title: Progressive striatal necrosis associated with anti-NMDA receptor antibodies.
abstract: 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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Neurology, Haukeland University Hospital, Bergen 5021, Norway. chtzoulis@yahoo.com
authors.forename: Charalampos
authors.initials: C
authors.lastname: Tzoulis
authors.forename: Christian
authors.initials: C
authors.lastname: Vedeler
authors.forename: Mette
authors.initials: M
authors.lastname: Haugen
authors.forename: Anette
authors.initials: A
authors.lastname: Storstein
authors.forename: Gia Tuong
authors.initials: GT
authors.lastname: Tran
authors.forename: Ivar Otto
authors.initials: IO
authors.lastname: Gjerde
authors.forename: Martin
authors.initials: M
authors.lastname: Biermann
authors.forename: Thomas
authors.initials: T
authors.lastname: Schwarzlmüller
authors.forename: Laurence A
authors.initials: LA
authors.lastname: Bindoff
mesh_terms: D000293:Adolescent; D000906:Antibodies / Q000378:metabolism*; D001927:Brain Diseases / Q000188:drug therapy / Q000473:pathology* / Q000503:physiopathology; D003342:Corpus Striatum / Q000000981:diagnostic imaging / Q000473:pathology*; D018450:Disease Progression; D019788:Fluorodeoxyglucose F18; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D008297:Male; D009336:Necrosis / Q000000981:diagnostic imaging / Q000473:pathology; D049268:Positron-Emission Tomography; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology*; D014057:Tomography, X-Ray Computed
chemical_list: D000906:Antibodies; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D016194:Receptors, N-Methyl-D-Aspartate; D019788:Fluorodeoxyglucose F18