Ophelia syndrome limbic encephalitis

Paraneoplastic limbic encephalitis ple in the hl setting is also known as the ophelia syndrome and was first described by carr in his. Ophelia syndrome is an exceedingly rare paraneoplastic neurological syndrome characterized by hodgkin lymphoma and limbic. The diagnostic criteria of paraneoplastic limbic encephalitis formulated by paraneoplastic neurological syndromes euronetwork 3 include. Graus f, delattre jy, antoine jc, dalmau j, giometto b, 4.

Ophelia syndrome, a medical condition characterized by hodgkin lymphoma with autoimmune limbic encephalitis, caused by anti metabotropic glutamate receptor 5 antibodies mglur5. Haberlandt e, bast t, ebner a, holthausen h, kluger. Ophelia syndrome is the association of hodgkin lymphoma with an autoimmune limbic encephalitis, as a result of antimetabotropic glutamate receptor 5 antibodies mglur5 1. To report the metabotropic glutamate receptor 5 mglur5 as the autoantigen of antibodies from 2 patients with hodgkin lymphoma hl and limbic encephalopathy ophelia syndrome. Limbic encephalitis, an immunemediated encephalitis, results from inflammation in the medial temporal lobes. Unusual presentation of hodgkin lymphoma with limbic. Autoimmune encephalitis can be divided broadly into two groups, based on whether or not antibodies are. Autoimmune encephalitis update neurooncology oxford academic. Limbic encephalitis as presentation of hodgkin lymphoma in a. Ophelia syndrome, a medical condition characterized by hodgkin lymphoma with autoimmune limbic encephalitis, caused by antimetabotropic glutamate. Autoimmune encephalitis radiology reference article. Recognition of this disorder is important because it can affect young individuals and is reversible. The cardinal sign of limbic encephalitis is a severe impairment of shortterm memory. Antibodies to metabotropic glutamate receptor 5 in the.

Antibodies to mglur5 should be considered in patients with symptoms of limbic encephalitis and hl ophelia syndrome. By sarosh irani, associate professor and consultant neurologist, university of oxford and john radcliffe hospital, oxford, uk. Paraneoplastic encephalitis pe in association with hl was first reported in 1982 by. Ophelia syndrome with metabotropic glutamate receptor 5. Early treatment of the underlying tumor helps in successful neurological recovery. Ophelia syndrome with metabotropic glutamate receptor 5 antibodies in csf. Classic antibodyassociated paraneoplastic syndromes. Paraneoplastic limbic encephalitis ple in the hl setting is also known as the ophelia syndrome and was first described by carr 1 in his teenage daughter. This clinical entity was termed the ophelia syndrome, after shakespeares. Limbic encephalitis associated with ovarian dermoid cyst section. Paraneoplastic limbic encephalitis ophelia syndrome should be considered in all patients with hodgkin lymphoma who presents with mental state changes, since. Mri brain scan showed features suggestive of limbic encephalitis and eeg showed. Autoimmune encephalitis, also known as autoimmune limbic encephalitis, is an antibodymediated brain inflammatory process, typically involving the limbic system, although all parts of the brain can be involved. Presentation of hodgkins lymphoma with ophelia syndrome.