Cerebellar pilocytic astrocytoma with leptomeningeal dissemination: case report.
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Arachnoid↗
Biomedical subjects
T Nagashima
Publications and source records attributed to T Nagashima.
Acute effects of phorbol ester and insulin on insulin-induced glucose uptake and protein kinase C activation in rat adipocytes.
We examined the acute effect of pretreatment with phorbol ester and insulin on insulin-induced glucose uptake and protein kinase C (PKC) translocation from cytosol to the membrane in rat adipocytes. Adipocytes were preincubated with 1 microM tetradecanoylphorbol 13-acetate (TPA) and 10 nM insulin for 60 min and then stimulated with 10 nM insulin for 10 and 30 min to measure PKC activity in cytosol and membrane fractions using a Mono Q column connected onto an HPLC system and [3H]2-deoxyglucose (DOG) uptake, respectively. Pretreatment with 1 microM TPA and 10 nM insulin for 60 min resulted in the marked decreases of insulin-induced [3H]2-DOG uptake. Translocation of Mono Q column-purified cytosolic PKC enzyme activity and PKC beta immunoreactivity from cytosol to the membrane was suppressed by pretreatment with TPA and insulin for 60 min. These results indicate that acute treatment with TPA and insulin which are PKC activators suppress translocation/activation of PKC, and accordingly inhibit insulin-induced glucose uptake. We suggest that a decrease of cytosolic PKC activity may mainly-contribute to the impaired responsiveness of the glucose transport system after acute TPA and insulin treatment.
Adipocytes↗
A novel point mutation of thyroid hormone receptor beta gene in a family with resistance to thyroid hormone.
Resistance to thyroid hormone (RTH) is characterized by variable tissue hyporesponsiveness to thyroid hormone caused by mutations of thyroid hormone receptor beta (TRbeta) gene. We found a novel point mutation of the TRbeta gene in a family (F123) with RTH, a transition of a guanine to adenine at nucleotide 1215, which replaced the normal Met-310 with Ile. This substitution was found in only one allele of affected family members. In vitro transcription and translation of this mutant TRbeta demonstrated a 12-fold reduction of the affinity for triiodothyronine (T3) compared with the wild type TRbeta. Thyroid function tests were similar to a previously reported RTH family (F99) who had a different mutation in the same codon (Thr 310).
Adolescent↗
Use of a split bone graft to correct the cosmetic deformity associated with the transpetrosal-transtentorial surgical approach: technical report.
OBJECTIVE: Split bone graft reconstruction was performed after a standard transpetrosal-transtentorial approach to correct the cosmetic deformity in 18 patients. TECHNIQUE: The split bone graft was made by dividing the inner table of the temporosuboccipital bone graft and covered the bone effect created by petrosectomy and mastoidectomy. The split bone graft was created and shaped during closure of the dura, so that the operation was not prolonged. RESULTS: This technique produced excellent cosmetic results immediately after surgery; the results were maintained during the follow-up period of between 20 and 46 months. No complication associated with this technique was recognized. CONCLUSION: Split bone graft reconstruction is a simple and safe technique for preventing the cosmetic deformity associated with a transpetrosal-transtentorial approach.
Bone Transplantation↗
Two unusual cases of multiple dural arteriovenous fistulas.
OBJECTIVE AND IMPORTANCE: Two patients with dural arteriovenous fistulas involving the transverse sinus and superior sagittal sinus are described, with a focus on the unique type of venous drainage of the fistula. CLINICAL PRESENTATION: Both patients presented with papilledema and progressive visual disturbance. Angiography and magnetic resonance imaging showed that the fistulas involving the superior sagittal sinus had a dilated venous channel, separate from the sinus lumen, located within the wall of the sinus. INTERVENTION: Transvenous embolization of the venous channel of the fistula, proximal to its drainage into the superior sagittal sinus, resulted in closure of the fistula and restoration of the superior sagittal sinus function. The clinical symptoms were reversed; the symptoms are believed to have reflected venous hypertension in the superior sagittal sinus, resulting from the shunted flow and interfering with normal venous drainage. CONCLUSION: This unique type of dural arteriovenous fistula may be a variant, occurring in the developmental process of the fistula. It is significant clinically because transvenous embolization can be used to close the fistula and restore sinus function.
Aged↗
Impact velocities of the teeth after a sudden unloading at various initial bite forces, degrees of mouth opening, and distances of travel.
A potentially dangerous situation arises when an individual bites on hard and brittle food which suddenly breaks, since the impact velocity of the lower teeth onto the upper teeth after the food is broken can be high and may cause dental damage. The present experiments were designed to study the magnitude of the impact velocity after a sudden unloading at various initial bite forces, degrees of mouth opening, and distances of travel. Subjects were asked to perform a static biting task during which the resistance to the bite was suddenly removed. The upward mandible movement was arrested after a certain distance. The velocity of the lower teeth at impact was calculated just before the mandible came to a standstill in combinations of 4 different bite forces (100, 80, 60, and 40 N), 4 different initial degrees of mouth opening (33.5, 30.5, 27.5, and 24.5 mm), and 3 different distances of travel of the mandible (4.5, 3.0, and 1.5 mm). We found that the bite force rapidly declined after the unloading, resulting in a small impact velocity of the lower front teeth. This impact velocity largely depended on the magnitude of the initial bite force and the distance traveled; it was barely sensitive to variations in degree of initial mouth opening. The maximal velocity of the lower teeth was 0.43 m/s (at an initial bite force of 100 N). This maximum was reached after a distance of travel of about 4 mm in 12 ms. The data suggest that the rapid decline in bite force coupled with a limitation of impact velocity is due to the force-velocity properties of the active jaw muscles and is not caused by neural control.
Biomechanical Phenomena↗
Modulation of protein kinases and microtubule-associated proteins and changes in ultrastructure in female rat pituitary cells: effects of estrogen and bromocriptine.
This study focused on the intracellular signal transduction system and microtubule-associated proteins (MAPs), such as MAP-2 and Tau protein. The modulation of these proteins and their correlation with ultrastructural changes were investigated in rat pituitary prolactin (PRL) cells. Adult female Wistar rats were treated with estrogen and bromocriptine and their pituitary glands were removed for analysis of the expression of tubulin, MAP-2, Tau protein, protein kinase C (PKC), and calcium calmodulin (CaM) kinase. Western blot analysis showed that estrogen increased and bromocriptine decreased the expression of PKC alpha, beta 1, beta 2, CaM kinase alpha, beta, MAP-2, and Tau protein. MAP-2 and Tau protein, which are cytosolic proteins, being translated on free ribosomes, were associated with the membrane of whirling rough endoplasmic reticulum (RER) in estrogen-treated cells and dissociated with vesiculated RER induced by bromocriptine. These results suggested that the modulation of MAP-2 and Tau protein may reflect changes of PKC and CaM kinase, and that the quantitative changes and intracellular modulation of MAPs induced by estrogen and bromocriptine, i.e., estrogen-induced association and bromocriptine-induced dissociation of MAP-2 and Tau protein with membrane of RER, may reflect the dynamics of microtubules and are associated with structural changes in the RER and changes in the synthesis and intracellular transport of PRL.
Animals↗
Detection of skewed X-inactivation in two female carriers of vasopressin type 2 receptor gene mutation.
Most cases of congenital nephrogenic diabetes insipidus (NDI) are inherited in an X-linked manner, which is due to the mutations of the vasopressin type 2 receptor (V2R) gene. However, recent reports have presented female NDI patients with heterozygote V2R gene mutations. The mechanism of inheritance was thought to be skewed X-inactivation. We present a family with congenital NDI. Three male members were diagnosed with NDI, and examination of their V2R gene revealed a G inserted at nucleotide 804 of the open reading frame. Three female individuals display different degrees of symptoms of NDI, and all of them possess both the normal and abnormal genes. The X-inactivation patterns of the female members were investigated via the detection of methylated trinucleotide repeat in the human androgen receptor gene. The grandmother showed extremely skewed methylation of one X chromosome, and the mother revealed moderately skewed methylation. The daughter of the grandmother's sister, who has no symptoms of NDI, showed random methylation. The highly skewed X-inactivation pattern of the grandmother suggests that her NDI phenotype is caused by dominant methylation of the normal allele of V2R gene.
Adult↗
Molecular basis of nonclassical steroid 21-hydroxylase deficiency detected by neonatal mass screening in Japan.
Since 1989, neonatal mass screening for congenital adrenal hyperplasia (CAH) has been performed in Japan, and the frequency of the classical form of 21-hydroxylase deficiency was found to be nearly identical to that in other countries. However, it has not yet been determined whether our mass screening program can detect the nonclassical (NC) form. From 1991 to 1994, about 4,500,000 infants underwent CAH mass screening in Japan. During this period, we identified by screening 2 siblings and 2 unrelated patients who had mild elevation of serum 17-hydroxyprogesterone levels at 5 days of age, but who revealed no symptoms of CAH. They were diagnosed as having probable NC steroid 21-hydroxylase deficiency. To clarify the molecular basis of NC CAH detectable by neonatal screening in Japan, the steroid 21-hydroxylase (CYP21) genes from these cases were analyzed. The 2 siblings (patients 1 and 2) had I172N and R356W mutations in 1 allele and in the other allele had local gene conversion, including the P30L mutation in exon 1. Patient 3, who was unrelated, had gene conversion encoding the same P30L mutation in 1 allele and in the other allele had an intron 2 mutation (668-12 A-->G), causing aberrant ribonucleic acid splicing, and the R356W mutation. Patient 4, also a compound heterozygote, had the R356W and 707del8 mutations. The estimated rate of detection of the NC form by mass screening (1:1,100,000) seemed low compare to the established detection rate for the classical form (1:18,000). As all of our 4 patients were compound heterozygotes with at least 1 allele bearing 1 or more mutations associated with classic CAH, it may be difficult to detect NC cases carrying only NC-associated alleles using our current neonatal mass screening methods.
17-alpha-Hydroxyprogesterone↗
Cytologic morphometric approach for the prediction of lymph node involvement in papillary thyroid cancer.
OBJECTIVE: Papillary thyroid cancer frequently metastasizes to lymph nodes; however, it is generally difficult to make a precise diagnosis preoperatively. The present study was carried out to evaluate lymph node status preoperatively by using quantitative estimations of various cytologic features obtained by fine needle aspiration biopsy from 110 patients with papillary thyroid cancer. STUDY DESIGN: Using a microscope connected to a computerized video system, mean nuclear area (NA) mean circumferential length of nuclei (CL), circular rate (CR), largest to smallest diameter ratio of nuclei (LS), coefficient of variation of NA (NACV), and rates of intranuclear cytoplasmic inclusions (CIs) and nuclear grooves (NG) were calculated and analyzed. RESULTS: LS, NACV and NG were significantly higher in cases with metastasized lymph nodes than node-negative cases (P < .05, P < .0001, P < .05, respectively). Furthermore, all parameters except CR and LS correlated exactly with the histopathologic features (P < .0001). The number of positive lymph nodes correlated well with the value of NACV, and all cases with recurrences proved to have NACV > 22%. CONCLUSION: Preoperative quantitative estimations of cytologic features are useful parameters for the prediction of lymph node metastases in papillary thyroid cancers.
Adolescent↗
[Growth factors and neurotrophic factors with relevance to gliomas].
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Brain Neoplasms↗
Gradenigo's syndrome: CT and MRI findings.
We report the case of an 8-year-old girl with Gradenigo's syndrome. Involvement of the petrous portion of the left temporal bone was demonstrated by CT and an inflammatory lesion of the left petrous apex was clearly shown by MRI, which is useful in diagnosis and management of apical petrositis.
Abducens Nerve↗
Simultaneous ultrastructural identification of growth hormone and its messenger ribonucleic acid using combined immunohistochemistry and non-radioisotopic in situ hybridization: a technical note.
The present electron microscopical study is concerned with the simultaneous visualization of messenger ribonucleic acid (mRNA) and its encoded protein in the same specimen. Pre-embedding electron microscopical in situ hybridization (EM-ISH) on rat pituitary gland tissue localized growth hormone mRNA in the polysomes of the rough endoplasmic reticulum, and subsequent postembedding immunolabelling using protein A-colloidal gold particles identified growth hormone mainly in the secretory granules. We believe that our report provides the first simultaneous ultrastructural identification of mRNA and its encoded protein using combined pre-embedding EM-ISH and immunohistochemistry. In this method, the signals for mRNA were localized specifically as highly electron dense products on the polysomes of the endoplasmic reticulum, and those for its encoded protein were recognized as gold particles both in the cisternae of the reticulum and in the secretory granules. Our ultrastructural double labelling method for mRNA and protein may provide a tool to find important clues for elucidating the intracellular correlation of mRNA translation and secretion of translated protein, because of its high resolution, good morphological preservation, and the specific localization of the reaction products.
Animals↗
Clinical and histopathological analysis of proliferative potentials of recurrent and non-recurrent meningiomas.
Proliferative potentials of meningiomas from 127 patients were examined immunohistochemically using the anti-Ki-67 monoclonal antibody, MIB-1, on paraffin sections, and the correlation among MIB-1 staining index (SI), histopathological finding, and clinical course of the disease was analyzed retrospectively. The mean MIB-1 SI of 50 male patients with meningioma was 5.5%, whereas that of 77 female patients was 2.7%. Higher MIB-1 SI were observed for younger patients. These age- and sex-related differences in MIB-1 SI were statistically significant. The patients were assigned to one of three groups: those with non-recurrent meningioma (n = 73); those with recurrent meningioma in whom the specimens obtained during the initial surgery were used to calculate the MIB-1 SI (n = 21); and those with recurrent meningioma for whom the specimens obtained during the surgery for recurrent tumors were used to calculate the MIB-1 SI (n = 33). The mean MIB-1 SI in these patients were 1.6%, 3.6%, and 8.8%, respectively, and there were statistically significant differences among these three groups. Statistical analyses reveal that meningiomas with a MIB-1 SI of 3% or more have a significantly high tendency for recurrence during the clinical courses, especially within the first 10-year follow-up periods. Moreover, there is statistically significant correlation between MIB-1 SI and recurrence in each Simpson's grade. The time interval to the next recurrence for recurrent meningiomas is associated with the proliferative potential represented by the MIB-1 SI, and a correlation equation has been proposed to predict the date of the next recurrence. Analyses on cellularity of meningiomas revealed no statistically significant difference in cellularity between non-recurrent and recurrent meningiomas. There was no statistically significant relationship between cellularity and MIB-1 SI of meningiomas. In conclusion, examination on proliferative potentials of meningiomas using MIB-1 SI is very important for biological and histopathological analyses and the prediction of future recurrence.
Adult↗
Correlation between MIB-1 staining index and the immunoreactivity of p53 protein in recurrent and non-recurrent meningiomas.
Wild type p53 protein has been shown by recent investigations to be involved in the negative regulation of cell proliferation, whereas aberrant p53 protein has lost this negative regulation of cell growth. Wild type p53 protein, which has a very short half-life, has generally been considered to be undetectable using immunohistochemical methods; however, according to a recent report, wild type p53 protein may accumulate in the nuclei because of a defective ubiquitin pathway. Aberrant p53 protein has a longer half-life, and thus is visible using immunohistochemical methods. In this study, both the proliferative potential represented by the MIB-1 staining index (SI) and the immunoreactivity of p53 protein in 51 intracranial meningiomas were studied applying immunohistochemical staining methods to archival paraffin sections. The correlation among MIB-1 SI, p53 immunoreactivity, histopathologic findings and the clinical course of the meningiomas was also analyzed retrospectively. Although it is not possible with available reagents to distinguish between aberrant p53 protein and wild type p53 protein, statistical analyses show that p53 protein was immunostained both in meningiomas with high MIB-1 SI and in recurrent meningiomas. This demonstrates the close relationship among p53 immunoreactivity, MIB-1 SI, and recurrence; therefore, the presence of p53 protein by immunohistochemical examination may suggest the proliferative activity of meningioma and is capable of serving as a predictor of future recurrence.
Antibodies, Monoclonal↗
Syringomyelia associated with Hajdu-Cheney syndrome: case report.
OBJECTIVE AND IMPORTANCE: Hajdu-Cheney syndrome is a rare idiopathic bone disease based on a generalized bone dysplasia accompanied by acro-osteolysis. We describe a surgical case of this syndrome that was accompanied by neurological signs associated with cervical syringomyelia. CLINICAL PRESENTATION: A 41-year-old woman was referred to our hospital with mild quadriparesis and sensory disturbance resulting from a car accident. There was a neck injury. She showed almost all of the major characteristic clinical features and roentgenographic findings of Hajdu-Cheney syndrome with syringomyelia. INTERVENTION: Surgical treatment was indicated because of the progressive neurological deficits. Foramen magnum decompression and C1 laminectomy were performed, and the dura was exposed. The dura was opened at the area of the foramen magnum and C1. The occipitocervical posterior fusion was carried out with an iliac bone graft and titanium wires. CONCLUSION: Postoperatively, quadriparesis and sensory disturbance improved and the patient showed improved ambulation. Magnetic resonance imaging disclosed the well-decompressed foramen magnum. The syringomyelia disappeared in the segmental area of C2 and was decreased in the segmental areas of C5-T6. The treatment of this syndrome is symptomatic. In this patient, magnetic resonance imaging disclosed compression of the brain stem by basilar invagination and platybasia, disturbance of cerebrospinal fluid flow at the level of the foramen magnum, and syringomyelia. It was suspected that the obstruction of cerebrospinal fluid flow at the level of foramen magnum caused the cervical syringomyelia. However, the long-term prognosis remains uncertain. Follow-up is necessary to assess the final result of the treatment.
Adult↗
Three novel AVPR2 mutations in three Japanese families with X-linked nephrogenic diabetes insipidus.
We identified three novel mutations of the arginine vasopressin (AVP) V2 receptor (AVPR2) gene in Japanese families with X-linked congenital nephrogenic diabetes insipidus (NDI). In kindred #1 of siblings, a single base deletion of one out of three guanosines (nucleotides 786-788, 786delG) was detected. This deletion shifts the reading frame with an altered amino acid sequence and introduces a premature stop codon (TGA) at position 270. In kindred #2 of siblings and one unrelated additional patient (patient #3), point mutations that change the same Pro residue at codon 322 in the seventh transmembrane domain to either a Ser or His (P322S or P322H) were detected. This P322 residue is well conserved among rat V1 and V2 receptors, the human oxytocin receptor, and other G protein-coupled receptors, and is thought to be important for proper insertion of the receptor into the membrane. The AVPR2 mutations are heterogeneous both in Japanese and Caucasians populations.
Arginine Vasopressin↗
Insulin-like growth factors (IGFs) and IGF-binding proteins (IGFBP-1, -2 and -3) in diabetic pregnancy: relationship to macrosomia.
To evaluate the role of insulin-like growth factors (IGFs) and IGF-binding proteins (IGFBPs) in excessive fetal growth (macrosomia) in diabetic pregnancy, 84 insulin-treated diabetic mothers and their infants were tested for serum concentrations of IGF-I, IFG-II, and IGFBP-1, -2 and -3. These parameters were correlated with the birth weight of neonates and placental weight. IGF-I and II levels were determined by specific radioimmunoassays (RIAs) after serum samples were extracted with aid-ethanol. IGFBPs were measured by Western immunoblot with specific antibodies to the respective IGFBP species. Serum concentrations of both IGF-I and IGF-II in mothers with either IDDM or NIDDM increased with the gestational period, reached a plateau at the third trimester, and returned to non-pregnant levels within 7 days after delivery. These values were not different from those in normal mothers before and throughout pregnancy. As previously reported, IGF-I concentrations in cord serum of neonates born to diabetic mothers were (P < 0.01) higher than those of newborns of normal mothers. Likewise, cord blood IGF-II levels were 2-fold higher in babies of diabetic mothers (P <0.001). Fetal IGF-I and IGF-II correlated with each other and with maternal HbA1C, and they positively correlated with either birth weight or placental weight. Cord IGFBP-3 concentrations were significantly higher in diabetic pregnancy, but IGFBP-2 concentrations were not different from those in normal pregnancy. Cord IGFBP-1 concentrations were significantly higher only in babies of mothers with IDDM. None of these cord IGFBP concentrations correlated with birth weight or placental weight. The data suggest that fetal IGF-II, like IGF-I, is involved in fetal and placental growth in diabetic pregnancy. The role of IGFBPs remained to be determined.
Adult↗