Anti-NMDAR encephalitis and other glutamate and GABA receptor antibody encephalopathies
SourceHandbook of Clinical Neurology, 133, (2016), pp. 199-217
Article / Letter to editor
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Handbook of Clinical Neurology
SubjectRadboudumc 3: Disorders of movement DCMN: Donders Center for Medical Neuroscience
Over the last few year, antibodies to various central nervous system receptors, particularly the glutamate and gamma-aminobutyric acid (GABA) receptors, have been found to be associated with autoimmune neurologic disorders. The receptors include the N-methyl-d-aspartate receptor (NMDAR), the alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), the metabotropic glutamate receptors (mGluRs), and GABA type A and B receptors (respectively GABAAR and GABABR). Compared to the previously described paraneoplastic antibodies directed at intracellular targets, the patients with receptor antibodies are often younger, they less frequently have malignancies, and they respond better to immunotherapy. Many of the patients have limbic encephalitis with amnesia, disorientation, seizures, and psychological or psychiatric symptoms, but those with NMDAR antibodies usually develop a more widespread form of encephalitis, often leading to a decrease in consciousness and requirement for long-term intensive care treatment. The autoantibodies bind directly to the synaptic or extrasynaptic receptors on the membrane surface, and have direct effects on signal transduction in central synapses. These conditions are very important to recognize as the symptoms and complications can be fatal when not treated in time, whereas with immunotherapy many patients recover considerably.
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