010days\u2010ON, 4\u2010days\u2010OFF) regimen to offset tolerance. Seizures were assessed during epilepsy monitoring unit (EMU) recordings in four inpatients (one of whom also maintained an outpatient seizure diary chronicling 1203 seizures over 1079\u2009days); two outpatients self\u2010reported seizure frequencies. The extended diary revealed an inverse correlation between acetazolamide and proportion of seizures\/day: 6%, 2% (days 1, 2 ON); 3%, 13%, 31%, 45% (days 1, 2, 3, 4 OFF). This patient later developed focal status epilepticus upon wean of antiseizure medications during a seropositive AE relapse that was remarkably aborted with acetazolamide monotherapy. The other three EMU patients averaged .56 seizures\/day ON, and 3.81 seizures\/day OFF (<jats:italic>p<\/jats:italic>\u2009=\u2009.004). The two outpatients reported seizure reductions from 3\u20135\/day to 2\/week, and 15\u201320\/day to none, respectively, after initiation of cycling acetazolamide. Likely related to cerebral CO<jats:sub>2<\/jats:sub>\/pH sensitivity, acetazolamide can be unusually effective in controlling pilomotor seizures in AE, chronically or in acute settings.<\/jats:p>","DOI":"10.1111\/epi.17962","type":"journal-article","created":{"date-parts":[[2024,3,27]],"date-time":"2024-03-27T14:40:13Z","timestamp":1711550413000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Acetazolamide as an effective treatment for pilomotor seizures in autoimmune encephalitis"],"prefix":"10.1111","volume":"65","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1301-3690","authenticated-orcid":false,"given":"Kia","family":"Gilani","sequence":"first","affiliation":[{"name":"Division of Neurology, Department of Medicine, Krembil Brain Institute University Health Network, University of Toronto  Toronto Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Apameh","family":"Tarazi","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Medicine, Krembil Brain Institute University Health Network, University of Toronto  Toronto Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"Wennberg","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Medicine, Krembil Brain Institute University Health Network, University of Toronto  Toronto Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,3,27]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.seizure.2014.04.013"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.16571"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/epi4.12619"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1177\/106002809603000515"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuropharm.2015.04.015"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.1.4968.650"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1528-1157.2003.53002.x"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.17684"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.695"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.51566"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0195094"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000012465"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.seizure.2022.03.025"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40265-022-01826-9"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2003.023333"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.25666"},{"key":"e_1_2_9_18_1","first-page":"3144","article-title":"LGI1 antibodies alter Kv1.1 and AMPA receptors changing synaptic excitability, plasticity and memory","volume":"141","author":"Petit\u2010Pedrol M","year":"2018","journal-title":"Brain"},{"key":"e_1_2_9_19_1","first-page":"317","article-title":"New insights into the pathogenesis and therapeutics of episodic ataxia type 1","volume":"9","author":"D'Adamo MC","year":"2015","journal-title":"Front Cell Neurosci"}],"container-title":["Epilepsia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/epi.17962","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,20]],"date-time":"2024-05-20T14:54:19Z","timestamp":1716216859000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/epi.17962"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,3,27]]},"references-count":18,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2024,5]]}},"alternative-id":["10.1111\/epi.17962"],"URL":"https:\/\/doi.org\/10.1111\/epi.17962","archive":["Portico"],"relation":{},"ISSN":["0013-9580","1528-1167"],"issn-type":[{"value":"0013-9580","type":"print"},{"value":"1528-1167","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,3,27]]},"assertion":[{"value":"2024-01-02","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-03-11","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-03-27","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}