Cases reported "Paraneoplastic Syndromes"

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1/361. Cancer-associated retinopathy in a patient with non-small-cell lung carcinoma.

    Cancer-associated retinopathy (CAR) is a paraneoplastic syndrome most often associated with small-cell lung carcinoma (SCLC), and it has been reported in patients with other malignancies. Antibodies against recoverin, a 23-kDa protein, have been found in patients with CAR suggesting an autoimmune phenomenon. Herein is the first report of a patient with non-small-cell lung cancer (NSCLC) in whom anti-recoverin antibodies were detected in the serum. Steroid therapy, chemotherapy, and radiation therapy did not help the patient's vision. Progressive loss of vision in patients with lung cancer should, potentially, be tested for CAR.
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ranking = 1
keywords = cancer
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2/361. Antiamphiphysin antibodies are associated with various paraneoplastic neurological syndromes and tumors.

    BACKGROUND: Antiamphiphysin antibodies react with a 128-kd protein found in synaptic vesicles.They were first described in patients with paraneoplastic stiff-man syndrome and breast cancer, but studies suggest that they can also occur in patients with other tumors and neurological disorders. OBJECTIVE: To determine if antiamphiphysin antibodies are associated with various paraneoplastic neurological syndromes and tumors. patients AND methods: Of 2800 serum samples tested by routine immunohistochemical procedures on sections of paraformaldehyde-fixed rat brain for the detection of autoantibodies associated with paraneoplastic neurological syndromes, 5 were selected because of labeling suggestive of antiamphiphysin antibodies and subsequently confirmed by the results of Western blot analysis using recombinant amphiphysin protein. Controls consisted of 40 patients with various nonparaneoplastic neurological diseases; 101 patients with cancer but without paraneoplastic neurological syndrome; 9 patients with small cell lung cancer, anti-Hu antibodies, and paraneoplastic neurological syndrome; 3 patients with M2-type antimitochondrial antibodies but no neurological disorder; and 30 normal subjects. RESULTS: Of the 5 patients with antiamphiphysin antibodies, patient 1 had sensory neuronopathy, encephalomyelitis, and breast cancer; patient 2 had limbic encephalitis, and small cell lung cancer was detected in the mediastinum after 24 months of follow-up; patient 3 had encephalomyelitis and ovarian carcinoma; and patients 4 and 5 had lambert-eaton myasthenic syndrome and small cell lung cancer (patient 4 subsequently developed cerebellar degeneration). None of the 5 had stiffness. Two patients (Nos. 2 and 4) had antimitochondrial antibodies. The two patients (Nos. 4 and 5) with lambert-eaton myasthenic syndrome had antibodies directed against the voltage-gated calcium channel, and patient 2 subsequently developed anti-Hu antibodies. In the controls, antiamphiphysin antibodies were detected by Western blot analysis in 3 of 8 patients with anti-Hu antibodies, but in none of the other groups. CONCLUSIONS: These data indicate that antiamphiphysin antibodies are not specific for one type of tumor or one neurological syndrome and can be associated with other neural and nonneural antibodies. The simultaneous association of several antibodies in some patients suggests multimodal autoantibody production.
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ranking = 3
keywords = cancer
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3/361. Ovarian cancer presenting as leukocytoclastic vasculitis.

    We describe a 53-year-old woman with a 4-month history of palpable purpuric papules on the upper and lower extremities. biopsy of the skin lesions revealed leukocytoclastic vasculitis. Although she denied any systemic symptoms, urinalysis demonstrated hematuria and proteinuria. Although the patient's skin lesions responded to prednisone, her urinalysis did not improve. A 10-cm complex mass involving the left ovary and adnexa was incidentally discovered on renal ultrasound. serum CA-125, an ovarian cancer marker, was elevated. laparotomy revealed ovarian carcinoma confined to the left ovary. After the cancer was resected, the patient's urinalysis slowly improved. Leukocytoclastic vasculitis (LCV) is infrequently associated with underlying malignancy and only rarely with solid tumors. We postulate that the patient's vasculitis represented a paraneoplastic phenomenon that allowed a diagnosis of asymptomatic ovarian carcinoma. To our knowledge, this is the first report of LCV occurring as the presenting sign of ovarian cancer.
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ranking = 3.5
keywords = cancer
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4/361. Neuro-ophthalmologic manifestations of a paraneoplastic syndrome and testicular carcinoma.

    The authors report two patients with testicular cancer who exhibited supranuclear gaze disorders as a manifestation of a paraneoplastic brainstem encephalomyelitis. In the first patient, neuro-ophthalmic dysfunction was accompanied by a prominent limbic encephalitis whereas in the second patient, an unusual, mixed pendular and jerk nystagmus was manifested. neuroimaging revealed an enhancing hypothalamic mass in the first patient and was negative in the second. blood from both patients contained an antibody previously reported in a patient with limbic encephalitis and testicular cancer.
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ranking = 1
keywords = cancer
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5/361. Treatment of paraneoplastic visual loss with intravenous immunoglobulin: report of 3 cases.

    BACKGROUND: Paraneoplastic visual loss is an autoimmune disorder believed to be caused by the remote effects of cancer on the retina (cancer-associated retinopathy [CAR]) or optic nerve. Both disorders may result in rapid and complete blindness. Spontaneous recovery of vision has not been reported. The serum of patients with CAR contains autoantibodies against recoverin, enolase, or unidentified retinal proteins. autopsy examination results of eyes of blind patients with CAR show complete absence of the retinal neurons involved in phototransduction. Corticosteroids and plasmapheresis are the only treatment options previously described. OBJECTIVE: To treat paraneoplastic visual loss. DESIGN AND methods: Three patients with metastatic cancer developed rapidly progressive loss of vision. The first patient had visual acuity of hand movements in each eye before intravenous immunoglobulin treatment. The second patient had visual acuity of light perception in both eyes. The third patient's visual acuity was 20/400 OD and 20/20 OS. Diagnostic tests included magnetic resonance imaging of the head and cytologic examination of the cerebrospinal fluid to exclude metastasis as the cause of visual loss and then an electroretinogram and serum tests for autoantibodies against retinal antigens to confirm the clinical diagnosis of CAR. patients 1 and 2 were treated with intravenous immunoglobulin (400 mg/kg per day) for 5 days; however, patient 3 received only a single dose due to adverse effects consisting of shortness of breath and itching. RESULTS: Within 24 hours of taking the first dose of intravenous immunoglobulin, the visual acuity of patient 1 improved from hand movements only in both eyes to 20/50 OD and 20/200 OS. After the third day of treatment, visual acuity in the left eye further improved to 20/40. Even with the improved acuity, Goldmann visual field perimetry results showed poor responses in both eyes. However, 2 weeks later there was marked visual field improvement, and visual acuity was maintained at 20/50 OD and 20/40 OS. Patient 2 had no improvements and continued to have light perception in both eyes. Patient 3 had improvements in visual field defects but remained 20/400 OD and 20/20 OS. CONCLUSION: Intravenous immunoglobulin may be another treatment option offered to patients with paraneoplastic visual loss in addition to corticosteroids or plasmapheresis because a review of the medical literature has shown no spontaneous improvements of visual function without treatment.
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ranking = 1.5
keywords = cancer
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6/361. Paraneoplastic pemphigus with fatal pulmonary involvement in a woman with a mesenteric Castleman tumour.

    A 42-year-old woman presented with oral and labial erosions, conjunctivitis, facial rash and lichenoid erythematous papules on the trunk. Paraneoplastic pemphigus (PNP) was suspected, and a search for a neoplasm revealed an intra-abdominal Castleman tumour sized 7 x 5 x 6 cm. After removal of the Castleman tumour, the skin and mucosal inflammation gradually subsided over the next 12 months. However, due to irreversible pulmonary involvement the patient died of intractable respiratory distress 2 years after the onset of the disease. Systemic corticosteroids, azathioprine, cyclophosphamide, high-dose intravenous immunoglobulins and thalidomide were ineffective. The diagnosis of PNP was confirmed by keratinocyte antigen immunoprecipitation with the patient's serum.
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ranking = 0.10433193455955
keywords = neoplasm
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7/361. dermatomyositis in association with transitional cell carcinoma of the bladder.

    We report a case of dermatomyositis occurring in association with transitional cell carcinoma of the bladder. The case illustrates the importance of a thorough search for neoplasms in elderly patients with dermatomyositis and is a reminder that bladder cancer may be a rare cause of dermatomyositis. The case also shows that successful treatment of an underlying tumour may lead to resolution of paraneoplastic dermatomyositis, and relapse of cutaneous and muscle symptoms and signs may indicate recurrence of tumour.
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ranking = 0.60433193455955
keywords = cancer, neoplasm
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8/361. Immunoablative high-dose cyclophosphamide without stem cell rescue in paraneoplastic pemphigus: report of a case and review of this new therapy for severe autoimmune disease.

    Paraneoplastic pemphigus (PNP) is a refractory and life-threatening autoimmune mucocutaneous disease. We have recently reported the effectiveness and safety of ablative intravenous cyclophosphamide (200 mg/kg daily over 4 days) without stem cell rescue in patients with refractory autoimmune diseases including systemic lupus erythematosus, autoimmune cytopenias, chronic inflammatory demyelinating polyneuropathy, and aplastic anemia. We report chronic lymphocytic leukemia-associated PNP in a patient who presented with extensive and debilitating painful oral ulcerations and received ablative therapy. The patient tolerated the regimen well and showed a slow but sustained improvement despite persistence of the underlying neoplasm. Eighteen months after therapy, the oral ulcerations were almost completely healed and the circulating autoantibodies became negative. Currently, the patient remains on cyclosporine and a low dose of prednisone. This provides further evidence for the efficacy and safety of this regimen in the management of severe autoimmune diseases including PNP.
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ranking = 0.10433193455955
keywords = neoplasm
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9/361. Serous adenocarcinoma of the uterus presenting as paraneoplastic cerebellar degeneration.

    paraneoplastic cerebellar degeneration is a rare complication of cancer and is most frequently associated with lung, ovary, and breast cancers as well as Hodgkins lymphoma. A 74-year-old female with a past history of breast cancer presented with vomiting, ataxia, slurred speech, and dizziness. Her serum chemistry, thyroid and liver function tests, acetylcholine antibodies, serum cortisol, CT, and MRI imaging were all normal. serum testing for anti-YO antibodies was positive. Further evaluation including CT of the abdomen and pelvis revealed endometrial thickening. Subsequently, an endometrial biopsy showed a poorly differentiated serous adenocarcinoma. Surgical staging was consistent with a stage IIIc serous adenocarcinoma of the uterus. The risk factors, symptoms, signs, differential diagnosis, and clinical and antibody associations of the paraneoplastic cerebellar degeneration syndrome are reviewed. In addition, an efficient approach to the diagnostic evaluation of such patients is proposed.
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ranking = 1.5
keywords = cancer
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10/361. association of anti-Yo (type I) antibody with paraneoplastic cerebellar degeneration in the setting of transitional cell carcinoma of the bladder: detection of Yo antigen in tumor tissue and fall in antibody titers following tumor removal.

    Anti-Yo (type I) autoantibodies reactive with Purkinje cell cytoplasmic antigens of 34 and 62 kd are found in the serum and cerebrospinal fluid of patients with paraneoplastic cerebellar degeneration associated with cancer of the ovary, uterus, adnexa, or breast. Anti-Yo antibody response is rarely associated with other tumors. Here, we present a patient who developed paraneoplastic cerebellar degeneration and anti-Yo antibody response in association with transitional cell carcinoma of the bladder. The presence of anti-Yo antibodies was confirmed by immunofluorescence assay and by Western blot analysis against both Purkinje cell lysates and the CDR62 fusion protein. Yo antigen was demonstrated in sections of the patient's tumor. Antibody titers fell after tumor removal. Transitional cell carcinoma should be considered in patients presenting with subacute cerebellar degeneration and anti-Yo antibody response in whom ovarian, adnexal, uterine, or breast cancer cannot be detected.
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ranking = 1
keywords = cancer
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