Open Access Case report

Progressive striatal necrosis associated with anti-NMDA receptor antibodies

Charalampos Tzoulis12*, Christian Vedeler12, Mette Haugen1, Anette Storstein12, Gia Tuong Tran2, Ivar Otto Gjerde1, Martin Biermann34, Thomas Schwarzlmüller3 and Laurence A Bindoff12

Author Affiliations

1 Department of Neurology, Haukeland University Hospital, Bergen 5021, Norway

2 Department of Clinical Medicine, University of Bergen, Bergen, Norway

3 Center for Nuclear Medicine/PET, Department of Radiology, Haukeland University Hospital, Bergen, Norway

4 Institute for Surgical Sciences, University of Bergen, Bergen, Norway

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BMC Neurology 2013, 13:55  doi:10.1186/1471-2377-13-55

Published: 31 May 2013

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.

Keywords:
Dystonia; NMDA; MRI; PET; Encephalitis