Adenylate kinase 5 autoimmunity in treatment refractory limbic encephalitis.
We report two men with limbic encephalitis (LE) refractory to corticosteroids, IVIg and plasma exchange. Both patients had serum/CSF antibodies that reacted with the cytoplasm of neurons. Probing of a hippocampal cDNA library resulted in the isolation of adenylate kinase 5 (AK5). Patients' antibodies, but not those of 111 controls, recognized AK5-expressing phage plaques. Human AK5-affinity purified antibodies reproduced the neuronal immunolabeling of patients' antibodies, and co-localized with a rabbit AK5 antibody, confirming that the brain autoantigen was AK5. Detection of antibodies to AK5 in LE patients carries a poor prognosis, and suggests the prompt use of aggressive immunosuppression.
Tüzün
Erdem
E
Division of Neuro-oncology, Department of Neurology, Hospital of the University of Pennsylvania, Philadelphia, PA 19104, USA.
Rossi
Jeffrey E
JE
Karner
Steve F
SF
Centurion
Alejandro F
AF
Dalmau
Josep
J
D000263:Adenylate Kinase / Q000097:blood / Q000134:cerebrospinal fluid / Q000276:immunology*; D000368:Aged; D000818:Animals; D015551:Autoimmunity; D015723:Gene Library; D006624:Hippocampus / Q000378:metabolism; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007527:Isoenzymes / Q000097:blood / Q000134:cerebrospinal fluid / Q000276:immunology*; D020363:Limbic Encephalitis / Q000097:blood / Q000134:cerebrospinal fluid / Q000473:pathology / Q000628:therapy*; D008297:Male; D010951:Plasma Exchange / Q000379:methods; D051381:Rats
D016756:Immunoglobulins, Intravenous; D007527:Isoenzymes; D000263:Adenylate Kinase; C118782:adenylate kinase 5