Biomedical subjects

T Negoro

Publications and source records attributed to T Negoro.

At least 19 recordsLinked to original sources

PubMed2001

Inflammatory pathological changes in a 2-year-old boy with Charcot-Marie-Tooth disease.

We report histopathological findings in a 2-year-old boy with Charcot-Marie-Tooth (CMT) 1A, which had some similarities to those of chronic inflammatory demyelinating polyneuropathy. These findings are unusual in adult CMT patients but are reported in some patients with corticosteroid-responsive hereditary motor and sensory neuropathy (HMSN) that are characterized by rapid worsening of symptoms. We administered betamethasone based on the inflammatory pathological features but no improvement was seen. Active demyelination is also reported as an early morphological feature in HMSN. It is probable that edema, active demyelination and a few onion bulb formations, which were recognized in this patient, are characteristic early histological changes of HMSN rather than those of corticosteroid-responsive HMSN.

Charcot-Marie-Tooth Disease↗

PubMed2001

Five-year follow-up of patients with partial epilepsies in infancy.

The aim of this study is to clarify the long-term outcome of patients with partial epilepsies that begin in the first 2 years of age. Thirty-nine infants who experienced partial epilepsies with onset by 2 years of age were followed until they were at least 5 years of age. Seizure and developmental outcome were investigated in relation to the following items: delayed psychomotor development before onset of seizures; interictal electroencephalogram (EEG) abnormalities; abnormal neuroimaging; family history of epilepsy; and history of neonatal seizures. Thirty-three infants achieved freedom from seizures and 27 achieved normal development. Delayed development before the onset and abnormal interictal EEG correlated with both poor seizure and developmental outcome. Abnormal neuroimaging findings correlated with a poor seizure outcome. A history of neonatal seizures and lack of a family history of epilepsy correlated with poor developmental outcome. The combination of the first four factors predicted a normal outcome with a sensitivity of 0.79 and a specificity of 0.87. The seizure and developmental outcomes were better in this study than those in previous studies. The combination of the four factors will be useful for predicting the outcomes in patients with partial epilepsies in infancy.

Brain↗

PubMed2001

Induction of CD4+ regulatory T cells by 12-O-tetradecanoylphorbol 13-acetate in mice. Part 1: The strain difference in this induction.

12-O-Tetradecanoylphorbol 13-acetate (TPA), an activator of signal transduction, induced antigen-nonspecific regulatory T (Tr) cells for delayed-type hypersensitivity (DTH) in the spleen. A marked strain difference in the induction of Tr cells was observed when the study was performed by using the several strains of mice at 6-8 weeks old. TPA painting induced CD4+Tr cells in C3H/He (H-2k), C3H/HeN (H-2k), DBA/2 (H-2d) and AKR/N (H-2k) mice, but not in C57BL/6 (H-2b), C57BL/10 (H-2b), BALB/c (H-2d) and A/J (H-2a). Regulatory cells were also induced by incubating spleen cells from unprimed mice with TPA in vitro and were seemed to act by the production of soluble factor(s). A downregulatory activity of the soluble factor(s) was abrogated by SXC-1 (anti-IL-10 monoclonal antibody), but not by SXC-2 (anti-IL-10 monoclonal antibody) and anti-IL-4. For purification of the factor(s), we established the T cell hybridoma 4C5-1 by the fusion of spleen cells from TPA-treated C3H/He mice with AKR thymoma Bw5147 cells. The 4C5-1 cells secrete the factor(s) which can inhibit DTH response. The inhibitory activity of the factor(s) could be neutralized by SXC-1, but not by SXC-2, anti-IL-4, anti-IL-6 and anti-TGF-beta antibodies. The factor(s) could not affect the proliferation and IFN-gamma production of alpha s1-casein-specific 3D20 Th1 cells. The factor(s) termed DIF (DTH Inhibitory Factor) may be a novel cytokine, since they have reduced the footpad swelling response by local injection, and have no immune crossreactivity with the DTH regulatory cytokines and no inhibitory activity for in vitro Th1 response.

9,10-Dimethyl-1,2-benzanthracene↗

PubMed2001

A pilot study on benign partial epilepsy in children with complex partial seizures.

In a review of 145 children with partial onset epilepsy, the authors were able to determine a focus of children whose complex partial seizures (CPS) ran a benign course, who had no identifiable lesion on scanning and whose EEG focus was not fixed, but tended to shift. The authors suggest that a benign form of CPS in children can be recognised.

Adolescent↗

PubMed2000

Cerebral oxygenation state in childhood moyamoya disease: a near-infrared spectroscopy study.

Near-infrared spectroscopy was used to measure the relative concentration changes of oxygenated hemoglobin (HbO(2)), deoxygenated hemoglobin (HbR), and total hemoglobin (t-Hb) before, during, and after hyperventilation in eight patients with childhood moyamoya disease who underwent surgery. The patients were divided into the following two groups: those with and those without re-build-up phenomenon on electroencephalography. All patients except one exhibited decreased concentrations of HbO(2) and t-Hb during hyperventilation and decreased concentrations of HbO(2) and increased concentrations of HbR after hyperventilation. The difference between the hemoglobin concentration during and after hyperventilation was significantly greater in the group with the re-build-up phenomenon than the group without it (P <0.03, Mann-Whitney U test). The patients with the re-build-up phenomenon had lower HbO(2) and higher HbR concentrations after hyperventilation. The concentrations of HbO(2) and HbR reflect an alteration in the oxygenated blood supply and/or oxygen use. Thus, it is strongly suggested that regional cerebral hypoxia and metabolic oxygen disturbances play an important role in the occurrence of the re-build-up phenomenon. Near-infrared spectroscopy is a useful noninvasive method to evaluate the efficacy of surgical treatment in childhood moyamoya disease.

Adolescent↗

PubMed2000

Transient seizure remission in intractable localization-related epilepsy.

We sought to elucidate the clinical features of transient seizure remission in intractable cryptogenic or symptomatic localization-related epilepsy of childhood onset. Transient seizure remission has been reported to occur in mesial temporal sclerosis or focal cortical dysplasia, but few reports have focused on this phenomenon. We retrospectively scrutinized the temporal profiles of seizure frequency of 99 patients with intractable localization-related epilepsy by reviewing their medical charts. Ten patients (10%) had transient seizure remissions that lasted for 2 years or longer. When an appropriate antiepileptic agent was administered, seizure remission occurred within 1-18 months. Without any triggering factors, the seizures recurred abruptly in seven patients and gradually in three. Epileptiform discharges on electroencephalography disappeared during the transient remission in seven patients and reappeared in five of them after recurrence. After recurrence, no antiepileptic agent was able to control the seizures. In comparison with those without transient seizure remission, these 10 patients tended to have normal intelligence and a positive family history for epilepsy. Transient seizure remission occurs in a variety of pathologic changes and may be a result of an interaction between the progressive nature of some types of epileptogenic foci and an effect of the antiepileptic drugs.

Adolescent↗

PubMed2000

Increase in participation of vasoactive intestinal peptide in relaxation of the distal colon of Wistar rats with age.

Changes in participation of vasoactive intestinal peptide (VIP) in nonadrenergic noncholinergic (NANC) relaxation of longitudinal muscle of the distal colon with age were studied in 2- to 50-week-old Wistar rats in vitro. The extent of the VIP-mediated component of the relaxation induced by electrical field stimulation (EFS) was determined by the effect of VIP(10 - 28), a VIP receptor antagonist. In 2-week-old rats, the extent of the VIP-mediated component of the relaxation was scarce, about 10%, whereas the component gradually increase with age and reached the maximum extent 66% at 50-week-old. Since our previous results suggest that VIP induces NANC relaxation via activation of charybdotoxin (ChTx, a blocker of large conductance Ca(2+)-activated K(+) channel)-sensitive K(+) channels with concomitant slow hyperpolarization in the muscle cells, we next studied whether ChTx-sensitive component and slow hyperpolarization changes with age. Extent of ChTx-sensitive component of the relaxation increased with age, showing a very similar pattern to VIP-mediated one. EFS induced monophasic inhibitory junction potentials (i.j.ps) in longitudinal muscle cells of the distal colon of 2- and 4-week-old. EFS also induced biphasic i.j.ps in many longitudinal muscle cells of 8- and 50-week-old: rapid and subsequent slow hyperpolarization. A VIP receptor antagonist selectively inhibited the slow hyperpolarization. Exogenously added VIP induced no appreciable change in the membrane potential of longitudinal muscle cells of 2-week-old, whereas it induced slight slow hyperpolarization of the cell membrane in 4-week-old and magnitude of the hyperpolarization increased with age. On the other hand, relaxant response of the longitudinal muscle to exogenously added VIP was high in younger rats. The present results suggest that the role of VIP in mediating NANC relaxation of longitudinal muscle of the Wistar rat distal colon is very little at neonatal stage, but it increases with age.

Age Factors↗

PubMed2000

MRI findings in patients with symptomatic localization-related epilepsies beginning in infancy and early childhood.

We studied MRI findings in patients with symptomatic localization-related epilepsy (S-LRE) beginning in infancy and early childhood and their relation to some clinical features. MRI findings were investigated in 183 patients with S-LRE beginning before 6 years of age. S-LRE was defined as an epileptic syndrome manifesting with only partial seizures at least for a certain period during the clinical course, having specific neuroimaging abnormalities considered to be related to epileptic seizures, moderate or severe mental retardation, or cerebral palsy. The timing of injury was divided into the following four groups; prenatal, perinatal, postnatal and unknown. It was presumed prenatal in 56 patients (31%), perinatal in 25 (14%), postnatal in 32 (18%) and unknown in 70 (38%). The median age at the onset of partial seizures was 24 months in the congenital (prenatal and unknown) group, 39 months in the perinatal group and 25 months in the postnatal group. The onset of partial seizures was later in the perinatal group than in the congenital group. There were no significant differences in the other clinical characteristics among groups except for more frequent association of cerebral palsy in the perinatal group than in the congenital group. Our study suggests that congenital (prenatal and unknown group) disorders may be most common in patients with S-LRE beginning in infancy and early childhood. The onset of partial seizures occurred later in the perinatal group.

Brain↗

PubMed2000

Early recognition of benign partial epilepsy in infancy.

PURPOSE: The aim of this study is to determine how precisely we can recognize the outcome in infants with epilepsy beginning in the first year of life. METHODS: We performed a prospective 5-year follow-up study on 63 patients who developed epilepsy in the first year of life. We first judged that patients met the criteria of "possible benign partial epilepsy in infancy (BPEI)" on enrollment in this study. At 2 years of age, we reevaluated the seizure and developmental outcome in the patients who were diagnosed as having "possible BPEI." We finally judged that patients met the criteria of "definite BPEI" at age 5 years. "Possible BPEI" was defined as epilepsy meeting all the following conditions: (a) complex partial seizures and/or secondarily generalized seizures; (b) normal psychomotor development and neurologic findings before onset; (c) normal interictal electroencephalograms; (d) normal cranial computed tomography (CT) and magnetic resonance imaging (MRI) findings; and (e) no seizures during the first 4 weeks of life. "Definite BPEI" was defined as epilepsy meeting all the following criteria in addition to those of "possible BPEI": (a) normal psychomotor development beyond age 5 years, and (b) no seizures beyond age 2 years. RESULTS: Thirty-two of the 63 patients met the inclusion criteria completely and were included in the "possible BPEI" group. Twenty-five of the 32 patients completed the 5-year follow-up. At age 2 years, four patients were excluded from the "possible BPEI" group because of seizure recurrence and/or delayed development. By age 5 years, one had a recurrence of seizures, and another exhibited mildly delayed psychomotor development. We finally diagnosed 19 patients as having "definite BPEI." "Definite BPEI" accounted for 76% of the patients diagnosed as having "possible BPEI" at the first presentation and 90% of those who met the conditions on reevaluation at age 2 years. CONCLUSIONS: Recognition of BPEI is possible, to some extent, at the first presentation, and reevaluation at age 2 years is useful for a more precise diagnosis.

Age Factors↗

PubMed2000

EMG activities of two heads of the human lateral pterygoid muscle in relation to mandibular condyle movement and biting force.

Electromyographic (EMG) activities of the superior (SUP) and inferior heads (INF) of the lateral pterygoid muscle (LPT) were recorded in humans during voluntary stepwise changes in biting force and jaw position that were adopted to exclude the effects of acceleration and velocity of jaw movements on the muscle activity. The SUP behaved like a jaw-closing muscle and showed characteristic activity in relation to the biting force. It showed a considerable amount of background activity (5-32% of the maximum) even in the intercuspal position without teeth clenching and reached a nearly maximum activity at relatively lower biting-force levels than the jaw-closing muscles during increment of the biting force. Stretch reflexes were found in the SUP, the function of which could be to stabilize the condyle against the biting force that pulls the condyle posteriorly. This notion was verified by examining the biomechanics on the temporomandibular joint. The complex movements of the mandibular condyle in a sagittal plane were decomposed into displacement in the anteroposterior direction (Ac) and angle of rotation (RAc) around a kinesiological specific point on the condyle. In relation to Ac, each head of the LPT showed quite a similar behavior to each other in all types of jaw movements across all subjects. Working ranges of the muscle activities were almost constant (Ac <3 mm for the SUP and Ac >3 mm for the INF). The amount of EMG activity of the SUP changed in inverse proportion to Ac showing a hyperbola-like relation, whereas that of the INF changed rather linearly. The EMG amplitude of the SUP showed a quasilinear inverse relation with RAc in the hinge movement during which the condyle rotated with no movement in the anteroposterior direction. This finding suggests that the SUP controls the angular relationship between the articular disk and the condyle. On the other hand, the position of the disk in relation to the maxilla, not to the condyle, is controlled indirectly by the INF because the disk is attached to the condyle by tendinous ligaments.

Adult↗

PubMed2000

[Psoas compartment block for surgery of the femoral neck (trochanteric) fracture in a patient with severe heart failure due to rheumatoid myocarditis].

A 72-year-old female with severe heart failure due to rheumatoid myocarditis underwent open reduction of the left femoral neck (trochanteric) fracture. We performed psoas compartment block (PCB) at L3/4 level in the lateral position with the fractured side up, using a 22 G Tuohy needle to inject 10 ml of normal saline and 20 ml of 2% mepivacaine. Anesthesia was maintained with propofol (1-2 mg.kg-1.hr-1) under spontaneous breathing with oxygen (2 l.min-1) through mask. The patient did favorably during and after the operation. We conclude that PCB is useful for surgery of the lower extremity in patients with heart failure.

Aged↗

PubMed1999

Epileptic seizures induced by animated cartoon, "Pocket Monster".

PURPOSE: A large number of children had fits while watching the animated cartoon television (TV) program "Pocket Monster." To elucidate the seizures associated with the TV program, we administered a questionnaire survey in Aichi Prefecture, Japan. METHODS: The questionnaires were sent to 75 hospitals located in and around Aichi prefecture. The presence of epileptic seizures and the types of seizures were determined by three pediatric neurologists. RESULTS: Sixty-one hospitals responded to the questionnaire survey. Among 95 patients living in Aichi prefecture for whom enough information on seizure manifestations and EEG was available, < or =93 patients were considered to have epileptic seizures while watching the TV program. Most seizures occurred at a scene in which red and blue frames alternated at 12 Hz. Sixty-nine (74%) patients had no history of epilepsy. Thirty-nine patients had generalized seizures, and 49 patients had partial seizures. Partial seizures occurred more frequently in the younger age group than did generalized seizures. The EEG revealed a photoparoxysmal response (PPR) in 43% of patients. PPR was present not only in patients with a history of epilepsy (54%) but also in those with no history of epilepsy (38%). CONCLUSIONS: Almost all seizures induced by the TV program "Pocket Monster" were epileptic, and partial seizures were induced more frequently than generalized seizures. The incidence of this "Pocket Monster"-induced seizures was roughly estimated as > or =1 in 4,923 individuals aged 6-18 years.

Adolescent↗

PubMed1999

Epileptic spasms preceded by partial seizures with a close temporal association.

PURPOSE: To investigate the distinctive features of patients with West syndrome who had partial seizures followed by epileptic spasms (PS-ES). METHODS: We examined 45 patients with West syndrome whose epileptic spasms were recorded with simultaneous video-electroencephalography (EEG) monitoring between 1982 and 1996. We investigated the patients who had PS-ES and compared the PS-ES patients with the 37 patients without PS-ES. RESULTS: Of the 45 patients who had epileptic spasms in clusters (ES) and hypsarrhythmia on the interictal EEG, eight (17%) had ES preceded by partial seizures (PS) with a close temporal association. Seven of these eight were female patients. The underlying disorders were tuberous sclerosis (one patient), Aicardi syndrome (one), nonketotic hyperglycinemia (one), and focal cortical dysplasia (one). The etiology was unknown in the remaining four patients, but was suspected to be of prenatal origin in three. Three types of seizure sequence were identified: PS followed several seconds later by ES (two patients), alternating PS and ES starting with PS (three), and PS gradually replaced by ES with overlapping of the two (three). PS-ES disappeared or was replaced by other types of seizures in 1-34 months. Six patients could not walk, and all patients could not speak any sentences at age 3 years. CONCLUSIONS: Compared with patients without PS-ES, those with PS-ES more often had organic brain lesions of prenatal origin, other types of seizures before the onset of ES, asymmetric hypsarrhythmia on the EEG, and poor psychomotor outcome.

Age of Onset↗

PubMed1998

Novel, highly potent aldose reductase inhibitors: (R)-(-)-2-(4-bromo-2-fluorobenzyl)-1,2,3,4- tetrahydropyrrolo[1,2-a]pyrazine -4-spiro-3'-pyrrolidine-1,2',3,5'-tetrone (AS-3201) and its congeners.

A series of novel tetrahydropyrrolo[1,2-a]pyrazine derivatives were synthesized and evaluated as aldose reductase inhibitors (ARIs) on the basis of their abilities to inhibit porcine lens aldose reductase (AR) in vitro and to inhibit sorbitol accumulation in the sciatic nerve of streptozotocin-induced diabetic rats in vivo. Of these compounds, spirosuccinimide-fused tetrahydropyrrolo[1, 2-a]pyrazine-1,3-dione derivatives showed significantly potent AR inhibitory activity. In the in vivo activity of these derivatives, 2-(4-bromo-2-fluorobenzyl)-1,2,3,4-tetrahydropyrrolo[1, 2-a]pyrazine-4-spiro-3'-pyrrolidine-1,2',3,5'-tetrone (23t) (SX-3030) showed the best oral activity. The enantiomers of 23t were synthesized, and the biological activities were evaluated. It was found that AR inhibitory activity resides in the (-)-enantiomer 43 (AS-3201), which was 10 times more potent in inhibition of the AR (IC50 = 1.5 x 10(-8) M) and 500 times more potent in the in vivo activity (ED50 = 0.18 mg/kg/day for 5 days) than the corresponding (+)-enantiomer 44 (SX-3202). From these results, AS-3201 was selected as the candidate for clinical development. The absolute configuration of AS-3201 was also established to be (R)-form by single-crystal X-ray analysis. In this article we report the preparation and structure-activity relationship (SAR) of tetrahydropyrrolopyrazine derivatives including a novel ARI, AS-3201.

Aldehyde Reductase↗

PubMed1998

Functional analysis of diastrophic dysplasia sulfate transporter. Its involvement in growth regulation of chondrocytes mediated by sulfated proteoglycans.

Mutations in the diastrophic dysplasia sulfate transporter (DTDST) gene constitute a family of recessively inherited osteochondrodysplasias including achondrogenesis type 1B, atelosteogenesis type II, and diastrophic dysplasia. However, the functional properties of the gene product have yet to be elucidated. We cloned rat DTDST cDNA from rat UMR-106 osteoblastic cells. Northern blot analysis suggested that cartilage and intestine were the major expression sites for DTDST mRNA. Analysis of the genomic sequence revealed that the rat DTDST gene was composed of at least five exons. Two distinct transcripts were expressed in chondrocytes due to alternative utilization of the third exon, corresponding to an internal portion of the 5'-untranslated region of the cDNA. Injection of rat and human DTDST cRNA into Xenopus laevis oocytes induced Na+-independent sulfate transport. Transport activity of the expressed DTDST was markedly inhibited by extracellular chloride and bicarbonate. In contrast, canalicular Na+-independent sulfate transporter Sat-1 required the presence of extracellular chloride in the cRNA-injected oocytes. The activity profile of sulfate transport in growth plate chondrocytes was studied in the extracellular presence of various anions and found substantially identical to DTDST expressed in oocytes. Thus, sulfate transport of chondrocytes is dominantly dependent on the DTDST system. Finally, we demonstrate that undersulfation of proteoglycans by the chlorate treatment of chondrocytes significantly impaired growth response of the cells to fibroblast growth factor, suggesting a role for DTDST in endochondral bone formation.

Amino Acid Sequence↗

PubMed1998

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↗

PubMed1998

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↗

PubMed1998

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↗