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- Title
Progressive striatal necrosis associated with anti-NMDA receptor antibodies.
- Authors
Tzoulis, Charalampos; Vedeler, Christian; Haugen, Mette; Storstein, Anette; Gia Tuong Tran; Gjerde, Ivar Otto; Biermann, Martin; Schwarzlmüller, Thomas; Bindoff, Laurence A
- 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. 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. 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.
- Subjects
IMMUNOGLOBULINS; DRUG receptors; EXTRAPYRAMIDAL disorders; DYSTONIA; NECROSIS
- Publication
BMC Neurology, 2013, Vol 13, Issue 1, p1
- ISSN
1471-2377
- Publication type
Article
- DOI
10.1186/1471-2377-13-55