Ma-2 Paraneoplastic Encephalitis in the Presence of Bilateral Testicular Cancer: Diagnostic and Therapeutic Approach

Testicular Germ Cell Tumors

“…Neurological symptoms precede the detection of the tumor in the majority of patients, and about a third of them respond to appropriate treatment of the tumor [264]. Frequently, tumors associated with anti-Ma2 antibodies are small, or have undergone regression, as it is believe that the antibodies are a reflection of an effective immune response to the tumor [265][266][267]. It is still debated whether blind orchiectomy should be performed in symptomatic patients with no clinically documented tumor, as frequently an occult neoplasm may be found [264][265][266].…”

“…Frequently, tumors associated with anti-Ma2 antibodies are small, or have undergone regression, as it is believe that the antibodies are a reflection of an effective immune response to the tumor [265][266][267]. It is still debated whether blind orchiectomy should be performed in symptomatic patients with no clinically documented tumor, as frequently an occult neoplasm may be found [264][265][266]. Before orchiectomy, clinical exclusion of an extragonadal tumor needs to be performed [268].…”

“…A variety of neurological paraneoplastic syndromes have been described in patients with testicular GCT, the most common one being Ma2 antibody-mediated limbic encephalitis [262][263][264][265][266]. Patients develop a constellation of symptoms that include short-term memory disturbance, epileptic seizures, acute confusional syndrome, personality change, hallucinations, depression, and cognition disturbances.…”

Neurologic disorders associated with disease of the ovaries and testis

“…Approximately 10% of all nonmetastatsic neurologic complications in patients with cancer are paraneoplastic (Tenner and Einhorn, 2009). In two-third of cases, the neurologic syndrome precedes the diagnosis of ovarian or testicular tumor (Table 52.1).…”

“…These paraneoplastic neurologic syndromes are frequently the initial symptom of these tumors and may represent a formidable diagnostic and treatment challenge The first reported paraneoplastic syndrome due to ovarian cancer was a progressive cerebellar degeneration associated with the anti-Yo tumor marker, often reported among women in the sixth decade and associated with epithelial carcinoma of the ovaries (Greenlee and Brashear, 1983). Subsequently, the anti-Ma-2 serum marker was identified in patients with testicular germ cell tumors and three clinical phenotypes were recognized: limbic encephalitis, hypothalamic hypersomnia (narcolepsy/cataplexy), and a slowly progressive upper brainstem ophthalmoplegia (Voltz et al, 1999;Dalmau et al, 2004;Tenner and Einhorn, 2009). More recently, an anti-N-methyl-D-aspartate (NMDA) receptor encephalitis associated with ovarian teratoma was described (Dalmau et al, 2007;Dalmau et al, 2008;Florance et al, 2009;Tuzun et al, 2009;Lancaster et al, 2010).…”