| paraneoplastic cerebellar degeneration (PCD) is a type of paraneoplastic syndrome that primarily affects women with gynecological cancers. patients typically experience pancerebellar symptoms, including gait ataxia, dysarthria, nystagmus, and truncal and appendicular ataxia. We present the case of a 50-year-old woman with PCD and presumed ovarian cancer who initially complained of ataxia and dysarthria. PCD was diagnosed on the basis of her symptoms, diagnostic imaging, and laboratory work. PCD symptoms may precede the diagnosis of malignancy by months or years. early diagnosis and treatment of these syndromes, including rehabilitation, may result in improvements in quality of life for this population of patients. ( info) |
| paraneoplastic cerebellar degeneration with anti-Yo antibodies is a rare but disabling neurodegenerative disease that may point to an occult ovarian cancer. Symptoms usually accompanying paraneoplastic cerebellar degeneration include truncal and limb ataxia, dysarthria, dysphagia, nystagmus, vertigo, and diplopia. The pathogenesis of paraneoplastic neurological syndromes is unknown. Treatment results of the neurological symptoms are disappointing. The present case illustrates how neurological symptoms pointed to an occult ovarian cancer. ( info) |
| IMPLICATIONS: We report an unusual case in which a patient with paraneoplastic subacute cerebellar degeneration (a brain disorder resulting from antibody production by a tumor located outside the skull) developed lambert-eaton myasthenic syndrome (antibody-mediated skeletal muscle weakness) that was not apparent until she underwent surgery. Failure to recognize this disease process can cause life-threatening respiratory distress. ( info) |
14/39. Anti-Yo antibody-positive cerebellar degeneration associated with endometrial carcinoma: case report and review of the literature. paraneoplastic cerebellar degeneration (PCD) is a rare, severely debilitating disease, often with a rapid onset and progression, which predate the diagnosis of malignancy. Despite characteristic features, diagnosis is commonly difficult and successful therapy, which relies on early instigation of treatment, is rare. Here we present a patient in whom anti-Yo antibody-positive PCD was associated with endometrial carcinoma and an extensive review of the literature outlining the clinical features, pathogenesis and treatment of PCD. ( info) |
15/39. A case of paraneoplastic syndrome accompanied by two types of cancer. A case of paraneoplastic syndrome accompanied by two types of cancer is reported. The patient was a 62 year old man who progressively developed cerebellar ataxia, especially an abnormal gait. The anti-Hu antibody titre was high. A small tumour was detected in the middle lobe of the right lung and was surgically treated. The histology was adenocarcinoma. After lobectomy, however, the ataxia deteriorated, and plasma exchange, 250 ml/kg/day, was conducted for 6 days. After plasma exchange, the anti-Hu antibody titre decreased and the ataxia temporarily ceased to progress. A week after the last plasma exchange, a mass appeared in the anterior cervical region and rapidly increased in size. The biopsy of the neck tumour disclosed a small cell carcinoma. Five months later small cell carcinoma appeared in the left lung. This case shows the importance of searching for small cell carcinoma when anti-Hu antibodies are detected. It is assumed that plasma exchange removed not only a pathogenic factor of ataxia but also a factor which inhibited the growth of the small cell carcinoma. It is recommended that plasmapheresis should be performed with caution in paraneoplastic syndrome when the origin of a tumour is obscure. ( info) |
| A patient presented with symptoms of cerebellar degeneration and nephrotic syndrome. A work-up at that time failed to reveal an underlying disease; however, 20 months later Hodgkin's disease was diagnosed. Hodgkin's lymphadenopathy developed 2 wk after prednisone therapy for the nephrotic syndrome had been discontinued. Systemic polychemotherapy resulted in complete remission of both Hodgkin's disease and nephrotic syndrome, while the neurological deficit persisted. patients with unexplained cerebellar degeneration and/or nephrotic syndrome demand extensive evaluation for the presence of Hodgkin's disease, and steroid therapy may delay diagnosis. ( info) |
| paraneoplastic cerebellar degeneration is a neurologic syndrome associated with carcinoma. Medical management is limited to treatment of the underlying malignancy with excision, plasmapharesis, immunosuppression, or chemotherapy. We report on the case of a woman with paraneoplastic cerebellar degeneration who showed improvements in functional mobility after 3 wk of comprehensive inpatient rehabilitation. This case demonstrates the functional improvements of one patient with paraneoplastic cerebellar degeneration after intensive rehabilitation and describes the specific treatment interventions utilized. ( info) |
18/39. Atypical paraneoplastic syndrome associated with anti-Yo antibodies. OBJECTIVE: Polyneuropathy, myopathy and spasticity have not been described as a manifestation of a neurologic paraneoplastic syndrome (NPS) associated with anti-Yo antibodies (anti-Yo). CASE history: The patient is a 60-year-old woman with a history of ovarectomy, salpingectomy, hysterectomy and omentectomy because of ovarian cancer with peritoneal carcinosis. From May to September 1999, she received chemotherapy with carboplatin and docetaxel. In June 1999, weaknesses of the lower limbs began to appear. Neurologic investigation revealed bilateral ptosis with right-sided predominance, exaggerated deep tendon reflexes, discrete distal weakness, wasting of the upper limbs and diffuse weakness of the lower limbs. She had slight CK elevation, elevated lactate dehydrogenase and aldolase levels. Testing for anti-neuronal antibodies revealed high serum titers of antibodies against the cytoplasm of purkinje cells, confirmed as anti-Yo by immunoblot with recombinant proteins. CSF investigations showed 12/3 cells and positive oligoclonal bands. MRI of the brain showed bilateral, old ischemic basal ganglia lesions exclusively. Visually evoked potentials gave prolonged P100 latencies bilaterally. Nerve conduction studies and electromyography revealed motor polyneuropathy of the lower limbs. Muscle biopsy from the right anterior tibial muscle showed non-specific myopathic features. CONCLUSION: Polyneuropathy, myopathy and tetraspasticity may be the exclusive manifestations of an atypical NPS associated with anti-Yo. Anti-Yo may persist for years without relapse of the primary tumor. ( info) |
| paraneoplastic cerebellar degeneration (PCD) is a debilitating neuro-degenerative disease associated with antibodies directed against the purkinje cells of the cerebellum. Treatment using chemotherapy or other treatment of the primary tumor to various immunologically directed therapies has been attempted but outcomes have been poor. We discuss a patient with ovarian carcinoma and PCD seen in our institution who showed a marked beneficial response to intravenous immunoglobulin (IVIG) and methylprednisolone. A medline search from 1966-2002 produced fifteen cases of PCD confirmed by antibody testing that were treated with IVIG, either alone, or with a combination of other therapies. The clinical characteristics and treatment responses of these patients are analyzed in this review. Most patients that were treated with IVIG and had what was defined as a good response were treated within one month of symptoms. patients treated between one month and three months often had stable disease and patients treated after three months of symptoms usually had a poor outcome. Early treatment with sufficiently high doses of IVIG seems to provide a better chance of treatment success. The additional benefit of early high dose intravenous methylprednisolone is unclear. Due to the devastating nature of the disease, a trial of IVIG and steroids is warranted as early as possible in a dose of 2g/kg to any patient with a clinical picture of PCD and positive antibodies. ( info) |
20/39. Cerebellous metastases in patients with uterine cervical cancer. Two cases reports and review of the literature. brain metastases from cervical cancer are extremely rare. We report on two patients who developed cerebellous metastases following uterine cervical cancer. The interval between diagnosis of the primary cancer and diagnosis of brain metastasis was 8 months. The main complaint was symptoms of increased intracranial pressure and cerebellous syndrome. Surgical excision of the brain lesion followed by radiation therapy was performed in the first case. The second patient received palliative radiation therapy. The first patient died 8 months after diagnosis. The second patient is alive 2 months after diagnosis. ( info) |