1/58. 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.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/58. Paraneoplastic painful ulnar neuropathy.A 58-year-old woman developed painful, bilateral ulnar neuropathy in conjunction with small cell lung carcinoma and high serum titer of anti-Hu antibody. An incidental stage I plasma cell dyscrasia, with immunoglobulin g kappa monoclonal protein, was also present. Electropysiological assessment excluded a generalized neuropathy, and nerve biopsy showed marked loss of myelinated and small unmyelinated fibers, without inflammatory changes or amyloid deposition. High titers of circulating anti-Hu antibody can be associated with symptoms resembling a paraneoplastic mononeuropathy.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
3/58. Immunofluorescent analysis of antibodies against neurons in the case of paraneoplastic syndrome.The authors report clinical and neuropathological findings especially immunofluorescent detection of antineuronal antibodies in the case of paraneoplastic syndrome in course of the small-cell lung carcinoma. The clinical symptoms, observed in 48-year-old woman, covered bilateral pyramidal syndrome, cerebellar syndrome, myasthenic syndrome and impairment of the cranial nerves. Neuropathological investigation revealed paraneoplastic encephalopathy in the form of encephalitis. Immunofluorescent analysis showed brightly fluorescent neurons standing out against a dull background.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
4/58. Reversibility of paraneoplastic bilateral diaphragmatic paralysis after nephrectomy for renal cell carcinoma.Bilateral diaphragmatic paralysis is usually caused by anatomic lesions of both phrenic nerves (e.g., after cardiothoracic surgery), generalized neurologic diseases (e.g., primary motor neuron disease, amyotrophic lateral sclerosis) or is without a known cause (idiopathic). We report a case of a patient with renal cell carcinoma complicated by an isolated bilateral diaphragmatic paralysis without clinical or electromyographic signs of other muscle or nerve involvement. There has been progressive, though till now partial, recovery of his vital capacity rising from 44% to 72% of predicted values, and maximal inspiratory pressures during the two years following the curative resection of his renal cell carcinoma. We believe this is the first report of a paraneoplastic bilateral diaphragmatic paralysis with actual recovery after tumour therapy.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
5/58. Primary angiitis of the central nervous system as a first presentation in Hodgkin's disease: a case report and review of the literature.OBJECTIVE AND IMPORTANCE: Granulomatous angiitis of the central nervous system is a rare cause of neurological deterioration. It is often diagnosed posthumously, and a high index of suspicion is necessary to make the correct diagnosis on a timely basis. CLINICAL PRESENTATION: A 27-year-old woman presented to the emergency room with complaints of worsening headache, nausea, and vomiting for 10 days, which were preceded by swelling of her tongue. At the examination, she had mild ocular tenderness, but no cranial nerve abnormalities. Radiographic examination revealed a right temporal lobe area with edema, and mild contrast enhancement was noted on computed tomography and magnetic resonance imaging. A similar but smaller region was present in the left frontal lobe. INTERVENTION: Stereotactic biopsy of the left temporal lobe revealed granulomatous angiitis. Further workup revealed Hodgkin's disease in the mediastinum. dexamethasone as well as chemotherapy for Hodgkin's disease was initiated. The patient's symptoms resolved, and she returned to work with her disease in remission. CONCLUSION: Previous reports of central nervous system angiitis have shown an association with sjogren's syndrome, herpes zoster infection, human immunodeficiency virus, and Hodgkin's disease. A review of the literature revealed a total of 12 patients with central nervous system angiitis and Hodgkin's disease. As a group, these patients had very poor outcomes. However, of six patients who presented with central nervous system angiitis and concurrent Hodgkin's disease and who underwent aggressive treatment for both conditions, three had a full recovery, two had a partial recovery, and one died.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
6/58. classification, clinical manifestations, and immunopathological mechanisms of the epithelial variant of paraneoplastic autoimmune multiorgan syndrome: a reappraisal of paraneoplastic pemphigus.BACKGROUND: Recent studies suggest that paraneoplastic pemphigus (PNP) is a heterogeneous autoimmune syndrome involving several internal organs and that the pathophysiological mechanisms mediating cutaneous, mucosal, and internal lesions are not limited to autoantibodies targeting adhesion molecules. OBJECTIVE: To classify the diverse mucocutaneous and respiratory presentations of PNP and characterize the effectors of humoral and cellular autoimmunity mediating epithelial tissue damage. methods: We examined 3 patients manifesting the lichen planus pemphigoideslike subtype of PNP. A combination of standard immunohistochemical techniques, enzyme-linked immunosorbent assay with desmoglein (DSG) baculoproteins, and an immunoprecipitation assay were used to characterize effectors of humoral and cellular autoimmunity in patients with PNP and in neonatal wild-type and DSG3-knockout mice with PNP phenotype induced by passive transfer of patients' IgGs. RESULTS: In addition to the known "PNP antigenic complex," epithelial targets recognized by PNP antibodies included 240-, 150-, 130-, 95-, 80-, 70-, 66-, and 40/42-kd proteins but excluded DSG1 and DSG3. In addition to skin and the epithelium lining upper digestive and respiratory tract mucosa, deposits of autoantibodies were found in kidney, urinary bladder, and smooth as well as striated muscle. Autoreactive cellular cytotoxicity was mediated by CD8( ) cytotoxic T lymphocytes, CD56( ) natural killer cells, and CD68( ) monocytes/macrophages. Inducible nitric oxide synthase was visualized both in activated effectors of cellular cytotoxicity and their targets. Keratin 14-positive basal epithelial cells sloughed from the large airways and obstructed small airways. CONCLUSIONS: The paraneoplastic disease of epithelial adhesion known as PNP in fact represents only 1 manifestation of a heterogeneous autoimmune syndrome in which patients, in addition to small airway occlusion and deposition of autoantibodies in different organs, may display a spectrum of at least 5 different clinical and immunopathological mucocutaneous variants (ie, pemphiguslike, pemphigoidlike, erythema multiforme-like, graft-vs-host disease-like, and lichen planus-like). We suggest that the more encompassing term "paraneoplastic autoimmune multiorgan syndrome," or PAMS, be applied. The pathophysiological mechanisms of PAMS involve both humoral and cellular autoimmunity responses. Epithelial cell membrane antigens other than DSG1 or DSG3 are targeted by effectors of PAMS autoimmunity. apoptosis of damaged basal cells mediates epithelial clefting, and respiratory failure results possibly from obstruction of small airways with sloughed epithelial cells.- - - - - - - - - - ranking = 1.3622051511268keywords = organ (Clic here for more details about this article) |
7/58. Renal cell carcinoma with skull base metastasis preceded by paraneoplastic signs in a chronic hemodialysis patient.A 59-year-old man who had received chronic hemodialysis developed left occipital pain and hypoglossal nerve palsy. He was diagnosed as having skull base metastasis from renal cell carcinoma related to acquired cystic kidney. Retrospective analysis revealed the patient had had elevated serum c-reactive protein and alkaline phosphatase levels before the symptoms appeared. radiotherapy to the skull base relieved the pain. Finally he died with generalized metastases. serum interleukin-6 levels measured during admission had been elevated, and interleukin-6 mRNA was detected in the autopsy specimen of renal cell carcinoma. interleukin-6 might be involved in the etiology of paraneoplastic signs.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
8/58. Paraneoplastic dysmotility: loss of interstitial cells of cajal.Autoimmune impairment and destruction of the enteric nervous plexus are thought to play a central role in the pathogenesis of paraneoplastic motility disorders. We present a case of a small-cell lung carcinoma-related paraneoplastic motility disorder associated with abnormal interstitial cells of cajal networks. Antibodies against c-Kit and protein gene product 9.5 were used to selectively stain interstitial cells of cajal and the enteric nervous plexus, respectively. A 68-yr-old man presented with anorexia, early satiety, nausea, and weight loss. Investigations revealed gastroparesis, delayed small intestinal transit, and mediastinal lymphadenopathy. The patient was seropositive for type 1 antineuronal nuclear autoantibody and P/Q-type calcium channel antibody. biopsy of mediastinal lymph nodes revealed metastatic small-cell carcinoma cells that were immunoreactive for c-Kit. Immunohistochemical staining of a full-thickness small intestinal biopsy revealed a relatively intact myenteric plexus but a sparse and disorganized interstitial cells of cajal network. The histopathology of this case suggests that interstitial cells of cajal may be a target in the pathogenesis of paraneoplastic motility disorders.- - - - - - - - - - ranking = 0.19460073587526keywords = organ (Clic here for more details about this article) |
9/58. Full-blown graft-versus-host disease presenting with skin manifestations, jaundice and diarrhoea: an unusual paraneoplastic phenomenon of a thymoma.We describe a patient who presented with chronic diarrhoea, skin lesions and jaundice. Based on histopathological examinations of the affected organs combined with the clinical features, it appeared that the patient fulfilled the criteria for graft-versus-host disease (GVHD). GVHD occurs especially after allogeneic stem cell transplantation and sometimes after organ transplantations. However, this patient had never undergone such a procedure. Further examination revealed that the patient also suffered from a thymoma, which was concluded to be the cause of GVHD. Unfortunately, the patient died after resection of the thymoma. This patient is probably the second case with thymoma and full-blown GVHD and shows that GVHD can occur in the absence of a previous transplantation.- - - - - - - - - - ranking = 0.38920147175052keywords = organ (Clic here for more details about this article) |
10/58. facial pain as first manifestation of lung cancer: a case of lung cancer-related cluster headache and a review of the literature.facial pain can, on rare occasions, be the presenting symptom of lung cancer. This report describes a patient with non-metastatic lung cancer, which was associated with attacks of debilitating facial pain, presenting as cluster headache. Moreover, 32 reported cases of lung cancer-related facial pain (including the present one) are reviewed, and their clinical features are summarized. The facial pain is almost always unilateral, and is most commonly localized to the ear, the jaws, and the temporal region. The pain is frequently described as severe and aching, and may be continuous or intermittent. Aggravation and expansion of the pain, digital clubbing, increased erythrocyte sedimentation rate, and hypertrophic osteopathy, may contribute to the diagnosis. Referred pain, due to invasion or compression of the vagus nerve, as well as paraneoplastic syndrome secondary to the production of circulating humoral factors by the malignant tumor cells, is implicated in the pathophysiology of facial pain associated with non-metastatic lung cancer. radiotherapy and tumor resection with vagotomy are very effective in aborting the facial pain. Thus, lung cancer should be included in the differential diagnosis of facial pain that is atypical and/or refractory to treatment.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
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