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dc.contributor.authorTzoulis, Charalamposeng
dc.contributor.authorVedeler, Christian A.eng
dc.contributor.authorHaugen, Metteeng
dc.contributor.authorStorstein, Anetteeng
dc.contributor.authorTran, Gia Tuong Thieng
dc.contributor.authorGjerde, Ivar Ottoeng
dc.contributor.authorBiermann, Martineng
dc.contributor.authorSchwarzlmüller, Thomaseng
dc.contributor.authorBindoff, Laurenceeng
dc.date.accessioned2014-04-25T08:17:43Z
dc.date.available2014-04-25T08:17:43Z
dc.date.issued2013-05-31eng
dc.identifier.issn1471-2377eng
dc.identifier.urihttp://hdl.handle.net/1956/7910
dc.description.abstract<p>Background: We report a case of childhood onset, generalized dystonia due to slowly progressive bilateral striatal necrosis associated with anti-N-methyl-D-aspartate receptor (NMDAR) antibodies. This clinical phenotype has not been previously associated with NMDA receptor autoimmunity.</p><p>Case presentation: An eighteen year old man presented with a history of childhood-onset, progressive generalized dystonia. Clinical examination revealed a pure generalized dystonia with no cognitive or other neurological findings. Magnetic resonance imaging showed bilateral high T2 signal striatal lesions, which were slowly progressive over a period of nine years. New parts of the lesion showed restricted water diffusion suggesting cytotoxic oedema. Positron emission tomography of the brain showed frontal hypermetabolism and cerebellar hypometabolism. Antibodies against the NR1 subunit of the NMDA receptor were detected in the patient’s serum and cerebrospinal fluid. There was no neoplasia or preceding infection or vaccination.</p><p>Conclusion: This is the first report of chronic progressive bilateral striatal necrosis associated with anti-NMDAR antibodies. Our findings expand the clinical spectrum of disease associated with anti-NMDAR antibodies and suggest that these should be included in the work-up of dystonia with striatal necrosis.</p>en_US
dc.language.isoengeng
dc.publisherBioMed Centraleng
dc.rightsAttribution CC BYeng
dc.rights.urihttp://creativecommons.org/licenses/by/2.0eng
dc.subjectDystoniaeng
dc.subjectNMDAeng
dc.subjectMRIeng
dc.subjectPETeng
dc.subjectEncephalitiseng
dc.titleProgressive striatal necrosis associated with anti-NMDA receptor antibodieseng
dc.typeJournal articleeng
dc.date.updated2013-08-23T08:48:56Z
dc.description.versionPeer Reviewed
dc.rights.holderCopyright 2013 Tzoulis et al.; licensee BioMed Central Ltd.
dc.rights.holderCharalampos Tzoulis et al.; licensee BioMed Central Ltd.eng
dc.type.versionpublishedVersioneng
bora.peerreviewedPeer reviewedeng
bora.journalTitleBMC Neurologyeng
bibo.volume13eng
bibo.number55eng
bibo.doihttp://dx.doi.org/10.1186/1471-2377-13-55eng
bora.accessRightsinfo:eu-repo/semantics/openAccesseng
dc.identifier.cristinID1046085eng
dc.identifier.doi10.1186/1471-2377-13-55


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Except where otherwise noted, this item's license is described as Attribution CC BY