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Biomedical subjects

T Negoro

Publications and source records attributed to T Negoro.

At least 37 records · Page 2Linked to original sources

Necrotizing toxoplasmic encephalitis in a child with the X-linked hyper-IgM syndrome.

UNLABELLED: We report on a 9-year-old boy with the hyper-IgM syndrome who presented with rapid impairment of consciousness. The brain CT scan showed multiple round lucencies, and the brain histology revealed necrotizing toxoplasmic encephalitis. This patient, whose CD40/CD40 ligand system was impaired, indicates the importance of this system for defence against toxoplasmic infection. CONCLUSION: Although disseminated toxoplasmosis is a rare complication of the hyper-IgM syndrome, it must be included in the differential diagnosis of infections.

Child↗

Benign myoclonic epilepsy in infants: video-EEG features and long-term follow-up.

We report video-EEG findings and a long-term follow-up study in 10 patients with benign myoclonic epilepsy in infants (BMEI). A high incidence of a past and family history of febrile convulsions was noted. Six of the 10 patients manifested characteristic vocalization associated with myoclonic seizures. It consisted of a sudden, brief expiratory noise and is considered to be characteristic of BMEI. Afebrile convulsions occurred before the onset of myoclonic seizures or during the clinical course in six patients, but the accurate type of these seizures remains to be clarified. Monotherapy with valproatic acid (VPA) was very effective, but plasma VPA levels over 100 micrograms/ml were initially necessary in most of the cases, although they did not need to be maintained for further seizure control. All patients showed a favorable long-term seizure outcome, although one showed moderate mental retardation.

Adolescent↗

Successful polymerase chain reaction-based diagnosis of fungal meningitis in a patient with chronic granulomatous disease.

Meningitis is not a common complication of chronic granulomatous disease (CGD). Here, we present details of a 3-year-old boy with X-linked CGD, who suffered from fungal meningitis. While 19 samplings using conventional cerebrospinal fluid (CSF) cultures failed to detect any organisms, fungal DNA was identified in the CSF by a new polymerase chain reaction (PCR)-based method. The patient recovered without any sequelae after treatment with a combination of antifungal agents, interferon-gamma and granulocyte infusions. This case report demonstrates that fungal meningitis must be included in the differential diagnosis of infections in CGD patients and that the PCR-based detection of fungal DNA is a powerful tool for diagnosis.

Child, Preschool↗

Evolutional changes and outcome of West syndrome: correlation with magnetic resonance imaging findings.

The prognosis and evolutional changes of 77 patients with West syndrome (WS) were studied after patients were classified into four groups on the basis of their magnetic resonance imaging (MRI) findings: anomaly, perinatal injury, normal, and the other groups. The average age at onset of spasms was earliest in the patients with anomalies and latest in patients with normal MRI findings. Patients with normal MRI findings had the shortest duration of spasms, and patients with anomalies had the longest duration of spasms. Antecedent seizures were observed in 6 patients (3 patients with anomalies, 1 patient with normal MRI findings, and 2 patients with other abnormalities). Thirty-five patients had subsequent seizures. Patients with anomalies often had partial seizures and patients with perinatal injuries often had generalized seizures. Seizures were infrequent in patients with normal MRI findings. Developmental outcome was best in the patients with normal MRI findings and worst in patients with perinatal injuries. Various types of epileptic syndromes occurred subsequent to WS in patients with anomalies, although nonspecific symptomatic generalized epilepsy was common in patients with perinatal injuries. These results suggest that seizure prognosis, evolutional changes in seizures, and developmental outcome are different among the types of brain lesions.

Adolescent↗

[Dramatic improvement with immunoabsorption therapy in a 7-year-old girl with severe Guillain-Barré syndrome after unsuccessful gammaglobulin therapy].

We report a 7-year-old girl with severe Guillain-Barré syndrome (GBS) who showed dramatic improvement after immunoabsorption therapy. She had progressive muscle weakness with resultant respiratory failure. On the 7th day of the illness, she was nearly quadriplegic and dependent on mechanical ventilation. In addition, she had autonomic manifestations, areflexia, abolished or delayed peripheral nerve conduction, and increased CSF protein. Administration of a high dose of gammaglobulin failed to improve the symptoms. Immunoabsorption therapy using a tryptophan column was performed 9 times during 17 days. After 5 times of immunoabsorption therapy, the muscle weakness improved dramatically; she was extubated 16 days after the institution of the therapy and walked without assistance on the 41st day. She regained normal muscle power without any sequelae. The immunoabsorption therapy caused hemolysis and decrease of serum fibrinogen, but no serious complications. The clinical course of this patient suggests the efficacy of immunoabsorption therapy in GBS.

Child↗

Structure and expression of human fibroblast growth factor-10.

We isolated the cDNA encoding a novel member of the human fibroblast growth factor (FGF) family from the lung. The cDNA encodes a protein of 208 amino acids with high sequence homology (95.6%) to rat FGF-10, indicating that the protein is human FGF-10. Human FGF-10 as well as rat FGF-10 has a hydrophobic amino terminus ( approximately 40 amino acids), which may serve as a signal sequence. The apparent evolutionary relationships of human FGFs indicate that FGF-10 is closest to FGF-7. Chromosomal localization of the human FGF-10 gene was examined by in situ hybridization. The gene was found to map to the 5p12-p13 region. Human FGF-10 (amino acids 40 to 208 with a methionine residue at the amino terminus) was produced in Escherichia coli and purified from the cell lysate. Recombinant human FGF-10 (approximately 19 kDa) showed mitogenic activity for fetal rat keratinizing epidermal cells, but essentially no activity for NIH/3T3 cells, fibroblasts. The specificity of mitogenic activity of FGF-10 is similar to that of FGF-7 but distinct from that of bFGF. In structure and biological activity, FGF-10 is similar to FGF-7.

Amino Acid Sequence↗

Severe myoclonic epilepsy in infancy: evolution of seizures.

Changes in seizure type of severe myoclonic epilepsy (SME) in infancy were reviewed retrospectively in 14 patients (11 males and 3 females) who were followed-up to the age of 7 years or more. The observation period ranged from 5 to 16 years with a mean of 10 years. During the follow-up, three or four types of seizures were seen per patient, but the pattern of appearance and disappearance of each seizure type varied considerably among the patients. Tonic-clonic convulsion, either generalized or unilateral, was seen most consistently through the entire course, and it continued to the end of follow-up in 11 patients (79%). On the contrary, myoclonic seizure, complex partial seizure, and atypical absence often disappeared and reappeared repeatedly during the course. In SME, seizure symptoms varied widely among patients in comparison with other neurological symptoms, and the most consistent core seizure type was tonic-clonic convulsions.

Child↗

Decrease of N-acetylaspartate after ACTH therapy in patients with infantile spasms.

Apparent brain atrophy has been frequently observed at CT and MRI after ACTH therapy in patients with infantile spasms. There are several hypotheses to explain ACTH-induced brain shrinkage: 1) a catabolic effect of ACTH on brain tissue, 2) a mineralocorticoid effect resulting in a loss of water and 3) an increase in cerebrospinal fluid (CSF) pressure compressing the brain. An average of 0.21 +/- 0.03 mg/kg of ACTH was administered to nine patients over a period of 14 to 17 days. Water content and concentrations of N-acetylaspartate (NAA), creatine and phosphocreatine (Cr + PCr), and choline (Cho) were measured before, immediately after, and several months after the ACTH therapy by using in-vivo 1H magnetic resonance spectroscopy (MRS). Only NAA concentration exhibited a significant change during the study (6.6 +/- 1.5 mmol/kg, 5.4 +/- 1.1, and 7.0 +/- 1.5, p = 0.017). There was no significant change in Cr + PCr, in Cho, or in water content. These data suggest catabolic effects of ACTH on brain tissue, such as cell loss, decrease in NAA synthesis in mitochondria, and leakage of NAA from cell membrane.

Adrenocorticotropic Hormone↗

Surgical treatment for pediatric moyamoya disease: use of the superficial temporal artery for both areas supplied by the anterior and middle cerebral arteries.

OBJECTIVE: To revascularize ischemic territories of both the anterior cerebral artery (ACA) and the middle cerebral artery (MCA), a simple and effective combined bypass operation was performed in 36 pediatric patients with moyamoya disease during the past 8 years. METHODS: The branches of the superficial temporal artery (STA) were used to revascularize the ACA and MCA territories. In children older than 5 years, the parietal branch of the STA was usually used for an end to side anastomosis with a cortical branch of the MCA. In children who were younger than 5 years, the parietal branch of the STA was used for an encephaloduroarteriosynangiosis instead of a direct anastomosis. Encephaloduroarteriosynangiosis, using the proximal part of the intact frontal branch of the STA, and encephalomyosynangiosis, using the temporal muscle, were also performed in all patients to stimulate spontaneous anastomosis. In addition, bilateral frontal burr holes were made in all patients to induce vascularization of the ACA territories from the distal part of the intact frontal branch of the STA. The first operation was performed on the dominant side, then a similar procedure was performed on the opposite side after an interval of at least 3 months. RESULTS: Postoperative clinical symptoms and the findings from magnetic resonance imaging, magnetic resonance angiography, angiography, and electroencephalography demonstrated improvement in all patients. CONCLUSION: These results suggest that the placement of bilateral burr holes (while leaving the frontal branch of the STA intact), in addition to the STA-MCA anastomosis, encephaloduroarteriosynangiosis, and encephalomyosynangiosis, is very effective in vascularizing the ischemic ACA and MCA territories in pediatric patients with moyamoya disease.

Adolescent↗

[Postoperative electroencephalographic change in old patients with cardiac surgery].

The purpose of this study was to evaluate cerebral dysfunction in aged patients associated with cardiac surgery using quantitative electroencephalograph (QEEG). Seventeen consecutive patients undergoing cardiac surgery were investigated. There were nine males and eight females whose ages ranged from 65 to 77 years (mean 70.6 years). The cardiac procedures consisted primarily of coronary artery bypass (eleven patients) and valve replacement (six patients). They had good postoperative course, no complications were recognized. Each patient had an EEG examination using digital EEG equipment in first day before operation. Follow up investigations were repeated three times (first day, fifth day and one month after operation). QEEG analysis consisted of fast Fourier transform method was performed in each record. A fall in the peak frequency at basic rhythm was seen in 1st and 5th postoperative day. A power spectra corresponding to slow wave showed an increase at parieto-occipital area in 1st and 5th postoperative day. Alpha activity at occipital area was increased in one month after operation. The EEG change in first or fifth postoperative day suggested the patients had cerebral dysfunction without clinical findings.

Age Factors↗

Cellular induction mechanism of CD8+ suppressor T cells by DMBA and TPA: formation of CD4+ suppressor-inducer T cells.

We investigated the cellular induction mechanism of antigen-nonspecific CD8+ T suppressor cells which suppressed delayed-type hypersensitivity to sheep red blood cells, by treating BALB/c mice with 7,12-dimethylbenz[a]anthracene (DMBA), a tumor initiator, and 12-O-tetradecanoylphorbol 13-acetate (TPA), a tumor promoter. Macrophages were activated by painting with 400 nmol of DMBA on mice. And the macrophages plus TPA induced CD4+ T suppressor inducer cells in the mice spleens. These cells were efficient at inducing CD8+ T suppressor-effector cells. When 8 nmol of TPA was painted daily on mice for 3 days following the treatment with 400 nmol of DMBA, or when spleen cells from mice pretreated with 400 nmol of DMBA were cultured in 32 nmol/5 ml of TPA for 3 days, inducer cells were formed in the spleen. Both T suppressor-inducer cells and macrophages from mice treated with DMBA were shown to act with the formation of soluble factors, which may be different from IL-10.

9,10-Dimethyl-1,2-benzanthracene↗

Ossification of the distal phalanx of the first digit as a maturity indicator for initiation of orthodontic treatment of Class III malocclusion in Japanese women.

The influence of mandibular growth on the stability of orthodontic treatment has been well established. A particular problem is late mandibular growth in patients with Class III malocclusions, because of skeletal jaw discrepancies that may influence the timing and course of treatment, as well as the stability of posttreatment. We have used the ossification of the distal phalanx of the first digit as an indicator of the skeletal maturity of the patient and of their potential for further growth. Our previous studies have shown that fusion of the epiphysis and the diaphysis of the distal phalanx of the first digit occurs from 1 to 3 years after the pubertal growth maximum in Japanese women. In these case reports, the orthodontic treatment of two female patients with mild Class III skeletal malocclusions is presented to show the possible clinical application of the ossification of the first digit as an indicator of the completion or near cessation of mandibular growth in the timing of treatment of Class III malocclusions. The case reports show that, although both patients experienced some mild degree of mandibular growth after treatment, this method can be helpful in determining residual mandibular growth potential in Japanese female patients with Class III malocclusions and mild skeletal discrepancies.

Age Determination by Skeleton↗

Cryptogenic localization-related epilepsy of neonatal onset.

We report three patients with localization-related epilepsy of neonatal onset. They exhibited favourable psychomotor development and had no cerebral lesions on neuroimaging studies despite the presence of intractable partial seizures of neonatal onset. Although rare, some cases of epilepsy of neonatal onset may be cryptogenic, i.e. they belong to neither the symptomatic nor the idiopathic group.

Blood Glucose↗

Clinico-epidemiological features of infantile hydrocephalus in Japan.

In 1988, a nationwide survey was conducted in Japan, in order to examine the prevalence and clinico-epidemiological features of hydrocephalus that developed before 1 year of age. The study was based on questionnaires that were sent to all hospitals in Japan with more than 200 beds. There were 1435 patients who were eligible for the analysis. They were born before 1987 and diagnosed as having infantile hydrocephalus. Approximately two-thirds of the patients had primary hydrocephalus. Neural tube defects were the most frequent cause of the primary hydrocephalus and common among full-term infants. On the other hand, secondary hydrocephalus occurred more often in preterm infants, mainly following intracranial hemorrhage. Intracranial hemorrhage as a cause of hydrocephalus increased over the year, while the incidence of hydrocephalus secondary to intracranial infection decreased during the same period.

Brain↗

Cortical hypometabolism and delayed myelination in West syndrome.

PURPOSE: We examined the relation between cortical hypometabolism and delayed myelination in patients with West syndrome (WS). METHODS: Serial positron emission tomography (PET) with [18F]fluorodeoxyglucose ([18F]FDG) and magnetic resonance imaging (MRI) were performed in 18 patients with WS, first at the onset of epileptic spasms and later at age 10 months. The age at onset of seizures ranged from 2 to 7 months. Ten patients were diagnosed as having cryptogenic WS and 8 as having symptomatic WS. RESULTS: Cortical hypometabolism was detected in many patients at onset of epilepsy, but disappeared later, whereas delayed myelination tended to become evident with age. PET showed diffuse or focal cortical hypometabolism in 12 patients at onset, but in only 6 patients at age 10 months. MRI showed delayed myelination in only 2 patients at onset of epilepsy, but the number of patients with delayed myelination increased to 12 at age 10 months. Delayed myelination was more often present in patients with cortical hypometabolism. Delayed myelination was noted in 11 (85%) of 13 patients with cortical hypometabolism on first or second PET scans, but in only 1 (20%) of 5 patients who did not show PET abnormalities. Hypometabolism on the first or second PET scan was positively correlated with delayed myelination at age 10 months. CONCLUSIONS: In patients with WS, assessing myelination with MRI again at age 8-10 months is important even when MRI at the onset of epilepsy appears normal. Serial MRI and PET scans disclose more detailed pathophysiology of WS.

Age of Onset↗

Do ictal, clinical, and electroencephalographic features predict outcome in West syndrome?

Ictal electroencephalographic/video recordings of 42 patients with West syndrome (WS) were reviewed to define the relation between ictal, clinical, and EEG features and etiology or prognosis. The duration and type of spasms, the number of spasms per cluster, and the interval between spasms did not correlate with the etiology or the short-term prognosis. However, eye deviation, asymmetric spasms, and partial seizures concomitant with spasms were observed only in symptomatic WS patients with poor outcome. In particular, all 8 patients with concurrent partial seizures had severe psychomotor retardation. The ictal EEG characteristics did not correlate with etiology and prognosis. There was no significant difference between the patients with persistence or reappearance of hypsarrhythmia and the patients without interspasm hypsarrhythmia.

Electroencephalography↗