Paraneoplastic Limbic Encephalitis: Pendekatan Diagnosis dan Penatalaksanaan

Setiawan, Michael

Cermin Dunia Kedokteran
Vol 41, No 1 (2014): Neurologi

Paraneoplastic limbic encephalitis (PLE) merupakan entitas yang termasuk dalam paraneoplastic neurological syndromes (PNS), berhubungan dengan beberapa jenis tumor, disebabkan oleh gangguan sistem imun yang dicetuskan oleh ekspresi aberan antigen onkoneural tumor. Dalam beberapa dekade ini sudah diidentifikasi sejumlah antibodi yang berkaitan dengan PLE yaitu antibodi onkoneural klasik terhadap antigen intraseluler, dan antibodi terhadap antigen permukaan sel. Masing-masing antibodi memberikan karakteristik gejala klinis, gambaran magnetic resonance imaging (MRI), dan respon pengobatan yang berbeda. Gambaran klinis khas adalah onset subakut atau akut gangguan memori jangka pendek, gejala psikiatrik, dan kejang. Walaupun spektrum klinis belum sepenuhnya diketahui, sindrom klinis PLE penting dikenali karena dapat memberikan respon terhadap imunoterapi. Secara umum, pasien dengan antibodi terhadap antigen permukaan sel prognosisnya lebih baik karena perjalanan klinisnya cenderung tidak terlalu berat, dan memberikan respons baik dengan terapi imunomodulasi.Paraneoplastic Limbic Encephalitis (PLE) is an entity belonging to Paraneoplastic Neurological Syndromes (PNS), associated with several tumors, caused by immune mediated disorders triggered by aberrant expression of onconeural antigens in the tumor. In the last few decades, 2 groups of antibodies associated with PLE have been identified, classical onconeural antibodies directed against the intracellular antigens, and surface antigen antibodies. Each antibody gives characteristic clinical manifestations, magnetic resonance imaging (MRI) findings, and different response to therapy. Characteristic clinical manifestations are subacute or acute onset of recent memory loss, psychiatric manifestations, and seizures. Although the clinical spectrum has not yet been fully investigated, the clinical importance lies in their good response to immunotherapies. Generally, patients with surface antigen antibodies have more favorable prognosis because the clinical course tends to be less severe, and respond well with immunomodulation treatment.