Cervical spine pseudogout with myelopathy and Charcot joints.

Myelopathy due to calcium pyrophosphate dihydrate (CPPD) crystals in the cervical spine is rare. Neuropathic-like joints are also uncommon manifestations of CPPD crystal disease. We describe a 79-year-old woman with multiple neuropathic joints containing CPPD crystals. She also had a large mass containing CPPD crystals impinging on the cervical spinal cord, causing a myelopathy. She appears to be the first patient with this combination of unusual clinical features with CPPD disease.

Reversible cardiogenic shock in an angry woman--case report and review of the literature.

A previously healthy woman presented with ischemic cardiac pain and ST elevation suggestive of acute myocardial infarction following a 45 min argument. Despite receiving tissue plasminogen activator, she developed cardiogenic shock and objective evidence of recurrent ischemia, with only a small creatine kinase rise. Angiography revealed the unexpected findings of normal coronary anatomy and akinesis of the distal two-thirds of the left ventricle. Apart from an iliac vein thrombosis, the remainder of her course was characterized by dramatic recovery of cardiac function. The differential diagnosis of myocardial infarction with angiographically normal coronary arteries is discussed, with emphasis on aspects relevant to this case. The presence of high titre anticentromere antibodies, anticardiolipin antibodies, protein S deficiency and supportive physical findings, suggested the diagnosis of concurrent antiphospholipid antibody syndrome (with secondary acquired protein S deficiency) and CREST syndrome. The pathogenesis likely involved an interaction between stress, vasospasm, and thrombosis.

Superoxide production from cytokine-treated adherent rheumatoid neutrophils.

We have compared superoxide (O2-) production from cytokine-treated rheumatoid arthritis (RA) vs. control neutrophils (PMN). Exposure of adherent peripheral blood PMN to cytokines known to be present in RA joints (IL-1 beta, TNF-alpha, GM-CSF) resulted in enhanced O2- production from both RA and controls. With few exceptions, we did not find significant differences in enhanced O2- production, between RA and controls. By enhancing O2- production from PMN adherent to articular cartilage, cytokines may influence the potential for joint damage in RA.

Necrotizing mesenteric vasculitis after longstanding cutaneous polyarteritis nodosa.

We describe a 21-year-old woman with cutaneous polyarteritis nodosa (PAN) who developed necrotizing mesenteric vasculitis 6 years after the onset of skin disease. Repeated investigation during that 6-year interval failed to reveal any evidence of systemic PAN. We were unable to wean the patient from prednisone or completely control her skin disease with topical and systemic therapy, including supersaturated potassium iodide, dapsone, azathioprine, methotrexate or plasmapheresis before the development of the mesenteric vasculitis, which responded to cyclophosphamide and prednisone. Development of visceral vasculitis after long-standing cutaneous PAN has very rarely been reported.

Relapsing polychondritis in pregnancy.

A 25-year-old patient presented at 14 weeks of her 3rd pregnancy with chondritis affecting the cartilaginous portion of her right ear. The clinical syndrome of recurrent chondritis, scleritis, iritis and arthritis supported a diagnosis of relapsing polychondritis. Fetal and maternal outcomes were favorable despite steroid dependent active disease during the period of fetal organogenesis. Chondritis was limited to the 3rd pregnancy, ocular inflammation occurring in the 2nd and 3rd pregnancies. A 4th pregnancy was uneventful. This may represent the first case of relapsing polychondritis in pregnancy.

Cytolocalization artifacts with immunofluorescent probes.

Formaldehyde fixation, nonionic detergent extraction, and ligand binding are commonly used in conjunction with immunofluorescence microscopy to visualize antigens and lectin-reactive molecules in cytoskeletal preparations. These procedures have the potential to produce serious artifacts in cytolocalization studies, as is shown in the present investigation of wheat-germ agglutinin (WGA) binding and localization in BeWo choriocarcinoma cells. Formaldehyde fixation of intact cells reduced the binding of 125I-labeled WGA by 30% and altered the pattern of staining with fluorescein isothyocyanate (FITC)-WGA. Except for perinuclear sites which were brightly stained, the binding of FITC-WGA to other cell surface regions was significantly decreased. Nonionic detergent extractions had two different effects on lectin binding activity depending on whether or not the cells had been pretreated with lectin. In lectin-pretreated cells, 50% of bound lectin was solubilized by detergent but all of the surface binding sites appeared to be retained in active form in the detergent-insoluble residue. Nuclear-cytoskeletal monolayers prepared from cells that were not lectin pretreated lost considerable binding activity, however. These results suggest that a number of erroneous conclusions can be drawn from studies on cytoskeletal associations with membrane components using immunofluorescence microscopy on fixed and detergent-extracted cells.

Changes in cell-substratum adhesion and nuclear-cytoskeletal anchorage during cytodifferentiation of BeWo choriocarcinoma cells.

Changes in the substratum anchorage of cells and nuclei were examined during methotrexate (MTX)-induced cytodifferentiation of BeWo human choriocarcinoma cells. During this process cytotrophoblast-like cells (CTLs) transform into giant mono- and multinucleated syncytiotrophoblast-like cells (STLs). Cells treated with MTX for 24 h exhibited significantly faster rates of substratum detachment by EDTA, trypsin-EDTA, EDTA-glycine, and DMSO than did uninduced controls. The decrease in cell-substratum adhesiveness occurred prior to the onset of morphological transformation. By 48 h, when morphological transformation was first observed, there had occurred a marked change in nuclear-cytoskeletal anchorage to the substratum, as evidenced by a difference in sensitivity of Triton-extracted STL and CTL monolayers to detachment by KI. STL monolayers were completely detached within 5 min of exposure to 0.3 M KI, while CTL monolayers remained firmly attached to the substratum for at least 3 h. KI-extracted residues were examined by electron microscopy and found to consist of nuclear shells attached to intermediate filaments. When cytoskeletal residues and KI-extracted proteins of STL and CTL cells were compared by two-dimensional polyacrylamide gel electrophoresis (PAGE), qualitative and quantitative differences were seen in a number of minor components. Thus the sensitivity of STL nuclear-cytoskeletal monolayers to removal by KI, an effective actin depolymerizing agent, may involve changes in the organization, stability, or interactions of actin with other components of the cytoskeletal framework.

Congenital dyserythropoietic anaemia (CDA) with severe gout, rare Kell phenotype and erythrocyte, granulocyte and platelet membrane reduplication: a new variant of CDA type II.

A 43-year-old man with lifelong anaemia showed features which indicate him to have a previously undescribed variant of congenital dyserythropoietic anaemia (CDA), type II. The main clinical features--of which the first two are unique or very unusual in CDA--have been severe tophaceous gout, massive splenomegaly, gall stones mecessitating cholecystectomy and haemosiderosis affecting the liver and probably the heart. At age 41 he sustained a spontaneous retinal detachment. In the peripheral blood there were large numbers of nucleated red blood cells and marked macrocytosis; otherwise the picture was typical of CDA type II. The bone marrow contained many bi- and multi-nucleated erythrocyte precursors. There were increased levels of a number of red cell enzymes and a slightly raised level of HbF. Uncharacteristically, the red cells failed to lyse with acidified normal serum. The cells were strongly agglutinated by anti-i and were of the rare Kpb-negative phenotype. Plasma lipid analysis showed very low levels of cholesterol and vitamin E. Lipid peroxidation was markedly increased. Ultrastructural studies showed reduplication of the erythrocyte, granulocyte, and platelet cell membranes.

Ultrastructural studies of an unusual variant of congenital dyserythropoietic anaemia type II.

Electron microscopy of a new variant of congenital dyserythropoietic anaemia type II has yielded several unexpected results. The remarkable features include the relatively high percentage of erythrocytes with peripheral cisternae, the involvement of all bone marrow precursor cells, and the presence of ultrastructural defects in the granulocytes and platelets and on the cell surface of the erythrocytes. Experiments with vinblastine showed that this drug was capable of producing (in vitro) strikingly similar ultrastructural defects in healthy erythrocytes.

Scanning electron microscopy of blood cells.

Scanning electron microscopy (SEM) is an invaluable tool for studying the surface morphology of isolated blood cells. We have used a simple preparative technique for SEM to study the erythrocytes from a case of Congenital Dyserythropoietic Anaemia type II and the leucocytes from healthy individuals or patients with leukaemia or lymphoma. This rapid and inexpensive technique for fixation and dehydration of blood cells was found to be equally reliable for obtaining micrographs of healthy or diseased leucocytes or erythrocytes.

A simple method for processing erythrocytes for scanning electron microscopy.

A simple method for preparing erythrocytes for scanning electron microscopy by sequential fixation with glutaraldehyde and dehydration in a graded series of alcohols is presented. The method will allow visualization of membrane defects not seen under the light microscope and is therefore suitable for routine processing of erythrocytes for diagnosis of pathologic states.