Extradural extension of primitive neuroectodermal tumor--case report.

A 13-year-old boy presented with a primitive neuroectodermal tumor (PNET) with unusual extracranial extension. Precontrast computed tomography showed the tumor as a ring-shaped high-density area which was enhanced postcontrast, with a low-density center. Magnetic resonance imaging showed the tumor as a low-intensity area on the T1-weighted images with marked enhancement by gadolinium-diethylenetriaminepentaacetic acid, and high intensity on the T2-weighted images.

Calcineurin activates transcription from the GM-CSF promoter in synergy with either protein kinase C or NF-kappa B/AP-1 in T cells.

Two cis-acting elements GM-kappa B/GC-box and CLE0, of the granulocyte-macrophage colony-stimulating factor (GM-CSF) gene are required for maximal induction in Jurkat T cells by costimulation with phorbol-12-myristate acetate (PMA) and Ca2+ ionophore (A23187). The GM-kappa B sequence is recognized by NF-kappa B, which is mainly induced by PMA. The CLE0 sequence interacts with factors, related to a PMA-induced AP-1 and a PMA/A23187-induced NF-AT.

Parkinson-like symptoms as a manifestation of systemic lupus erythematosus.

A 42-year-old Japanese woman with systemic lupus erythematosus (SLE) developed Parkinsonian-like movements. Steroid pulse therapy was most effective and additional anti-Parkinsonian drugs were not required. Although psychosis, seizures and meningitis are common central nervous system (CNS) manifestations in SLE patients, Parkinsonian-like symptoms are extremely rare.

[Primary adenomatous tumor of the middle ear: a case report].

A case of primary adenomatous tumor of the middle ear is described. A 16-year-old male was admitted with a one-year history of left hearing disturbance. Skull X-ray disclosed destruction of the left petrous bone.

Regulation of protein kinase C isoform proteins in phorbol ester-stimulated Jurkat T lymphoma cells.

Activation of protein kinase C (PKC) in T cells leads to a variety of responses including IL-2 production and IL-2 receptor expression. PKC consists of several isoforms that exhibit some different in vitro properties. We have set up a Western blotting system to explore the regulation of PKC isoforms during T cell activation.

A case of sellar chordoma mimicking a non-functioning pituitary adenoma with survival of more than 10 years.

A rare case of sellar chordoma occurring in a 67-year-old woman who survived for more than 10 years is presented. Clinical signs and symptoms masqueraded as a non-functioning pituitary adenoma with visual disturbance and hypopituitarism. Initial computed tomography (CT) showed an intrasellar mixed dense mass with suprasellar extension, accompanied by non-homogeneous contrast enhancement.

Ossifying cementicular fibroma of the orbitofrontal bone in a child: case report.

A rare case of ossifying cementicular fibroma of the left orbitofrontal bone that developed in a 12-year-old boy is presented. A hard, painless mass that was incidentally noticed gradually enlarged over 2 years. Skull X-rays showed a well-demarcated lesion with mixed sclerotic and osteolytic radiolucent changes in the left orbitofrontal bone.

Clinicopathological study on craniopharyngioma with sebaceous differentiation.

We presented 2 cases of craniopharyngioma, which showed sebaceous differentiation. Both cases shared the following features: 1) neuroradiological detection of a cystic tumor accompanied by calcification, 2) clear yellowish fluid detected in the cyst during surgery, 3) histological rating as an adamantinomatous type, 4) the presence of a nest-like distribution of multivacuolated cells in the innermost layer of the cyst.

The effect of serum Ca2+ compensation on the efficacy of chlorpropamide in alloxan-diabetic rabbits.

A decrease in serum Ca2+ concentration was observed in alloxan-diabetic rabbits, with recovery of serum Ca2+ levels achieved following insulin therapy. This suggested that the diabetic condition directly relates to the decrease in serum Ca2+ levels. The efficacy of chlorpropamide in alloxan-diabetic rabbits after intravenous injection (150 mumol kg-1) was less than that in normal rabbits as measured by the serum insulin levels, and the efficacy did not change when the dose was increased.

Transient down-regulation of PKC-zeta RNA following crosslinking of membrane IgM on WEHI-231 B lymphoma cells.

Regulation of protein kinase C (PKC) isoform mRNAs has been studied in the immature, murine B lymphoma WEHI-231 by the MAPPing protocol and by slot blot analysis of unamplified mRNA. This membrane IgM (mIgM)-positive cell line has been previously used as a model to study signal transduction by mIgM in immature B lymphocytes and the role of those signals in the induction of immune tolerance in the B cell compartment. Stimulation of the cells by anti-mu antibodies, phorbol ester, or Ca2+ ionophore caused growth arrest and death of the cells.

Evidence for clonal heterogeneity of the expression of six protein kinase C isoforms in murine B and T lymphocytes.

Protein kinase C (PKC), which plays a pivotal role in lymphocyte activation, represents a homologous family of at least nine proteins. Seven genes that encode PKC proteins have been identified. Since the regulatory properties and substrate specificities of the isoforms are not identical in vitro, it is possible that each isoform plays a unique role in cell activation.

Intravenous recombinant tissue plasminogen activator in acute carotid artery territory stroke.

To determine the effect of intravenous recombinant tissue plasminogen activator (rt-PA) on vascular and neurologic outcomes, we enrolled 31 patients with acute carotid artery-territory ischemic stroke within 6 hours from symptom onset in a randomized, double-blind, placebo-controlled study. We gave either rt-PA (duteplase at the dose of 20 or 30 mega-international units [MIU]) or placebo intravenously for 60 minutes in patients randomly assigned to the three groups. A comparison between the baseline and postinfusion angiograms showed that complete or partial reperfusion occurred in 50% (5/10) of patients treated with 30 MIU rt-PA, 44% (4/9) of those treated with 20 MIU rt-PA, and 17% (2/12) in the control group.

Surface mu heavy chain expressed on pre-B lymphomas transduces Ca2+ signals but fails to cause growth arrest of pre-B lymphomas.

Little is known about the role of signals transduced by cell surface IgM (sIgM) expressed during early B cell development. A subclone (1.6) of the late pre-B cell lymphoma 70Z/3.

The identification of human semen by a chemiluminescent assay of choline.

A method for proving the presence of semen has been established by utilizing chemiluminescence for an assay of choline, a nonprotein constituent of semen, and medicolegal testings were carried out to evaluate its effectiveness. The sensitivity of this method to choline was 3 nmol/ml, and as a result of assays of the choline content in semen and in several other human body fluids and secreta, the choline level in semen was found to be 10.88-26.

Protein losing enteropathy associated with collagen diseases.

Four patients with collagen diseases are described, who developed protein losing enteropathy in the course of their disease. Their protein losing enteropathies subsided after treatment with 60 mg/day oral prednisolone. Immunohistological studies of the small intestine showed deposits of C3 in the capillary walls of villi in two patients.

An autopsy case of mitochondrial encephalomyopathy with prominent degeneration in olivo-ponto-cerebellar system.

We describe a sporadic case of adult-onset, complex I deficiency mitochondrial encephalomyopathy (MEM), the clinical and pathological features of which failed to fit any of the known subgroups of MEM, such as Kearns-Sayre syndrome, mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes or myoclonus epilepsy with ragged-red fibers. Clinically, this patient had only progressive cerebellar ataxia, generalized muscle weakness and hearing loss. The principal finding at autopsy was degeneration of the olivo-ponto-cerebellar system.

[A case of venous hemangioma in the mediastinum].

A thirteen-year-old boy was admitted to our hospital for further evaluation of abnormal shadow on chest film with a low grade fever. Laboratory findings revealed within normal value except for CRP. Chest film showed a smooth round mass in right middle mediastinum.

Infantile myofibromatosis with a solitary lesion in the skull--case report.

The term "infantile myofibromatosis" was coined in 1981 to describe a rare type of soft-tissue tumor in infants. Solitary lesions are usual, but extremely rare in the skull. An infantile case involving a lesion in the left parietal bone is described.

Optic nerve degeneration caused by supraophthalmic carotid artery infusion with cisplatin and ACNU. Case report.

A 28-year-old woman with a left frontoparietal anaplastic astrocytoma was treated postoperatively with a combination of cisplatin and 1-(4-amino-2-methylpyrimidine-5-yl) methyl-3-(2-chloroethyl)-3-nitrosourea hydrochloride (ACNU). The drugs were infused via the left supraophthalmic internal carotid artery in an attempt to avoid ocular toxicity. The patient subsequently developed blindness in the left eye and a right temporal hemianopsia from marked degeneration of the left optic nerve and tract.

The relationship between anti-IgE auto-antibodies and the IgE response to wasp venom during immunotherapy.

During immunotherapy with wasp venom, levels of venom-specific IgE antibodies increase and then fall, whereas the concentration of IgG antibodies rises and then remains at a high level. Successful treatment is therefore associated with both increased concentrations of serum IgG and decreased serum IgE antibodies to venom. In this study we have investigated the possible role of auto-antibodies in inducing the decrease in serum IgE antibody.

IgG class anti-cardiolipin antibody as a possible marker for evaluating fetal risk in patients with systemic lupus erythematosus.

To identify the correlation between incidence of anti-phospholipid antibodies and fetal prognosis in pregnant SLE patients, we measured the amount of anti-cardiolipin antibody in their sera, using solid-phase enzyme immunoassay (EIA) methods. Findings in the group having poor obstetric results (fetal loss group) and in those with a history of full-term births (live birth group) were compared with regard to other anti-phospholipid antibodies. The incidence of IgG class anti-cardiolipin antibody was 60% in the fetal loss group and 19% in the live birth group, (P less than 0.

[Adult polysaccharide storage myopathy].

A first Japanese case of an adult polysaccharide storage myopathy (APSM) was reported. A 30-year-old Japanese male was admitted because of weakness of the lower limbs. The onset of the symptoms was at the age of 23.

[Adult-onset Hartnup disease presenting with neuropsychiatric symptoms but without skin lesions].

Hartnup disease is an inborn abnormality of renal and intestinal transport involving the neutral amino acids. Intermittent pellagra-like rash, attacks of cerebellar ataxia and psychiatric disturbance are characteristic symptoms of this disease. We described here a patient with adult-onset Hartnup disease who presented unique neuropsychiatric symptoms but no dermatologic symptoms, and reported features of amino acids transport in this patient and his family.