Antiphospholipid syndrome: a clinical and laboratorial challenge.

Antiphospholipid syndrome (APS) is an acquired autoimmune thrombophilia characterized by the presence of a heterogeneous family of antibodies that bind to plasma proteins with affinity for phospholipid surfaces. The two major protein targets of antiphospholipid antibodies are prothrombin and β2-glycoprotein I (β2GPI). APS leads to aprothrombotic state, and it is characterized by the occurrence of arterial, venous or microvascular thrombosis or recurrent fetal loss.

Adult nemaline myopathy with trabecular muscle fibers.

The term "trabecular myopathy" has been used to designate a syndrome resembling limb-girdle muscular dystrophy in which the predominant pathological feature is an abundance of lobulated or trabecular muscle fibers. However, the validity of this nosological entity has not been verified. Herein we describe a 63-year-old man with a severe, progressive myopathy who exhibited the typical pathological features of both trabecular myopathy and nemaline myopathy in association with a biclonal gammopathy.

Sporadic Creutzfeldt-Jakob disease mimicking variant Creutzfeldt-Jakob disease.

Background: The determination of the form of prion disease and early diagnosis are important for prognostic, public health, and epidemiologic reasons.

Objective: To describe a patient with sporadic Creutzfeldt-Jakob disease (sCJD) who had a clinical history and initial electroencephalogram and magnetic resonance imaging findings consistent with variant CJD (vCJD).

Results: Results of a repeated electroencephalogram were suggestive of sCJD, and a subsequent brain biopsy confirmed this diagnosis.

Pathophysiology of cleft lip muscles following the initial surgical repair.

Muscle biopsy specimens taken from the upper lip and perialar area during the time of secondary lip revision and studied by histochemical techniques demonstrate persistent connective-tissue and muscle abnormalities even at a distance from the cleft margins. Some of these changes are consistent with surgically induced denervation-reinnervation of muscle groups in the surgical field. Increased amounts of connective tissue also were found, most likely secondary to the original deformity and the subsequent surgical procedures.

Effect of retinoids on the proliferation, morphology and expression of glial fibrillary acidic protein of an anaplastic astrocytoma cell line.

We studied the effect of retinoids on the growth and differentiation of a cell line (U 343 MG-A) derived from a human malignant astrocytoma. Cultures treated with all-trans or 13-cis retinoic acid showed a dose-dependent inhibition of proliferation and a marked reduction in the mean cell number at the plateau phase of growth (3.5 x 10(6) vs.

Proliferative characteristics of intracranial and spinal tumors of developmental origin.

The proliferative potential of 17 intracranial and spinal tumors (six craniopharyngiomas, four chordomas, three mature teratomas, one immature teratoma, one embryonal carcinoma, one choriocarcinoma, and one dermoid tumor) was assessed. The patients received a 30-minute to 60-minute infusion of bromodeoxyuridine (BUdR) (200 mg/m2 intravenously [IV]) at the time of surgery but before a biopsy of the tumor was performed, to label cells in the DNA synthesis phase. The labeling index (LI) was calculated by determining the percentage of BUdR-labeled cells.

High energy phosphate metabolism in experimental permanent focal cerebral ischemia: an in vivo 31P magnetic resonance spectroscopy study.

Relative levels of phosphate metabolites in the brain were examined in vivo by 31P magnetic resonance spectroscopy (MRS) in 50 Sprague-Dawley rats before, during, and after induction of focal permanent cerebral ischemia. After acquisition of baseline spectra, rats were subjected to injury within the core of the MR spectrometer, and 31P spectra were collected for 60 min after injury: in 7 rats, permanent, acute focal cerebral ischemia was induced (ischemia group); in 6 rats, mild hypoxia (FiO2 14%) was induced at the time of the ischemic insult and was maintained for 20 min (ischemia-hypoxia group); in 6 rats, mild hypoxia (FiO2 14%) only was induced for 20 min (hypoxia group). Control studies were performed in 25 rats.

Cell kinetics analysis in a case of teratoma of the thoracic spine.

The authors report cell kinetics studies in an infant who had multiple operations for removal of a rare benign thoracic spinal teratoma with retroperitoneal extension. Before the final surgical procedure for recurrent tumor, bromodeoxyuridine (BUdR), 200 mg/sq m, was administered intravenously to label tumor cells in the S (deoxyribonucleic acid (DNA) synthesis) phase of the cell cycle. Histologically, the tumor was a mature teratoma consisting of components derived from all three germ-cell layers.

Foci of MRI signal (pseudo lesions) anterior to the frontal horns: histologic correlations of a normal finding.

Review of all normal magnetic resonance (MR) scans performed over a 12-month period consistently revealed punctate areas of high signal intensity on T2-weighted images in the white matter just anterior and lateral to both frontal horns. Normal anatomic specimens were examined with attention to specific characteristics of this region. Three unique features typify the brain tissues that correspond to the foci of high signal.

Characterization of normal human brain cultures. Evidence for the outgrowth of leptomeningeal cells.

To establish the histogenetic identity of the predominant cell type in monolayer cultures of normal human adult brain, eight brain specimens were placed into culture and characterized according to cell kinetics, karyotype, antigenic expression, and ultrastructural features. The protein profiles of both the cell layer and the medium were analyzed in selected cultures using sodium dodecyl sulfate polyacrylamide gel electrophoresis and diethylaminoethyl cellulose chromatography. All cultures displayed a limited life span in vitro; marked contact inhibition at confluence; a normal karyotype; an intracytoplasmic and extracellular glycoprotein profile consisting of fibronectin, procollagen type III, laminin, and collagen type IV; specialized intercellular junctions; and interstitial collagen chain synthesis.

An unusual spinal meningioma in a child: case report.

Meningiomas seldom occur in children, and spinal meningioma in a child is rare. We report the case of a boy, 3 years and 5 months of age, who had an unusual atypical meningioma involving the subarachnoid space from T-2 to the cauda equina. To our knowledge, no similar case has been reported previously.

[Occupational and familial sporotrichosis].

The incidence of occupational mycoses and the main criteria to make a correct diagnosis are commented. The sources of infection of the superficial and deep professional mycotic infections are analysed. The occurrence of an epidemic outbreak of sporotrichosis in 13 members of one family is reported.