1/935. 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. ( info) |
2/935. 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. ( info) |
3/935. 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. ( info) |
4/935. Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma arising from Castleman's tumor. Paraneoplastic pemphigus (PNP) is associated with various forms of underlying neoplasia. We report a patient with PNP associated with an intraabdominal follicular dendritic cell sarcoma that developed from a Castleman's tumor that had preexisted for 40 years. ( info) |
5/935. Pigmented purpura-like eruption as cutaneous sign of mycosis fungoides with autoimmune purpura. We describe the clinical and laboratory findings of a young man with mycosis fungoides. The disease was associated, since the early stages, with autoimmune purpura. Interferon alfa (IFN-alpha) administration improved this patient's condition, both the purpuric eruption and patchy cutaneous lesions, thus suggesting T-cell abnormalities may be responsible for the development of the disease. ( info) |
6/935. Amyopathic dermatomyositis associated with transitional cell carcinoma of the bladder. Transitional cell carcinoma of the bladder is the most common tumor of the urinary tract. However, it has only been reported twice in the literature to be associated with the paraneoplastic syndrome dermatomyositis. We report a case of amyopathic dermatomyositis in a patient whose painless gross hematuria was due to transitional cell carcinoma of the bladder as well as review this association. ( info) |
7/935. 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. ( info) |
8/935. A case of the subacute brainstem encephalitis. A case of brainstem encephalitis of undetermined etiology is reported in 66-year-old woman who had a sudden onset of illness with left abducens palsy, nystagmus and ataxia. The symptoms progressed to complete paralysis of eye movements, dysphagia and left hemiparesis with generalized hyperreflexia. Examination of CSF, CT scan and MRI of the brain were normal. The patient died 4 months after onset of disease. Neuropathologic study disclosed in the brainstem numerous perivascular and nodular inflammatory cell infiltrations composed predominantly of lymphocytes T and B. Most intensive inflammation concerned midbrain and pontine tegmentum and to a lesser degree medulla oblongata, pontine nuclei and cerebellar nuclei. basal ganglia, cerebral and cerebellar cortex were unaffected. Neuropathological finding was reminiscent of brainstem encephalitides related to viral infection or to paraneoplastic syndrome. However, HSV-1, EBV, and CMV antigens were not detected by immunohistochemistry, as well as evidences of malignancy were not present in this case. ( info) |
9/935. A reversible cause of hypercapnic respiratory failure: lower motor neuronopathy associated with renal cell carcinoma. We describe a unique case of a patient with a reversible paraneoplastic motor neuronopathy who presented with hypercapnic respiratory failure. The patient developed progressive respiratory and limb muscle weakness until treated with removal of a renal cell carcinoma, which was followed by a complete resolution of neuromuscular symptoms. The literature of paraneoplastic motor neuronopathies is reviewed, specifically in reference to respiratory failure. ( info) |
10/935. Vulvar paraneoplastic amyloidosis with the appearance of a vulvar carcinoma. Nodular cutaneous amyloidosis of the vulva is a rare phenomenon. We describe a patient with localized nodular lesions on the vulva that mimicked kissing ulcers such as are seen with vulvar carcinoma. These lesions were a result of multiple myeloma with subsequent primary systemic amyloidosis. The patient died of cardiac and renal decompensation 2 months after diagnosis. ( info) |