Following pulmonary metastases, alveolar soft part sarcoma has an unexplained predisposition to metastasize to the brain. Herein, a case of alveolar soft part sarcoma solitarily metastatic to the brain is described. A 23-year old female underwent the resection of alveolar soft part sarcoma from the right thigh. Three years after the surgery, a brain tumor was resected and pathology proved it was a metastatic alveolar soft part sarcoma. She is currently alive and well 6 years after brain surgery. Our report suggests that surgical resection for solitary brain metastasis from alveolar soft part sarcoma is an effective treatment modality.

Adult↗

PubMed1995

The membrane-bound and soluble forms of HLA-G bind identical sets of endogenous peptides but differ with respect to TAP association.

The class Ib antigen HLA-G is expressed as a membrane-bound protein like classical class Ia molecules (M.HLA-G) but, unlike typical class I, is also expressed as a soluble protein (S.HLA-G) with a unique C terminus. Our results show that, similar to classical class I proteins, the membrane-bound form of HLA-G associated with TAP, as evidenced by the ability to immunoprecipitate HLA-G class I heavy chain with TAP antisera. In contrast, the soluble G protein did not appear to associate with TAP in the same manner, since similar immunoprecipitation experiments failed to detect soluble G complex. A detailed analysis of peptides bound to the soluble and membrane HLA-G proteins expressed in the B lymphoblastoid cell line 721.221 showed that, like class Ia complexes, both HLA-G proteins consist of heavy and light chains complexed with nonameric peptides in a 1:1:1 ratio. The two proteins bind essentially the same set of peptides, which are derived from a variety of intracellular proteins and define a peptide motif for HLA-G. The peptides contain Leu at the C terminus and Pro or small hydrophobic amino acids in position 3 followed by Pro or Gly in position 4. The complexity of the bound peptides is lower than that found for some class Ia complexes, but is more similar to class Ia than to the limited repertoire of some murine class Ib molecules.

ATP-Binding Cassette Transporters↗

PubMed1995

Interferon-alpha 2 variants in the human genome.

Variants of human leukocyte interferon alpha 2 (IFN-alpha 2a, alpha 2b, and alpha 2c) differ from each other by changes in their coding regions at nucleotide positions 137 and 170. As a result of these nucleotide variations, the DNA sequences of the three variants can be distinguished by selective restriction enzyme analysis. Human genomic DNA obtained from over 28,000 normal healthy individuals was used as templates in the polymerase chain reaction (PCR) to amplify the human IFN-alpha 2 gene sequence. The resulting PCR products were analyzed with restriction nucleases to identify the specific IFN-alpha 2 variant sequences present in the genomic DNA of the population examined. The results show that IFN-alpha 2b was detected as the predominant species and IFN-alpha 2c as a very minor species (< 0.1%). The IFN-alpha 2a gene was not detected in this population.

Alleles↗

PubMed1995

Periventricular and subcortical nodular heterotopia. A study of 33 patients.

Grey matter heterotopias, demonstrated by MRI, may present with a broad spectrum of clinical severity. We have studied 33 patients with periventricular nodular heterotopias (PNH); 19 (58%) had unilateral and 14 (42%) bilateral lesions. Thirteen of the 19 patients (68%) with unilateral subependymal nodules of grey matter had, in addition, unilateral focal subcortical heterotopias (SNH), comprising 39% of the entire group. Most had normal intellectual and motor function but some presented with mild mental retardation and neurological deficits. Recurrent seizures were described in 82%, mainly partial attacks with temporo-parieto-occipital auras. Nodular heterotopias led to unilateral or bilateral independent temporal epileptic discharges in 47% of epileptic patients with PNH alone and in 61% of those who had SNH in addition. Extratemporal or multilobar, unilateral or bilateral interictal spiking was present in 10 other patients (36%). Two first degree relatives of patients with seizures were affected but had no seizures, three were investigated for other apparently unrelated neurological symptoms: memory impairment, vertigo or transient ischaemic attacks in one person each. Contiguous ovoid nodules of grey matter, symmetrically lining both lateral ventricles, were described in nine patients. Seven of them were female, including four with familial incidence of PNH. Such lesions may explain the familial occurrence of epilepsy in some families. Seven patients underwent anterior temporal resection: two patients with unilateral subependymal and focal subcortical heterotopias were seizure free or significantly improved. Four patients, three with PNH alone and one with additional subcortical nodules, did not improve significantly after surgery. The remaining patient was followed for less than 6 months.

Adolescent↗

PubMed1995

RG1, a new murine monoclonal antibody recognizing a "supertypic" determinant on HLA-A molecules.

Monomorphic and polymorphic anti-HLA monoclonal antibodies (mAb) are valuable reagents for assessment of the structural and functional importance of different class I determinants. We have generated a new mAb, RG1, reacting with an epitope variably expressed on normal and leukemic hematopoietic cells of different lineages. Immunoprecipitation of the RG1 antigen disclosed a bimolecular complex characteristic of class I proteins. The RG1 epitope was expressed on an HLA-A2 transfected cell line but not on cells transfected with HLA-E, -F or -G molecules. MAb reactivity with reference B-lymphoblastoid cell lines and HLA typing of RG1 reactive and unreactive cells demonstrated that the epitope was expressed in conjunction with defined HLA-A molecules. Cells expressing HLA-A2, -A24(9) and -A68(28) proteins were brightly stained with RG1 whereas mAb binding to HLA-A1, -A11 and a split of A3 molecules was significantly lower. In contrast, the RG1 epitope was apparently not expressed on HLA-A23(9), -A25(10), -A26(10), -A29(19), -A30(19), -A31(19), -A32(19), -A33(19) and some HLA-A3 molecules. Based on class I alpha sequence data, these results suggest that the RG1 epitope is localized to a region of the alpha 2 helix accessible to the T cell receptor for antigen on cytotoxic T lymphocytes. Lys in position 144 and His in position 151 are apparently critical for RG1 binding.

Amino Acid Sequence↗

PubMed1995

Fast spin-echo, magnetic resonance imaging-measured hippocampal volume: correlation with neuronal density in anterior temporal lobectomy patients.

To assess the value of magnetic resonance imaging (MRI)-measured hippocampal volume in the detection of hippocampal sclerosis, we studied 28 patients undergoing anterior temporal lobectomy for medically intractable mesial temporal lobe epilepsy. Hippocampal volumetry and visual analysis of T2 signal change were performed using fast spin-echo T2-weighed MRI. Quantitative neuronal density measurements were performed in the resected hippocampal specimens. There was a significant correlation between MRI-measured absolute hippocampal volume (AHV) and neuronal density in CA1, CA2, and CA3 subfields (p < 0.0001, p < 0.01, and p < 0.05, respectively). Differential hippocampal volume (side-to-side volume difference) failed to detect bilateral atrophy in three patients, but the bilateral hippocampal atrophy was recognized by considering AHV in these patients. This study suggests that MRI-measured AHV can be of value in elevating patients with mesial temporal lobe epilepsy, especially when there is no side-to-side difference in hippocampal volumetry.

Adolescent↗

PubMed1995

Fast spin-echo MR in hippocampal sclerosis: correlation with pathology and surgery.

PURPOSE: To identify the extent of hippocampal sclerosis in temporal lobe epilepsy with fast spin-echo MR and correlate it with histopathologic findings and surgical outcome. METHODS: MR images of 30 patients with temporal lobe epilepsy and pathologically proved hippocampal sclerosis and 30 control subjects were obtained using a fast spin-echo technique with 4000/100/4 (repetition time/echo time/excitations), 16 echo train, 2- to 3-mm section thickness with interleave, 256 x 256 matrix, and 18-cm field of view. Criteria for MR diagnosis of hippocampal sclerosis included hippocampal atrophy diagnosed with MR volumetry and/or T2-weighted signal change. Hippocampal sectional areas were plotted, and T2 signal changes were topographically evaluated to identify the extent of hippocampal sclerosis, which was subsequently correlated with histopathologic findings and surgical outcome. RESULTS: Hippocampal sclerosis was diffuse, involving both hippocampal head and body, in 96.7% of patients (29 of 30 patients). One patient had normal MR findings. Focal hippocampal sclerosis was not seen. Histopathologic findings of hippocampal sclerosis were present in all 29 patients who had abnormal MR findings. Eighty-six percent of patients (18 of 21 patients), who were followed for at least 1 year after temporal lobectomy, were seizure free (81%, 17 of 21 patients) or significantly improved (5%, 1 of 21 patients). CONCLUSION: Fast spin-echo MR enables accurate definition of the extent of hippocampal sclerosis in patients with temporal lobe epilepsy. All cases of hippocampal sclerosis identified in this study involved the hippocampus diffusely. However, leaving the posterior portion of the hippocampus during surgery does not seem to be a major factor influencing surgical outcome.

Adolescent↗

PubMed1995

Clinical significance of asymmetry of the fornix and mamillary body on MR in hippocampal sclerosis.

PURPOSE: To investigate the clinical significance of MR-defined asymmetry of the fornix and mamillary body for presurgical determination of the side of hippocampal sclerosis in patients with temporal lobe epilepsy. METHODS: Fast spin-echo MR images were evaluated for evidence of an asymmetrically small fornix and mamillary body in 33 patients with pathologically proved hippocampal sclerosis (presurgical hippocampal sclerosis group), 7 patients who had undergone anterior temporal lobectomy (mean, 3 years from surgery) because of hippocampal sclerosis (postsurgical hippocampal sclerosis group), and 34 healthy subjects (control group). Fast spin-echo hippocampal volumetry was performed in each patient. RESULTS: In the control group, 6% (2 of 34) of subjects had MR evidence of asymmetrically small fornix and none (0 of 34) of the subjects had asymmetrically small mamillary body. In the patient population, an asymmetrically small fornix was seen in 42% of presurgical hippocampal sclerosis group, 39% (13 of 33) ipsilateral, and 3% (1 of 33) contralateral, and in 71% of the postsurgical hippocampal sclerosis group (5 of 7), all ipsilateral. In the presurgical hippocampal sclerosis group, hippocampal atrophy measured with MR was more severe in patients with an ipsilaterally small fornix than in patients without. An asymmetrically small mamillary body was found ipsilaterally in 3% (1 of 33) of the presurgical hippocampal sclerosis group and in 57% (4 of 7) of the postsurgical hippocampal sclerosis group; all patients with an asymmetrically small mamillary body in the postsurgical hippocampal sclerosis group also had an asymmetrically small fornix on the same side. CONCLUSION: In presurgical hippocampal sclerosis patients, an asymmetrically small fornix can be seen ipsilaterally on the side of the hippocampal sclerosis; however, its low frequency, its association with severe hippocampal atrophy only, and the possibility of false-positive results limit its clinical usefulness in determining the side of the seizure focus. An asymmetrically small mamillary body is too rare to be used for presurgical location of hippocampal sclerosis. However, an asymmetrically small fornix and mamillary body are frequently seen on MR images after temporal lobectomy.

Adolescent↗

PubMed1995

Antimicrobial susceptibility and beta-lactamase production of Moraxella catarrhalis isolates in Taiwan.

Between 1 August 1993 and 31 July 1994, 135 clinical isolates of Moraxella catarrhalis were collected from 12 large medical laboratories in Taiwan. The majority of specimens came from sputum (124 isolates). Other specimens included four isolates from throats, three isolates from wounds or pus, two isolates from eyes, one isolate from blood and one from cerebrospinal fluid. Epidemiologically, M. catarrhalis isolates were found frequently in winter and spring with a peak in February, and only sporadically from April to September. The overall rate of beta-lactamase producing isolates was 98.5% (132/135). All isolates were considered to be ampicillin-resistant, none were found to be resistant to other beta-lactam agents. Among other antimicrobial agents, all isolates were susceptible to chloramphenicol, erythromycin, roxithromycin, ofloxacin and ciprofloxacin, but uniformly resistant to trimethoprim (minimum inhibitory concentration (MIC) > or = 4 micrograms/mL, zone diameter < or = 19 mm). There were 12 isolates (8.8%) resistant to sulfamethoxazole (MIC > or = 32 micrograms/mL, zone diameter < or = 19 mm) and 19 isolates (14.4%) resistant to tetracycline (MIC > or = 16 micrograms/mL, zone diameter < or = 19 mm). The high level of resistance to ampicillin due to beta-lactamase production indicates that this is no longer a reliable agent for the treatment of M. catarrhalis infections. Among the beta-lactam agents tested, amoxicillin + clavulanate and the cephalosporins were active. These agents appear to be reliable first-line therapies when infection with M. catarrhalis is suspected. Misidentification of the species and difficulties in determining susceptibility to ampicillin are still widespread in Taiwanese laboratories. The application of the butyrate hydrolysis test and an appropriate test for beta-lactamase production is necessary for the resolution of these problems.

Adolescent↗

PubMed1994

D2 dopamine receptor mRNA distribution in cholinergic and somatostatinergic cells of the rat caudate-putamen and nucleus accumbens.

An in situ hybridization procedure that identifies cells expressing D2 dopamine receptor mRNA was combined in double-labelling studies with immunohistochemical procedures that identify cells expressing either choline acetyltransferase (ChAT) or somatostatin. D2 receptor mRNA was detected in almost all of the ChAT positive caudate-putamen cells, approximately half of the ChAT positive nucleus accumbens cells and none of the somatostatin-positive cells in either brain region.

Acetylcholine↗

PubMed1994

Neuronal migration disorders: positron emission tomography correlations.

We analyzed the interictal [18F]fluoro-2-deoxy-D-glucose positron emission tomography (PET) findings of 17 epileptic patients with neuronal migration disorders (NMDs). Fifteen patients had abnormal PET findings, i.e., focal hypometabolism in 9 patients and displaced metabolic activity of normal gray matter in 6. All 15 patients had magnetic resonance imaging (MRI) abnormalities; however, PET abnormality assisted in the identification of NMDs on MRI in 3 patients. Two patients with negative MRI also had negative PET studies. PET hypometabolism appeared to correlate with severity of neuronal dysgenesis or temporal lobe involvement, or both. Displaced metabolic activity of gray matter is regarded as a unique interictal [18F]fluoro-2-deoxy-D-glucose-PET finding in NMD. This study demonstrates variable metabolic patterns in NMD and that PET may be a useful complement to MRI in the evaluation of NMD.

Adult↗

PubMed1994

Leiomyoblastoma causing acute gastric outlet obstruction in an infant.

A 1-year-old boy had acute gastric outlet obstruction, the cause of which was a prepyloric submucosal mass. The mass and the ulcerated overlying mucosa were excised. The histological diagnosis was leiomyoblastoma with parenchymatous hemorrhage. Apparently, he is the youngest patient reported to have this disease.

Acute Disease↗

PubMed1994

Assessment of four rapid urease test systems for detection of Helicobacter pylori in gastric biopsy specimens.

An in vitro experiment and an in vivo clinical trial were both performed in order to assess the efficacy of four biopsy urease test systems, including one commercial kit, Temmler CUT test (Temmler Pharma, Marburg, Germany), for the rapid detection of Helicobacter pylori. We first evaluated four rapid urease test systems by inoculating bacterial suspensions of different concentrations into urea-containing media and observing the color change at room temperature. We found that the CUT test was superior in vitro to the other three urease test systems. As was expected, the lower the concentration of the inoculum, the slower was the color change and the fewer were the positive results noted. The minimal concentration of H. pylori for a positive urease test at 24 h was 1000-10,000 colony-forming units/ml in 1 drop of bacterial suspension inoculated. We then evaluated four biopsy urease test systems for the rapid diagnosis of H. pylori infection in antral and fundal mucosa biopsy specimens of 37 patients undergoing upper gastrointestinal endoscopy. All four test systems were 100% specific when compared with culture. In 69 culture-positive biopsy specimens, the CUT test was not only more sensitive (72%) than the other three systems (42%, 51%, and 45%, respectively), but also gave the fastest reaction by detecting more culture-positive biopsy specimens after 3 h of incubation at room temperature. The differences were statistically significant.

Bacteriological Techniques↗

PubMed1994

Distribution of interferon-alpha 2 genes in humans.

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Cell Line↗