A biopsy of the recently discovered pelvic mass disclosed a poorly differentiated adenocarcinoma of Mullerian origin

A biopsy of the recently discovered pelvic mass disclosed a poorly differentiated adenocarcinoma of Mullerian origin. believed to occur when systemic malignancies express proteins, called onconeural antigens, that are made only in neurons (3). Although no studies to date have conclusively proven that paraneoplastic antibodies are pathogenic, they are still useful markers of autoimmunity that categorize the PNS subtypes (1). We present a case of paraneoplastic cerebellar ataxia (PCA) associated with anti-Yo antibody (anti-Purkinje cell antibody) due to an underlying gynecologic malignancy. CASE DESCRIPTION A 67-year-old white woman presented with a 3-day history of headache, severe imbalance, nausea, and binocular double vision UNC-2025 limiting her mobility. Two weeks earlier, UNC-2025 she had been diagnosed with a left 4 cm adnexal mass following a 6-week history of pelvic pain. She was known to have hypertension, coronary artery disease, aortic stenosis (mild), left ear cholesteatoma, and mastoiditis. Neurological examination revealed intact language and cognition, significant bilateral diplopia and nystagmus worse to the right, bilateral dysmetria worse on the left, and broad-based gait ataxia. No focal weakness or change in muscle tone was noted. Deep tendon reflexes were brisk at all sites, and the plantar reflexes were downgoing on the right and upgoing on the left. Routine laboratory work was unremarkable. A biopsy of the recently discovered pelvic mass disclosed a poorly differentiated adenocarcinoma of Mullerian origin. A computed tomography (CT) scan of the chest, abdomen, and pelvis revealed mediastinal lymphadenopathy. The patient was started on chemotherapy with carboplatin and paclitaxel. A serum paraneoplastic panel was positive for anti-Yo antibody with a high titer em (Table 1) /em . Cerebrospinal fluid (CSF) examination showed lymphocytic pleocytosis, high protein, and positive anti-Yo antibodies em (Table 2) /em . Magnetic resonance imaging (MRI) of the head on admission showed no evidence of metastatic disease. MRI done 10 days after admission, however, showed enhancement along the bilateral cerebellar sulci. A diagnosis of anti-YoCassociated PCA was made, and the patient was started on high-dose methylprednisolone and intravenous immunoglobulin (IVIG) at 2 g/kg given over a period of 3 days along with her chemotherapy drugs. Her headache resolved, and her diplopia and dysmetria were slightly UNC-2025 improved. The patient was transferred to a rehabilitation facility for continuation of palliative chemotherapy. Table 1. Laboratory blood work thead th align=”left” rowspan=”1″ colspan=”1″ Result /th th align=”center” rowspan=”1″ colspan=”1″ Tests /th /thead Paraneoplastic antibodies panel: positive resultsPurkinje cell cytoplasmic antibody, type 1: 1:61,440Paraneoplastic antibodies panel: negative resultsANNA-1, ANNA-2, ANNA-3, AGNA-1, PCA-2, PCA-Tr, amphiphysin antibody, CRMP-5, striational antibodies, P/Q-type calcium channel antibody, AChRAb, ganglionic AChR autoantibody, VGKC, NMDA-R AbOther pertinent positive resultsEBV IgGOther pertinent negative Rabbit Polyclonal to TAF15 resultsSerum Lyme, syphilis, CMV IgM, EBV IgM, HSV-1 PCR, HSV-2 PCR, fungal cultures, bacterial cultures Open in a separate window AchRAb indicates anti-acetylcholine receptor antibody; AGNA, anti-glial/neuronal nuclear antibody; ANNA, anti-neuronal nuclear antibody; CMV, cytomegalovirus; CRMP, collapsin response mediator protein; EBV, Epstein-Barr virus; UNC-2025 HSV, herpes simplex virus; Ig, immunoglobulin; NMDA-R Ab, anti-N-methyl D-aspartate receptor antibody; PCA, Purkinje cell cytoplasmic antibody; VGKC, voltage-gated potassium channel. Table 2. Cerebrospinal fluid examination thead th rowspan=”1″ colspan=”1″ /th th align=”center” rowspan=”1″ colspan=”1″ Analysis 1 /th th align=”center” rowspan=”1″ colspan=”1″ Analysis 2 (9 days later) /th /thead Protein (mg/dL)5988Glucose (mg/dL)4462White blood cell (cells/L): br / lymphocytes, neutrophils, monocytes41 (86%, 3%, 11%)45 (92%, 6%, 2%)Red blood cell (cells/L)06650Gram stain and culturesNegativeNegativeIgG synthesis, cryptococcal Ag, Histoplasma AgNegativeCytology for malignant cellsNegativeNegativeVenereal Disease Research br / LaboratoryNegativeParaneoplastic antibodiesPCA-1 positive, 1:4096 Open in a separate window IgG indicates immunoglobulin G; PCA-1, Purkinje cell cytoplasmic antibody type 1. DISCUSSION PNS are rare neurological syndromes that are not the consequence of direct invasion, metastasis, or side effects of cancer treatment (2). PNS can affect any level of the nervous system em (Table 3) /em . They can affect a single site, as in Lambert-Eaton myasthenic syndrome, a single UNC-2025 cell type, as in PCA, or multiple areas, as in paraneoplastic encephalomyelitis. The association of many PNS with specific antibodies that recognize.