| paraneoplastic cerebellar degeneration (PCD) is a rare nonmetastatic neurological complication in cancer patients. Anti-Yo is one of the anti-onconeural antibodies found in PCD patients. It is believed that anti-Yo occurs almost always in women and is most likely associated with gynecologic or breast cancers, although exceptions exist. Here we report a PCD patient with ovarian cancer having high-titer anti-Yo. The acute onset of her PCD symptoms mimicked that of a stroke. Her ovarian cancer tissue contained abundant plasma cells and lymphocytes. After a thorough review of the literature, we propose a schematic hypothesis for the autoimmune pathogenesis of PCD. Despite anecdotal case reports of neurological improvement with different combinations of treatment, including IVIg, there is still no definitely effective treatment for PCD. Further research on the pathogenesis of PCD may lead to more effective therapies. ( info) |
| paraneoplastic cerebellar degeneration (PCD) is a rare neurological complication in adults with extracerebral neoplasms. It is characterized by a diffuse cerebellar dysfunction, usually leading to severe neurological sequelae. In childhood, this complication is extremely rare. We report on PCD as primary manifestation of hodgkin disease (HD) in a thirteen-year old boy. On magnetic resonance imaging, irreversible atrophy of the cerebellum developed within three months. Antibodies against purkinje cells were detectable at diagnosis and normalised after successful treatment of the lymphoma. Cerebellar symptoms, however, only partially resolved. The necessity of a search for a malignant tumour is emphasised in the presence of an otherwise unexplained, subacutely developing, diffuse cerebellar dysfunction. ( info) |
| An 11-year-old boy admitted with a right cervical mass was found to have Hodgkin disease. On admission, he also had right Homer syndrome and severe cerebellar ataxia. Cranial MRI revealed marked cerebellar atrophy. He was treated with chemotherapy consisting of doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD), in addition to radiotherapy. Three months after initiation of therapy, he had a partial remission of tumor. Neurologic symptoms improved dramatically after chemotherapy started. hodgkin disease should be included in the differential diagnosis of children with cerebellar findings and horner syndrome. ( info) |
4/39. Anti-Ri-associated paraneoplastic cerebellar degeneration without opsoclonus in a patient with a neuroendocrine carcinoma of the stomach. We report a case of a 63-year-old man suffering from anti-Ri-associated paraneoplastic cerebellar degeneration (PCD) with gastric cancer. The neurologic presentation was limited to severe cerebellar ataxia without opsoclonus. The gastric cancer was composed of both poorly differentiated adenocarcinoma and neuro-endocrine carcinoma. The patient's serum reacted with recombinant Ri antigen and the neuroendocrine tumor component. It is thus considered that PCD without opsoclonus in the present case was related to the gastric neuroendocrine tumor and anti-Ri antibody. ( info) |
| paraneoplastic cerebellar degeneration (PCD) is the most frequently seen paraneoplastic syndrome affecting the brain. PCD is most commonly associated with cancers of the ovary, breast, and lung. The anti-Purkinje cell antibodies (anti-Yo) that specifically damage the purkinje cells of the cerebellum are found in the patient's serum and cerebrospinal fluid. The typical presentation of PCD includes limb and truncal ataxia, often along with dysarthria. This report describes the case of a 47-year-old woman without significant medical history who developed new onset of unsteady gait, headache, and vertigo. The imaging studies suggested rhombencephalitis. The patient initially responded to corticosteroid treatment. Unfortunately, her gait ataxia worsened and she developed dysarthria, neither of which responded to increasing dosages of corticosteroids. Extensive imaging studies showed no evidence of tumor, but the patient was found to have positive anti-Yo antibodies and elevated cancer antigen 125 (CA-125). pathology results from exploratory laparotomy revealed stage III C adenocarcinoma of the ovary. This case demonstrates that PCD may be the presenting symptom of an occult malignancy. The pathogenesis, diagnosis, and treatment of PCD, and its rehabilitation implications, are reviewed. ( info) |
6/39. Successful immunosuppressant therapy of severe progressive cerebellar degeneration and sensory neuropathy: a case report. A 56 year old woman had a 19 month history of a severe subacute progressive cerebellar degeneration, peripheral sensory neuropathy, and urinary incontinence. She was confined to a wheelchair, needed assistance with eating, and her speech was almost unintelligible. No underlying cancer was found despite repeated investigations, and no autoantibodies were demonstrated. She received a 3-month course of intensive immunosuppressant therapy with intravenous immunoglobulin 400 mg/kg per day for 5 days every month, oral cyclophosphamide 50 mg twice or three times a day to maintain the total lymphocyte count between 500 and 750/mm(3), and prednisone 60 mg per day. She experienced dramatic subjective and objective improvement. The dysarthria and the upper extremity dysmetria disappeared, and she regained the ability to write and cook. The lower extremity ataxia improved and she became able to walk with a cane. urinary incontinence disappeared. A trial of intensive immunosuppressant treatment is worth considering in a patient with a clinical syndrome resembling paraneoplastic disorders, even if an underlying neoplasm and autoantibodies are not demonstrated. ( info) |
| BACKGROUND: A paraneoplastic syndrome-a rare, indirect system disturbance located distant from a neoplasm-has been reported in association with ovarian cancer. CASE: Two paraneoplastic syndromes-nephrotic syndrome and paraneoplastic cerebellar degeneration (PCD)-developed in a patient who had no symptoms from ovarian cancer. She presented with the nephrotic syndrome. Evaluation for metastasis, including computed tomography (CT) scan and ultrasonography, showed a slightly enlarged right ovary and a 2-cm lower paraaortic lymph node on the right side. A 5-cm ovarian mass and multiple paraaortic lymph nodes were excised, and six cycles of carboplatin and paclitaxel were given. nephrotic syndrome symptoms had greatly subsided at 6 weeks after surgery. At 7 months after surgery, symptoms of PCD developed yet CT, CA-125 levels, and physical examination showed no evidence of ovarian cancer. CONCLUSION: To our knowledge, development of two paraneoplastic syndromes in one patient with ovarian cancer has not been reported. nephrotic syndrome resolved with treatment of the neoplasm whereas PCD did not. ( info) |
8/39. Anti-Yo antibodies and cerebellar degeneration in a man with adenocarcinoma of the esophagus. serum antibodies to the Yo antigen are usually associated with paraneoplastic cerebellar degeneration arising in female patients with gynecological or breast malignancy and are rarely associated with other tumors. We report a male patient who presented with paraneoplastic cerebellar degeneration and anti-Yo antibodies following removal of an esophageal adenocarcinoma. This is only the third report of anti-Yo antibodies occurring in a male patient. The Yo antigen was expressed by the esophageal tumor but not in a frontal lobe cerebral metastasis identified at postmortem. Interestingly, CD8 T-cell infiltration was also found in the tumor, but not in the metastasis, consistent with down-regulation of Yo expression by the tumor cells leading to evasion from immune-mediated tumor surveillance. ( info) |
| paraneoplastic cerebellar degeneration (PCD) with anti-Yo antibodies is almost always associated with ovarian and breast cancer. We describe a man with anti-Yo-positive PCD and gastric adenocarcinoma. The tumor cells were labeled with anti-Yo antibodies by immunohistochemistry. High serum titers of anti-Yo antibodies were found before surgery and decreased 6 months after resection of the tumor. The expression of Yo antigens by the tumor and the decrease in anti-Yo antibody titers after its removal suggest that the immune response against the purkinje cells of the cerebellum was triggered by the tumor. ( info) |
| The objective of this paper is to describe an 81-year-old woman with subacute cerebellar degeneration due to fallopian tube adenocarcinoma. serum anti-Yo antibodies were used to screen for pelvic malignancy. Their presence led to a meticulous search, which included bilateral salpingoophorectomy. Subsequently an occult fallopian tube adenocarcinoma was discovered. This case report highlights the diagnostic value of antineuroneal antibodies in females with subacute neurologic impairment in the form of paraneoplastic syndrome. ( info) |