Balloon angioplasty in a patient with Kabuki make-up syndrome.
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Abnormalities, Multiple↗
Biomedical subjects
Y Inoue
Publications and source records attributed to Y Inoue.
Interictal and ictal magnetoencephalographic study in patients with medial frontal lobe epilepsy.
PURPOSE: To determine whether magnetoencephalography (MEG) has any clinical value for the analysis of seizure discharges in patients with medial frontal lobe epilepsy (FLE). METHODS: Four patients were studied with 74-channel MEG. Interictal and ictal electroencephalographic (EEG) and MEG recordings were obtained. The equivalent current dipoles (ECDs) of the MEG spikes were calculated. RESULTS: In two patients with postural seizures, interictal EEG spikes occurred at Cz or Fz. The ECDs of interictal MEG spikes were localized around the supplementary motor area. In the other two patients with focal motor or oculomotor seizures, interictal EEG spikes occurred at Fz or Cz. The ECDs of interictal MEG spikes were localized at the top of the medial frontal region. The ECDs detected at MEG ictal onset were also localized in the same area as those of the interictal discharges. CONCLUSIONS: In medial FLE patients, interictal and ictal MEG indicated consistent ECD localization that corresponded to the semiology of clinical seizures. Our findings demonstrate that MEG is a useful tool for detecting epileptogenic focus.
Adolescent↗
Photosensitivity in relation to epileptic syndromes: asurvey from an epilepsy center in Japan.
PURPOSE: We examined the incidence and distribution of photosensitivity among the different age groups and different types of epilepsies and epileptic syndromes. Furthermore, we considered the influence of ethnic and geographic factors on the incidence of photoparoxysmal response (PPR) in epilepsy patients. METHODS: We analyzed the responses to intermittent photic stimulation (IPS) by using a Grass PS22 or PS33 photic stimulator for in 2,187 unselected patients with epilepsy who were treated in our center. RESULTS: The classic PPR was elicited in 37 (1.7%) patients. The mean age of these 37 patients was 17.0 years. The subpopulation of patients having PPR included 2.0% of all patients with symptomatic generalized epilepsy. 5.6% (p < 0.01) of those with idiopathic generalized epilepsy, 0.7% of those with symptomatic localization-related epilepsy, and 2.9% of those with undetermined epilepsy. PPR accounted for 17.4% (p < 0.01) of the patients with juvenile myoclonic epilepsy, 7.6% (p < 0.01) of those with grand mal on awakening, and 6.1% (p < 0.01) of those with symptomatic occipital lobe epilepsy. The incidence of PPR increased in patients up to age 15 years, and suddenly decreased after age 20 years. CONCLUSION: The present study presents the first report from eastern Asia, analyzing the incidence of PPR with a restricted definition comparable to the other studies, and the rate of PPR was relatively low compared with the studies performed in the European countries. We could confirm the clear relation between age and positive PPR.
Adolescent↗
Acute psychosis during intracranial EEG monitoring: close relationship between psychotic symptoms and discharges in amygdala.
PURPOSE: This report examined the underlying mechanism of psychosis associated with epilepsy. METHODS: An adult patient with epilepsy manifesting acute psychosis during long-term EEG monitoring is presented, together with a literature review on this subject. RESULTS: A 25-year-old woman with intractable temporal lobe epilepsy developed acute psychosis while she underwent long-term intracranial EEG monitoring. After a clustering of seizures, she manifested psychotic symptoms including hallucination, stupor, and repeated fear. The transition of psychotic symptoms corresponded to the changes in frequency and morphology of seizure discharges restricted to the left amygdala. Improvement of psychosis coincided with disappearance of seizure discharges. CONCLUSIONS: This case confirmed a close relationship between psychotic symptoms and seizure discharges in the left amygdala. It is suggested that paroxysmal bombardment of the medial temporal lobe structure may be a pathogenetic factor of acute psychosis associated with epilepsy.
Acute Disease↗
Epithelial-myoepithelial carcinoma of the palate: a case report.
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Aged↗
Troglitazone prevents the rise in visceral adiposity and improves fatty liver associated with sulfonylurea therapy--a randomized controlled trial.
Monotherapy with sulfonylurea may result in the exhaustion of pancreatic beta-cell function, fat accumulation, and dyslipidemia. We examined the possibility of dose reduction by administering sulfonylurea together with troglitazone, and investigated changes in insulin secretion and fat deposition. Seventy-eight patients with type 2 diabetes adequately controlled with glibenclamide were randomly allocated to a troglitazone (400 mg/d)-added group (n = 40) or a control group without placebo (n = 38) and monitored for 24 weeks. The daily dose of glibenclamide was adjusted to maintain stable HbA(1c) levels. Fat accumulation to the liver and thigh muscle were measured in mean Hounsfield units determined on computed tomography (CT) scan. Visceral fat accumulation (V), subcutaneous fat accumulation (S), and the V/S ratio were also determined by CT scan. The daily dose of glibenclamide and serum fasting insulin level in the troglitazone-added group significantly decreased (from 4.05 +/- 2.50 mg/d to 1.84 +/- 1.65 mg/d and from 8.47 +/- 4.62 microU/mL to 6.49 +/- 3.28 microU/mL, respectively) during the observation period compared with the control group (P < .01 and P < .01, respectively). Serum triglyceride and homeostasis model insulin resistance index (HOMA-R) in the troglitazone-added group decreased significantly in comparison to the control group (P < .05 and P < .01, respectively). The mean Hounsfield units of liver significantly decreased in the control group compared with the troglitazone-added group (P < .05). Visceral fat area and the V/S ratio significantly increased in the control group compared with the troglitazone-added group (P < .01 and P < .01, respectively). Glibenclamide monotherapy resulted in fat accumulation accompanied by dyslipidemia. An alternate conclusion is that troglitazone reversed type 2 diabetes (not sulfonylurea)-associated fat accumulation. The addition of troglitazone decreased daily doses of glibenclamide, preserved fasting insulin secretion, improved fat accumulation in liver, and prevented dyslipidemia.
Adipose Tissue↗
Surgical management of syringomyelia associated with spinal adhesive arachnoiditis.
The authors describe a new surgical technique to minimise the postoperative recurrence of adhesion after microlysis of adhesion to treat syringomyelia associated with spinal adhesive arachnoiditis. A 47 year old male presented with numbness of the lower extremities and urinary disturbance and was demonstrated to have a case of syringomyelia from C1 to T2 which was thought to be secondary to adhesive spinal arachnoiditis related to a history of tuberculous meningitis. Following meticulous microlysis of the adhesions, maximal expansion of a blocked subarachnoid space was performed by expansive duraplasty with a Gore-Tex surgical membrane, expansive laminoplasty and multiple tenting sutures of the Gore-Tex graft. Postoperatively, the syringomyelia had be en completely obliterated and improvement of the symptoms had been also achieved. The technique described may contribute to improvement of the surgical outcome following arachnoid dissection by maintaining continuity of the reconstructed subarachnoid space.
Arachnoiditis↗
Possible clinical and histologic manifestations of adult-onset type II citrullinemia in early infancy.
We describe 2 patients with adult-onset type II citrullinemia who developed transient hypoproteinemia and jaundice in early infancy. Liver histology showed a marked fatty change and fibrosis. After the patients had lived without symptoms to the ages of 5 and 16 years, respectively, the diagnosis was made by genetic analysis.
Adolescent↗
Corneal triple procedure.
Corneal diseases subject to keratoplasty are often combined with a certain degree of cataract. Therefore, appropriate strategies for the management of cataract are mandatory. One strategy is a sequential procedure performing cataract surgery after keratoplasty. Though this procedure enables us to estimate IOL power accurately, there are several drawbacks including multiple procedures, slow visual rehabilitation and endothelial damage during cataract surgery. On the other hand, triple procedure, consisting of penetrating keratoplasty, extracapsular lens extraction and IOL implantation, has several advantages, such as single procedure, rapid visual rehabilitation and no additional endothelial trauma. How-ever, open sky procedure may be accompanied by uncontrollable vitreous pressure followed by posterior capsule rupture and difficulty of IOL implantation, while the worst outcome may be expulsive hemorrhage. Combined core vitrectomy preceding corneal trephination is contrived to solve this problem. This procedure is simply performed from a single sclerotomy 3.5-4.01 mm posterior to the corneal limbus with a vitreous cutter under the external pressure to the eye, and vitreous pressure is sufficiently lowered to allow the following procedures to be done very safely. Another problem with triple procedure is the calculation of IOL power. This problem is caused by unpredictable keratometer readings after keratoplasty. Several methods for calculating IOL power in triple procedure are also discussed.
Cataract Extraction↗
Quality of life of vestibular schwannoma patients after surgery.
The quality of life of vestibular schwannoma (VS) patients after surgery was investigated. The subjects consisted of 236 unilateral VS patients who underwent tumor removal between 1990 and 1997. A questionnaire was sent to all patients regarding their hearing, tinnitus, dizziness and the changes in their daily life after surgery; 176 out of 204 patients (86%) who received the questionnaire completed and returned it. The answers were compared with recent data reported in other clinical studies. Ninety percent of the patients with postoperative class A hearing were satisfied with their hearing. However, only 30% of patients with postoperative class B hearing were satisfied. Tinnitus worsened after surgery more often in patients who underwent a labyrinthectomy than in those who did not. Dizziness improved after surgery in the majority of VS patients. However, 30% of patients had difficulty driving a car and 50% of patients could not enjoy activities such as playing sport after surgery.
Facial Nerve↗
Amatoxin poisoning from ingestion of Japanese Galerina mushrooms.
BACKGROUND: Although some Japanese Galerina species poisonings manifest as gastrointestinal symptoms followed by late-onset hepatorenal failure (phalloides syndrome), the toxin responsible for this has not been determined. CASE REPORT: We report a 6-year-old boy who developed characteristic cholera-like diarrhea and late-onset severe hepatic deterioration after eating mushrooms, later identified as a Galerina species, most likely Galerina fasciculata. A residual mushroom revealed alpha-amanitin. This account is the first known reported case of poisoning by Japanese Galerina species where an amatoxin was demonstrated to be responsible for the toxicity.
Agaricales↗
Association of structural polymorphisms in the human period3 gene with delayed sleep phase syndrome.
Recent progress in biological clock research has facilitated genetic analysis of circadian rhythm sleep disorders, such as delayed sleep phase syndrome (DSPS) and non-24-h sleep-wake syndrome (N-24). We analyzed the human period3 (hPer3) gene, one of the human homologs of the Drosophila clock-gene period (Per), as a possible candidate for rhythm disorder susceptibility. All of the coding exons in the hPer3 gene were screened for polymorphisms by a PCR-based strategy using genomic DNA samples from sleep disorder patients and control subjects. We identified six sequence variations with amino acid changes, of which five were common and predicted four haplotypes of the hPer3 gene. One of the haplotypes was significantly associated with DSPS (Bonferroni's corrected P = 0.037; odds ratio = 7.79; 95% CI 1.59-38.3) in our study population. Our results suggest that structural polymorphisms in the hPer3 gene may be implicated in the pathogenesis of DSPS.
Adolescent↗
An ultrasensitive chemical method for polysialic acid analysis.
An ultrasensitive method for analysis of polysialic acid (polySia) chains, using fluorescence-assisted high-performance liquid chromatography was developed. The new method is a substantial improvement of our earlier method in which the reducing terminal Sia residues of a homologous series of oligo/polySia hydrolytically released during derivatization reaction were simultaneously labeled with a fluorogenic reagent, 1,2-diamino-4,5-methylenedioxybenzene (DMB) in situ. We first studied extensively the stability of oligo/polySia in the acid (0.02 M trifluoracetic acid) used for 1,2-diamino-4,5-methylenedioxybenzene derivatization under various conditions of reaction time and temperature, analyzing the hydrolytic products by high-performance anion exchange chromatography with pulsed electrochemical detection (HPAEC-PED). Then we optimized the reaction conditions to minimize degradation of the parent polySia while maintaining high derivatization rate. Using a DNAPac PA-100 column rather than a MonoQ column, baseline resolution of polySia peaks up to DP 90 with a detection threshold of 1.4 femtomol per resolved peak was achieved. The new method was used to analyze the degree of polymerization of a polySia-containing glycopeptide fraction derived from embryonic chicken brain, and the results were compared with those obtained by HPAEC-PED.
Animals↗
Myxoid liposarcoma metastatic to the thoracic epidural space without bone involvement: report of two cases.
Myxoid liposarcoma can frequently metastasize to extrapulmonary sites. We present two cases of myxoid liposarcoma metastatic to the epidural space. Both patients complained of back pain, but plain radiography revealed no abnormality. MR imaging clearly demonstrated metastatic tumors in the epidural space, but no involvement of vertebra. When patients with myxoid liposarcoma complain of back pain, metastasis in the epidural space should be considered even in patients without bone involvement.
Adult↗
Conformation and recognition control of peptide ribonucleic acid containing pyrimidine/purine mixed sequence.
Peptide ribonucleic acids (PRNA), tethering 5'-amino-5'-deoxyribonucleoside as a recognition moiety for RNA/DNA, have been designed and synthesized, as a novel nucleic acid model. External switching of the recognition behavior of PRNA containing not only pyrimidine nucleobase but also purine nucleobase, with complementary oligomeric DNA/RNA has been demonstrated for the first time through both the orientational switching of the nucleobase of PRNA and electrostatic repulsion between borate ester anion of PRNA and phosphodiester anion of DNA/RNA, induced by added borates.
Base Sequence↗
Identification of functional domains of the extrinsic 12 kDa protein in red algal PSII by limited rroteolysis and directed mutagenesis.
The extrinsic 12 kDa protein in red algal photosystem II (PSII) functions to minimize the chloride and calcium requirement of oxygen-evolving activity [Enami et al. (1998) Biochemistry 37: 2787]. In order to identify functional domains of the 12 kDa protein, we prepared the 12 kDa protein lacking N-terminal peptides or C-terminal peptides or both by limited proteolysis and directed mutagenesis. The resulting 12 kDa protein fragments were examined for their binding and functional properties by reconstitution experiments. (1) A peptide fragment from Gly-6 to C-terminus of the 12 kDa protein was prepared by V8 protease. This fragment rebound to PSII completely, and it reactivated oxygen evolution partially in the absence of Cl(-) and Ca(2+) ions but significantly in the presence of Cl(-) ion. (2) A peptide from Leu-10 to Phe-83 was obtained by chymotrypsin treatment. This peptide rebound to PSII effectively, but the rebinding did not restore oxygen evolution in both the absence and presence of Cl(-) and Ca(2+) ions. (3) Two mutant proteins, one lacking five residues and the other lacking nine residues of the N-terminus, were able to bind to PSII effectively. Recovery of oxygen evolution by their binding was almost the same as that reconstituted with the V8 protease-treated peptide. (4) Three mutant proteins lacking ten, seven or three residues of the C-terminus effectively rebound to PSII, but their binding did not result in recovery of the oxygen evolution. In contrast, reconstitution with a mutant protein lacking one residue of the C-terminus showed the same high restoration of oxygen evolution as reconstitution with the full-length 12 kDa protein. (5) These results indicate that two residues from lysine of the C-terminus of the 12 kDa protein constitute an important domain for minimizing the chloride and calcium requirement of oxygen evolution. In addition, the N-terminus of the protein, at least five residues, has a secondary function for the chloride requirement.
Algal Proteins↗
Reliable, minimally invasive oromandibular reconstruction using metal plate rolled with pectoralis major myocutaneous flap.
The purpose of this study was to minimize the surgical invasiveness to the donor site and the amount of the primary reconstruction time after oromandibular tumor resection. Oromandibular reconstruction was performed only using a pectoralis major myocutaneous flap and a metal plate. The pectoralis major myocutaneous flap was grafted to the oral cavity defect by rolling and wrapping around the metal plate with the muscle of the flap. No early postoperative complications have been noted in all seven patients. An average of 2 years and 1 month has past since surgery, and to date no infections, plate exposure, or plate breakage have been observed in any of the patients. The safety of the oromandibular reconstruction using a metal plate was improved by rolling the muscle of the pectoralis major myocutaneous flap around the metal plate. The present method was shown to be a rational technique that allowed primary reconstruction of the oral cavity and mandible in a minimally invasive manner in a short time.
Adult↗
Avid Ga-67 uptake in a breast metastasis from cancer of the tongue.
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Breast Neoplasms↗