Paraneoplastic limbic encephalitis in a patient with extragonadal choriocarcinoma--significance of onconeural antibodies.
BACKGROUND
Paraneoplastic limbic or brainstem encephalitis is considered to be an autoimmune-mediated disorder of the nervous system associated with different types of cancer including germ cell tumors.


METHODS
We report on a 31-year-old patient presenting with eye motility dysfunction, dysarthrophonia, lethargy, depression, slow mentation, disorientation, dysgraphia, and retarded motion sequence. Neurologic tests, brain imaging, and blood chemistry tests failed to determine the cause of the symptoms. Further examinations including ultrasound of the abdomen led to the detection of a retroperitoneal mass. The biopsy of this mass showed fractions of a choriocarcinoma. The patient underwent curative chemotherapy, but although the cancer therapy was successful, the neurologic disorders did not improve. Concurrent examination for anti-Ma2 antibodies in the serum was positive and confirmed the paraneoplastic origin of these symptoms.


CONCLUSIONS
Patients with symptoms of limbic or brainstem encephalitis, especially young men, should be tested for anti-Ma2 antibodies in the serum to elucidate their origin. The detection of these antibodies supports the diagnosis of a paraneoplastic syndrome, and may lead to the earlier identification of an otherwise hidden extragonadal germ cell tumor.
Szkandera
Joanna
J
Division of Oncology, Department of Internal Medicine, Medical University of Graz, Austria.
Ploner
Ferdin
F
Bauernhofer
Thomas
T
Kasparek
Anne-Katrin
AK
Payer
Franz
F
Balic
Marija
M
Knechtel
Gudrun
G
Gerger
Armin
A
Gallè
Günter
G
Samonigg
Hellmut
H
Hofmann
Günter
G
D000328:Adult; D000912:Antibodies, Neoplasm / Q000276:immunology*; D000951:Antigens, Neoplasm / Q000276:immunology*; D003937:Diagnosis, Differential; D006801:Humans; D020363:Limbic Encephalitis / Q000175:diagnosis* / Q000276:immunology*; D008297:Male; D018309:Neoplasms, Gonadal Tissue; D009419:Nerve Tissue Proteins / Q000276:immunology*; D010534:Peritoneal Neoplasms / Q000175:diagnosis* / Q000276:immunology*
D000912:Antibodies, Neoplasm; D000951:Antigens, Neoplasm; C119249:Ma2 antigen; D009419:Nerve Tissue Proteins