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.

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.

• Authors: Charalampos Tzoulis, Christian Vedeler, Mette Haugen, Anette Storstein, Gia Tuong Tran, Ivar Otto Gjerde, Martin Biermann, Thomas Schwarzlmüller, Laurence A Bindoff
• Journal: BMC neurology (BMC Neurol) Vol. 13 Pg. 55 (May 31 2013) ISSN: 1471-2377 [Electronic] England
• PMID: 23725534 (Publication Type: Case Reports, Journal Article, Video-Audio Media)

Chemical References

• Antibodies
• Immunoglobulins, Intravenous
• Immunologic Factors
• Receptors, N-Methyl-D-Aspartate
• Fluorodeoxyglucose F18

Topics

• Adolescent
• Antibodies (metabolism)
• Brain Diseases (drug therapy, pathology, physiopathology)
• Corpus Striatum (diagnostic imaging, pathology)
• Disease Progression
• Fluorodeoxyglucose F18
• Humans
• Immunoglobulins, Intravenous (therapeutic use)
• Immunologic Factors (therapeutic use)
• Male
• Necrosis (diagnostic imaging, pathology)
• Positron-Emission Tomography
• Receptors, N-Methyl-D-Aspartate (immunology)
• Tomography, X-Ray Computed