{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T06:03:05Z","timestamp":1790834585395,"version":"4.1.0"},"reference-count":35,"publisher":"Cambridge University Press (CUP)","issue":"4","license":[{"start":{"date-parts":[[2014,12,2]],"date-time":"2014-12-02T00:00:00Z","timestamp":1417478400000},"content-version":"unspecified","delay-in-days":4049,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Can. j. neurol. sci."],"published-print":{"date-parts":[[2003,11]]},"abstract":"<jats:sec id=\"S0317167100003152_abs1\"><jats:title>Background:<\/jats:title><jats:p>Guillain-Barr\u00c8 syndrome (GBS) classically presents with a subacutely evolving areflexic paralysis, with typical laboratory findings of elevated cerebrospinal fluid protein and abnormal nerve conduction studies. There is now an increasing recognition of GBS variants that differ in clinical presentation, prognosis, electrophysiology and presumed pathogenesis. Fulminant cases of GBS have been reported in which a rapid deterioration evolves to a clinical state resembling \u00ecbrain death\u00ee.<\/jats:p><\/jats:sec><jats:sec id=\"S0317167100003152_abs2\"><jats:title>Methods:<\/jats:title><jats:p>A retrospective analysis of two such cases of fulminant neuropathy are described, that includes the clinical course, electrophysiology and neuropathology where available.<\/jats:p><\/jats:sec><jats:sec id=\"S0317167100003152_abs3\"><jats:title>Results:<\/jats:title><jats:p>We describe two patients that presented with a rapid course of neurological deterioration, lapsing into what resembled a \u00ecclinically brain-dead\u00ee state that was subsequently ascribed to a fulminant polyneuropathy. Investigations (electrophysiological, pathological) and the clinical course suggested an axonal neuropathy.<\/jats:p><\/jats:sec><jats:sec id=\"S0317167100003152_abs4\"><jats:title>Conclusion:<\/jats:title><jats:p>A fulminant neuropathy can result in a clinical state resembling \u00ecbrain death\u00ee through diffuse de-efferentation. Although generally attributed to aggressive demyelination with secondary axonal degeneration, a primary axonopathy can also lead to a similar clinical presentation.<\/jats:p><\/jats:sec>","DOI":"10.1017\/s0317167100003152","type":"journal-article","created":{"date-parts":[[2014,12,12]],"date-time":"2014-12-12T15:35:05Z","timestamp":1418398505000},"page":"397-404","source":"Crossref","is-referenced-by-count":24,"title":["Simulation of Brain Death from Fulminant De-efferentation"],"prefix":"10.1017","volume":"30","author":[{"given":"Yael","family":"Friedman","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liesly","family":"Lee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John R.","family":"Wherrett","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Ashby","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stirling","family":"Carpenter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"56","published-online":{"date-parts":[[2014,12,2]]},"reference":[{"key":"S0317167100003152_ref029","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-4598(199707)20:7<846::AID-MUS9>3.0.CO;2-7"},{"key":"S0317167100003152_ref023","doi-asserted-by":"publisher","DOI":"10.1136\/pgmj.67.785.280"},{"key":"S0317167100003152_ref015","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410330402"},{"key":"S0317167100003152_ref034","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.48.3.695"},{"key":"S0317167100003152_ref012","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410310416"},{"key":"S0317167100003152_ref033","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(93)90324-R"},{"key":"S0317167100003152_ref008","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/109.6.1115"},{"key":"S0317167100003152_ref013","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880170617"},{"key":"S0317167100003152_ref022","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2044.1990.tb14333.x"},{"key":"S0317167100003152_ref020","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.291.6494.535"},{"key":"S0317167100003152_ref011","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.54.9.768"},{"key":"S0317167100003152_ref027","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.52.7.857"},{"key":"S0317167100003152_ref005","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.52.3.635"},{"key":"S0317167100003152_ref010","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/107.1.219"},{"key":"S0317167100003152_ref014","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(91)90606-P"},{"key":"S0317167100003152_ref009","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410090705"},{"key":"S0317167100003152_ref001","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-194902010-00003"},{"key":"S0317167100003152_ref007","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199511233332110"},{"key":"S0317167100003152_ref030","doi-asserted-by":"publisher","DOI":"10.1007\/s001340051213"},{"key":"S0317167100003152_ref028","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-4598(199704)20:4<514::AID-MUS19>3.0.CO;2-Y"},{"key":"S0317167100003152_ref017","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410390105"},{"key":"S0317167100003152_ref021","doi-asserted-by":"publisher","DOI":"10.1016\/0013-4694(87)90161-1"},{"key":"S0317167100003152_ref006","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1007\/BF00812150","article-title":"The Guillain-Barr\u00e9 syndrome: no longer a simple concept","volume":"239","author":"Thomas","year":"1992","journal-title":"J Neurol"},{"key":"S0317167100003152_ref016","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/118.3.577"},{"key":"S0317167100003152_ref026","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.55.11.1079"},{"key":"S0317167100003152_ref031","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-4598(199712)20:12<1595::AID-MUS17>3.0.CO;2-#"},{"key":"S0317167100003152_ref032","doi-asserted-by":"publisher","DOI":"10.1097\/00003246-199711000-00035"},{"key":"S0317167100003152_ref025","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(95)00235-8"},{"key":"S0317167100003152_ref024","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(93)90034-V"},{"key":"S0317167100003152_ref003","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410090704"},{"key":"S0317167100003152_ref035","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.48.3.717"},{"key":"S0317167100003152_ref004","first-page":"243","article-title":"Management and outcome of severe Guillain-Barr\u00e9 syndrome","volume":"88","author":"Ng","year":"1995","journal-title":"QJ Med"},{"key":"S0317167100003152_ref002","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-196905000-00001"},{"key":"S0317167100003152_ref018","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1979.00500370076020"},{"key":"S0317167100003152_ref019","first-page":"150","article-title":"Total locked-in state resembling brain death in polyneuropathy","volume":"16","author":"Kotsoris","year":"1984","journal-title":"Ann Neurol"}],"container-title":["Canadian Journal of Neurological Sciences \/ Journal Canadien des Sciences Neurologiques"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cambridge.org\/core\/services\/aop-cambridge-core\/content\/view\/S0317167100003152","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,7,30]],"date-time":"2023-07-30T16:57:51Z","timestamp":1690736271000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cambridge.org\/core\/product\/identifier\/S0317167100003152\/type\/journal_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2003,11]]},"references-count":35,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2003,11]]}},"alternative-id":["S0317167100003152"],"URL":"https:\/\/doi.org\/10.1017\/s0317167100003152","relation":{},"ISSN":["0317-1671","2057-0155"],"issn-type":[{"value":"0317-1671","type":"print"},{"value":"2057-0155","type":"electronic"}],"subject":[],"published":{"date-parts":[[2003,11]]}}}