title: [Antibody-associated diseases of the gray matter of the CNS: diagnosis and treatment].
abstract: In the past 10 years, the diagnosis and understanding of immune-mediated diseases of the gray matter of the CNS have greatly advanced with the discovery of autoantibodies in serum and CSF of affected patients. The newly described antibodies against neuronal surface antigens, i.e., receptors, channels, and associated proteins, seem to have a direct pathogenic effect on CNS neurons. Fortunately, there is a beneficial effect of immunotherapy in many patients. The diagnosis of autoantibody-associated disease of the CNS gray matter requires the combination of a typical clinical syndrome or a characteristic paraclinical finding (MRI, CSF, histopathology) with the detection of a specific antibody. In the following, an overview will first be given of the syndromes typically associated with autoantibodies. Thereafter, the diagnosis of these syndromes is discussed, and finally, preliminary treatment recommendations are given.
authors.affiliation: Epilepsie-Zentrum Bielefeld-Bethel, Krankenhaus Mara, Maraweg 21, 33617 Bielefeld, Deutschland. christian.bien@mara.de
authors.forename: C G
authors.initials: CG
authors.lastname: Bien
mesh_terms: D000328:Adult; D000465:Algorithms; D000918:Antibody Specificity / Q000276:immunology; D000954:Antigens, Surface / Q000276:immunology; D001323:Autoantibodies / Q000378:metabolism*; D001921:Brain / Q000276:immunology / Q000473:pathology; D001927:Brain Diseases / Q000235:genetics / Q000276:immunology / Q000473:pathology / Q000628:therapy; D002648:Child; D020278:Demyelinating Autoimmune Diseases, CNS / Q000235:genetics / Q000276:immunology* / Q000473:pathology / Q000628:therapy; D003937:Diagnosis, Differential; D004827:Epilepsy / Q000235:genetics / Q000276:immunology / Q000473:pathology / Q000628:therapy; D019084:Fluorescent Antibody Technique, Indirect / Q000379:methods; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins; D020363:Limbic Encephalitis / Q000235:genetics / Q000276:immunology / Q000473:pathology / Q000628:therapy; D008279:Magnetic Resonance Imaging; D009474:Neurons / Q000276:immunology / Q000473:pathology; D040081:Protein Array Analysis / Q000379:methods; D011506:Proteins / Q000276:immunology; D013577:Syndrome
chemical_list: D000954:Antigens, Surface; D001323:Autoantibodies; D047908:Intracellular Signaling Peptides and Proteins; C116499:LGI1 protein, human; D011506:Proteins