Progressive striatal necrosis associated with anti-NMDA receptor antibodies

摘要: 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 NMDA autoimmunity. Case presentation: An eighteen year old man presented history childhood-onset, dystonia. Clinical examination revealed pure no cognitive or other neurological findings. Magnetic resonance imaging showed high T2 signal lesions, which were over period nine years. New parts the lesion restricted water diffusion suggesting cytotoxic oedema. Positron emission tomography brain frontal hypermetabolism and cerebellar hypometabolism. Antibodies against NR1 subunit detected in patient’s serum cerebrospinal fluid. There was neoplasia preceding infection vaccination. Conclusion: is first chronic anti-NMDAR Our findings expand spectrum disease antibodies suggest that these should be included work-up necrosis.