Moyamoya disease and transforming growth factor-beta1.
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Adolescent↗
Biomedical subjects
M Fukui
Publications and source records attributed to M Fukui.
[T-cell lymphoproliferative disorder involving the central nervous system in two cases].
Lymphoproliferative disorder (LPD) sometimes occurs in immunosuppressive patients. B-cell proliferation is predominantly seen in patients with LPD and T-cell proliferation is rare. We here report two patients with T-cell LPD involving the central nervous system (CNS). A 7-year-old boy developed increased intracranial pressure and computed tomography showed a ring-enhanced mass lesion in the right frontal lobe. A 10-year-old boy developed gait disturbance and magnetic resonance imaging showed a non-enhanced lesion in the left parietal lobe. Radiological findings could not differentiate CNS-LPD from other intracranial diseases. Only histologic examination could diagnose LPD in both cases, and Epstein-Barr virus associated RNA was found in the infiltrated T-cell of the latter case. Although chemotherapy with antitumor agents was effective for remission of LPD in both cases, both patients died 3 years after their operations. As CNS-LPD shows rapid progression and frequently results in fatality, this disease requires immediate histologic diagnosis followed by extensive chemotherapy.
Central Nervous System↗
[Surgical treatment for temporal lobe epilepsy in childhood].
We retrospectively analyzed 5 children (11-15 year) with intractable temporal lobe epilepsy (TLE) who underwent the anterior temporal lobectomy with hippocampectomy. Cases 1-3 had medial TLE (MTLE) with histologically verified hippocampal sclerosis, Case 4 had lateral TLE, and Case 5 had MTLE with old hemorrhagic lesion in the lateral temporal lobe. In Cases 3-5, chronic invasive electrocorticography recording using subdural electrodes was obtained, while in Cases 1 and 2, the epileptogenic region was defined by noninvasive preoperative evaluation. Postoperatively, Cases 1-3 became seizure free. All patients had psychosocial problems after the onset of their epilepsy, which was not improved even after the surgical control of epilepsy. Since most patients had morphological change and perfusional and metabolic disturbance outside the hippocampus at the time of surgery, earlier surgical consideration may be necessary.
Adolescent↗
[Medulloblastoma with neuronal differentiation: a report of five cases].
This report presents a retrospective analysis of 5 patients who were treated for cerebellar medulloblastoma with neuronal differentiation. Four males and 1 female ranged in age from 6 months to 9 years at the time of diagnosis. Total removal of the tumor was achieved in 3 patients, and partial removal in 2. While these tumors were composed of small cells and had regions resembling desmoplastic medulloblastoma, they in part showed neuronal characteristics which included parallel row or linear array arrangements of tumor cells in an eosinophilic fibrillary matrix. Postoperatively, 3 patients received craniospinal radiation therapy, one received local radiation to the primary site, and the remaining one received only systemic chemotherapy. During the follow-up period of 3.8-25.2 years, 4 patients have been in continuous remission with mild to moderate neurological deficits, while the remaining one died 3.9 years after surgery. The clinical and anatomic pathological features of medulloblastomas with neuronal differentiation are reviewed while the therapeutic problems associated with these tumors are also discussed.
Cerebellar Neoplasms↗
[Usefulness of ultrasonography in a case of spontaneous carotid-cavernous fistula: a case report].
Since spontaneous carotid-cavernous fistula (CCF) is a dural arterio-venous fistula at the cavernous sinus, which is different from traumatic CCF and CCF associated with a ruptured aneurysm at the cavernous internal carotid artery, cerebral angiography is required in order to differentiate these condition. We here report a case of spontaneous CCF, in which a result of ultrasonographic evaluation of cervical arteries well corresponded with that of cerebral angiography. Ultrasonography showed increased blood flow and decreased pulsatility index in the ipsilateral external carotid artery, contralateral internal and external arteries, and these values in all arteries resolved within normal range after the interventional embolization. Ultrasonography is less invasive examination and can be easily performed even in outpatients for observation of spontaneous CCF.
Blood Flow Velocity↗
[Occipital encephalocele and epilepsy].
We report the incidence of epilepsy in 7 patients with occipital encephalocele (meningocele: 6 cases, meningoencephalocystocele: 1 case). Two cases had epilepsy and a case without the history of epileptic seizure had an epileptic pattern on the EEG. All these 3 cases had cortical dysplasia(CD) such as schizencephaly and subcortical gray matter, and two of them were associated with mental retardation. The location of the prominent CD was correlated with the EEG abnormalities and clinical epileptic pattern in each case. Associated hydrocephalus and placement of VP shunt had no significant influence on the occurrence of the epilepsy. Thus, complicated CD is thought to be epileptogenic in patients with occipital encephalocele.
Adolescent↗
[A case of effective urgent irradiation therapy for metastatic spine tumor].
A 74-year-old man noticed weakness in bilateral lower limbs 2 months prior to admission. The weakness had rapidly progressed and he could not stand by himself on admission. All thoracic vertebral bodies showed mosaic pattern on T 2 weighted-image. At Th 10 and Th 11, two enhanced masses compressed spinal cord posteriorly. Basal-serum tumor markers, such as LDH, ALP, total-acid phosphatase and prostatic acid phosphatase were elevated, metastatic spine tumor from prostatic carcinoma was suspected. Biopsy specimen from its prostate revealed low differentiated adenocarcinoma. Because sphincter dysfunction progressed rapidly after admission, we started 3 Gy/day irradiation within 24 hr from its appearance targeting both Th 10 to L 1 vertebral bodies and the prostate in total 30 Gy. The clinical symptoms started to resolve immediately, and the disappearance completely within 7 months. This case illustrated that urgent irradiation was effective for broad metastatic spinal tumor with rapidly progressive neurological deficits.
Adenocarcinoma↗
[Small epidermoid induced trigeminal neuralgia unrecognized by conventional CT and MRI for over 25 years].
A 65-year-old woman had been conservatively treated as idiopathic trigeminal neuralgia for over 25 years, because conventional computed tomography (CT) and magnetic resonance imaging(MRI) showed no abnormality in the cerebello-pontine(CP) angle cistern. She received a detailed MRI by constructive interference in steady state and diffusion weighted image(DWI) sequences. Those sequences on MRI well demonstrated a epidermoid tumor in the CP angle cistern, and the removal of the tumor completely resolved the neuralgia. Since a small epidermoid in the CP angle cistern seems to be unrecognized by conventional CT and MRI, detailed evaluation by DWI sequence, which has been widespread recently, is required for patients with trigeminal neuralgia.
Aged↗
[Relationship between cardiac natriuretic peptide (ANP/BNP) and fluid intake in patients with subarachnoid hemorrhage].
Several authors described elevated natriuretic peptides, atrial natriuretic peptide(ANP) and brain natriuretic peptide (BNP), in patients with subarachnoid hemorrhage(SAH), which were account for inappropriate antidiuretic hormone(SIADH) or cerebral salt wasting syndrome(CSW). Although the secretion of natriuretic peptide depends on the total blood volume, central venous pressure, and cardiac output volume, the volume of fluid intake in patients with SAH had not been taken in consideration in previous report. We here examined the relationship between fluid intake and the natriuretic peptides in two cases without cardiac failure. ANP elevated 2 or 3 days after SAH and remained in normal range for 2 weeks. BNP elevated when the volume of fluid intake was increased, and BNP did not elevate during the periods with lower fluid intake. Several authors proposed the possibility of iatrogenic factor in natriuresis after SAH and these results supported this opinion.
Adult↗
cDNA cloning, expression and characterization of human prostaglandin F synthase.
A cDNA clone of prostaglandin F synthase (PGFS) was isolated from human lung by using cDNA of bovine lung-type PGFS as a probe and its protein expressed in Escherichia coli was purified to apparent homogeneity. The human PGFS catalyzed the reduction of prostaglandin (PG) D2, PGH2 and phenanthrenequinone (PQ), and the oxidation of 9alpha,11beta-PGF2 to PGD2. The kcat/Km values for PGD2 and 9alpha,11beta-PGF2 were 21000 and 1800 min(-1) mM(-1), respectively, indicating that the catalytic efficiency for PGD2 and 9alpha,11beta-PGF2 was the highest among the various substrates, except for PQ. The PGFS activity in the cytosol of human lung was completely absorbed with antihuman PGFS antiserum. Moreover, mRNA of PGFS was expressed in peripheral blood lymphocytes and the expression in lymphocytes was markedly suppressed by the T cell mitogen concanavalin A. These results support the notion that human PGFS plays an important role in the pathogenesis of allergic diseases such as asthma.
Amino Acid Sequence↗
Solitary fibrous tumor in neurosurgical practice.
Solitary fibrous tumors most often affect the pleura, but examples are increasingly being reported in a wide variety of sites including the central nervous system. This tumor shows characteristic expression of CD34, which facilitates histopathologic differentiation of this lesion from other more common and better recognized spindle-cell tumors such as fibrous meningioma, hemangiopericytoma, or nerve sheath tumors. In this paper, we review current information on cranial and paracranial solitary fibrous tumors and emphasize the need for clinical recognition of this lesion as a distinct entity.
Journal Article↗
Severe and recurrent fasting hypoglycemia due to growth hormone deficiency?
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Aged↗
Distinct pattern of PCR-SSCP analysis of p53 mutations in human astrocytomas.
Clarification of somatic mutations during the progression of human astrocytomas is important in order to understand the mechanisms underlying the development of these tumors. We analyzed surgical specimens of human astrocytomas for mutations in the p53 gene using single-strand conformation polymorphism analysis of polymerase chain reaction product (PCR-SSCP analysis) at a low pH. Klenow fragment treatment after PCR amplification was an effective means to get rid of some extra bands on the SSCP gel. Five mutations in three of 24 astrocytomas were identified by this improved SSCP method. The frequency of p53 gene mutations in astrocytomas examined was 12.5%. Further examination by direct sequencing showed that all five mutants had single-base substitutions resulting in missense mutations. The present studies revealed a loss of heterozygosity and two point mutations on the remaining allele in one of the fibrillary astrocytomas. Finally, the improvement of PCR-SSCP analysis using Klenow treatment and low pH showed a distinct electrophoresis gel pattern and could be relevant for the prognosis of human astrocytomas.
Astrocytoma↗
Characterization of a renal medium chain acyl-CoA synthetase responsible for glycine conjugation in mouse kidney mitochondria.
Glycine conjugation of a series of benzoic acid derivatives was investigated in mouse kidney mitochondria. The chlorine and methyl substitutions in the para- and meta-positions of the benzene ring yielded an increase in glycine conjugation. The acids with a methoxy group showed a low degree of glycine conjugation. In addition, the acids with nitro or amino groups were conjugated to a slight extent with glycine. The in vitro conjugation of salicylic acid with glycine occurred not in liver but in kidney. The specificity of the renal medium chain acyl-CoA synthetase catalyzing the first reaction of glycine conjugation was also examined. The enzyme accepted not only medium chain fatty acids but also aromatic and arylacetic acids. The highest activity was shown with hexanoic acid. High activities were observed for benzoic acid derivatives with alkyl and alkoxyl groups in the para- and meta-positions of the benzene ring. An ortho-substituted acid exhibited no activity. In addition, the enzyme was less active with valproic acid, tranexamic acid, indomethacin and ketoprofen. The enzyme was inhibited by diflunisal, 2-hydroxydodecanoic acid and salicylic acid, which did not act as substrates. There was a poor correlation between the activity of the medium chain acyl-CoA synthetase and glycine conjugation of eleven substituted benzoic acids. These findings suggest that the present medium chain acyl-CoA synthetase is involved in glycine conjugation of the substituted acids in mouse kidney mitochondria, but there may be a larger contribution of another isoenzyme.
Animals↗
Physiology, phylogenetic relationships, and ecology of filamentous sulfate-reducing bacteria (genus desulfonema)
Microscopy of organic-rich, sulfidic sediment samples of marine and freshwater origin revealed filamentous, multicellular microorganisms with gliding motility. Many of these neither contained sulfur droplets such as the Beggiatoa species nor exhibited the autofluorescence of the chlorophyll-containing cyanobacteria. A frequently observed morphological type of filamentous microorganism was enriched under anoxic conditions in the dark with isobutyrate plus sulfate. Two strains of filamentous, gliding sulfate-reducing bacteria, Tokyo 01 and Jade 02, were isolated in pure cultures. Both isolates oxidized acetate and other aliphatic acids. Enzyme assays indicated that the terminal oxidation occurs via the anaerobic C(1) pathway (carbon monoxide dehydrogenase pathway). The 16S rRNA genes of the new isolates and of the two formerly described filamentous species of sulfate-reducing bacteria, Desulfonema limicola and Desulfonema magnum, were analyzed. All four strains were closely related to each other and affiliated with the delta-subclass of Proteobacteria. Another close relative was the unicellular Desulfococcus multivorans. Based on phylogenetic relationships and physiological properties, Strains Tokyo 01 and Jade 02 are assigned to a new species, Desulfonema ishimotoi. A new, fluorescently labeled oligonucleotide probe targeted against 16S rRNA was designed so that that it hybridized specifically with whole cells of Desulfonema species. Filamentous bacteria that hybridized with the same probe were detected in sediment samples and in association with the filamentous sulfur-oxidizing bacterium Thioploca in its natural habitat. We conclude that Desulfonema species constitute an ecologically significant fraction of the sulfate-reducing bacteria in organic-rich sediments and microbial mats.
Journal Article↗
Melanotic neuroectodermal tumour of infancy at the anterior fontanelle.
We describe a 4-month-old girl presenting with a melanotic neuroectodermal tumour of infancy at the anterior fontanelle. According to the neuroimaging findings, this tumour was found to lie epidurally, adherent to the dura mater, with thickening of the adjacent frontal bone. The tumour was dense on CT, while MRI showed a major part of the tumour to be isointense with cerebral cortex on both T1- and T2-weighted images. The neuroimaging and clinical features are briefly discussed.
Cerebral Cortex↗
Serial MRI of cerebral infarcts before and after removal of an atrial myxoma.
We describe a cerebral infarct caused by atrial myxoma. A 30-year-old woman with an atrial myxoma presented with a right hemisensory deficit. MRI carried out before and after removal of the myxoma, showed multiple small bilateral white-matter infarcts which increased in number up to the surgery. A lesion in the left thalamus, which enlarged and showed contrast enhancement 4 months before surgery, resembled an old cerebral infarct by the time the myxoma was removed. The number of lesions stopped increasing after surgery. We suggest that atrial myxomas should be removed even in asymptomatic patients, to prevent cerebral infarcts due to embolism of tumour or thrombus.
Adult↗
Possible role of an endovascular provocative test in the diagnosis of glossopharyngeal neuralgia as a vascular compression syndrome.
We utilized endovascular provocative techniques to identify the indications for microvascular decompression surgery in a serious case of glossopharyngeal neuralgia. This is the first reported case in which an endovascular provocative test was applied for diagnosis of glossopharyngeal neuralgia as a vascular compression syndrome. A 68-year-old woman presented with severe paroxysmal facial pain which could not be controlled by medical therapy. Partial effectiveness to carbamazepine led us to wonder whether or not the selection of microvascular decompression surgery would be appropriate. Pre-operative angiography was performed. During the examination a microcatheter was inserted into the right posterior inferior cerebellar artery (PICA), and an attack of typical glossopharyngeal neuralgia occurred. The patient thus underwent microvascular decompression surgery. The PICA was verified to compress the glossopharyngeal nerve and therefore was moved to induce decompression. The patient has since experienced no further pain for one year postoperatively. The diagnosis of glossopharyngeal neuralgia is sometimes complex and it is difficult to select the most appropriate surgical modality. In such cases this endovascular provocative technique may thus be useful for making a definitive decision or microvascular decompression surgery.
Aged↗