Keiko Yanagihara

Publications (15)24.64 Total impact

• Article: Prolonged elevation of serum neuron-specific enolase in children after clinical diagnosis of brain death.

  Yasuhiro Suzuki, Yukiko Mogami, Yausihisa Toribe, Keitaro Yamada, Keiko Yanagihara, Ikuko Hirata, Toshiyuki Mano
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  ABSTRACT: To elucidate the time course of neuronal cell death after the clinical criteria for brain death are met, the authors reviewed serial changes of serum neuron-specific enolase levels in 3 children (age range, 3-15 years) clinically diagnosed as brain dead due to cardiopulmonary arrest. All patients survived for more than 2 months after brain death. Children with brain death had higher peak neuron-specific enolase values (1069-2849 ng/mL) than did 3 control children (256-1800 ng/mL) who did not become brain dead but had poor neurological outcome (1 death, 2 vegetative state) after cardiopulmonary arrest. A major finding is that children with brain death showed persistent elevation of neuron-specific enolase at 4 weeks (>400 ng/mL) and 8 weeks (>50 ng/mL) after cardiopulmonary arrest, in comparison with 2 surviving patients without brain death (<50 ng/mL at 4 weeks). This prolonged elevation of neuron-specific enolase suggests that total brain necrosis might not be present at the time of clinical diagnosis of brain death.
  Journal of child neurology 01/2012; 27(1):7-10. · 1.59 Impact Factor
• Article: SLC2A1 gene analysis of Japanese patients with glucose transporter 1 deficiency syndrome.

  Natsuko Hashimoto, Kuriko Kagitani-Shimono, Norio Sakai, Takanobu Otomo, Koji Tominaga, Shin Nabatame, Yukiko Mogami, Yukitoshi Takahashi, Katsumi Imai, Keiko Yanagihara, Takeshi Okinaga, Toshisaburo Nagai, Masako Taniike, Keiichi Ozono
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  ABSTRACT: Glucose transporter 1 deficiency syndrome (Glut1-DS) is a congenital metabolic disorder characterized by refractory seizures with early infantile onset, developmental delay, movement disorders and acquired microcephaly. Glut1-DS is caused by heterozygous abnormalities of the SLC2A1 (Glut1) gene, whose product acts to transport glucose into the brain across the blood-brain barrier. We analyzed the SLC2A1 gene in 12 Japanese Glut1-DS patients who were diagnosed by characteristic clinical symptoms and hypoglycorrhachia as follows: all patients had infantile-onset seizures and mild to severe developmental delay, and ataxia was detected in 11 patients. For the 12 patients, we identified seven different mutations (three missense, one nonsense, two frameshift and one splice-site) in exons and exon-intron boundaries of the SLC2A1 gene by direct sequencing, of which six were novel mutations. Of the remaining five patients who had no point mutations and underwent investigation by multiplex ligation-dependent probe amplification, a complex abnormality with deletion and duplication was identified in one patient: this is the first case of such recombination of the SLC2A1 gene. Changes in regulatory sequences in the promoter region or genes other than SLC2A1 might be responsible for onset of Glut1-DS in the other four patients (33%) without SLC2A1 mutation.
  Journal of Human Genetics 12/2011; 56(12):846-51. · 2.57 Impact Factor
• Article: MRI findings and steroid therapy for neuralgic amyotrophy in children.

  Keitaro Yamada, Toshiyuki Mano, Yasuhisa Toribe, Keiko Yanagihara, Yasuhiro Suzuki
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  ABSTRACT: Neuralgic amyotrophy is not uncommon in adults but is relatively rare in children. We recently encountered 2 cases of neuralgic amyotrophy in children. Patient 1 was a 7-year-old girl who developed a right leg paralysis after an epileptic seizure. Lumbar plexus T(2)-weighted magnetic resonance imaging (MRI) revealed a hyperintense and thickened portion extending from the root to the knee region of the right sciatic nerve, and T(1)-weighted conventional spin echo with gadolinium administration revealed enhancement. Patient 2 was a 4-year-old boy who experienced a sudden onset of severe right arm pain and paralysis. T(2)-weighted MRI with a short tau inversion recovery revealed a slightly thickened and high intensity region at the right C(6)-C(8) level. After high-dose methylprednisolone pulse therapy was performed in each case, patient 1 experienced complete recovery, whereas patient 2 experienced only amelioration of pain. A diagnosis of neuralgic amyotrophy in children was facilitated by an MRI study (T(2) weighed with short tau inversion recovery and T(1) weighted with gadolinium enhancement), and early steroid therapy might have improved the condition of these children.
  Pediatric Neurology 09/2011; 45(3):200-2. · 1.52 Impact Factor
• Article: Diagnostic accuracy of blood and CSF lactate in identifying children with mitochondrial diseases affecting the central nervous system.

  Keitaro Yamada, Yasuhisa Toribe, Keiko Yanagihara, Toshiyuki Mano, Mikihiro Akagi, Yasuhiro Suzuki
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  ABSTRACT: To determine the diagnostic accuracy of blood and cerebrospinal fluid (CSF) lactate and pyruvate concentrations in identifying children with mitochondrial diseases (MD) affecting the central nervous system (CNS). We studied lactate and pyruvate concentrations in paired samples of blood and CSF collected concurrently from 17 patients with MD (Leigh encephalomyelopathy 10, MELAS 5, Pearson disease 1, PDH deficiency 1) and those from control patients (n=49). Although blood and CSF variables (lactate, pyruvate concentrations and lactate/pyruvate ratio) were significantly higher in the mitochondrial group than in the control group, there was considerable overlap of individual values between these two groups. The maximum value of the area under the receiver operating characteristic curve (AUC) was observed for the CSF lactate concentration (0.994, optimal cut-off value 19.9 mg/dl, sensitivity 0.941 and specificity 1.00), followed by the CSF pyruvate level (0.983). There was an inverse relationship between blood lactate and lactate CSF/blood ratio. For blood lactate concentrations between 20 and 40 mg/dl, a significant difference was also noted in the lactate CSF/blood ratio between the two groups (AUC 1.0, optimal cut-off value 0.91, sensitivity 1.0 and specificity 1.0). Our study suggests that that CSF lactate level>19.9 mg/dl is the most reliable variable for identifying patients with MD affecting the CNS. When blood lactate concentrations are marginally elevated (20-40 mg/dl), lactate CSF/blood ratio>0.91 may also provide diagnostic information.
  Brain & development 08/2011; 34(2):92-7. · 1.74 Impact Factor
• Article: [Successful treatment with additional plasmapheresis for the exacerbation of acute neurological symptoms in a girl with multiple sclerosis].

  Yukiko Mogami, Keitaro Yamada, Yasuhisa Toribe, Keiko Yanagihara, Toshiyuki Mano, Yasuhiro Suzuki
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  ABSTRACT: Few studies have indicated the efficacy of plasmapheresis in children with multiple sclerosis (MS). We report a 10-year-old girl with MS who was successfully treated with plasmapheresis. She experienced the first episode (vomiting and unconsciousness) at the age of eight years. After two years and six months remission period, she had a relapse with left hemiplegia. Her symptoms did not respond to high-dose intravenous methylprednisolone or immunoglobulin therapies, and rapidly deteriorated. After bulbar palsy was observed, plasmapheresis (total : every two days, seven times) was initiated at the 17th day during the relapse. Neurological symptoms improved remarkably after the second trial of plasmapheresis. There were no complications associated with plasmapheresis. Plasmapheresis may be effective as an additional therapy for exacerbation of acute neurological symptoms in children with MS.
  No to hattatsu. Brain and development 01/2011; 43(1):36-40.
• Article: [Changes of brain edema after initiation of mild hypothermia therapy in children].

  Keitaro Yamada, Toshiyuki Mano, Yu Inada, Yukiko Mogami, Yasuhisa Toribe, Keiko Yanagihara, Kazuya Tachibana, Yasuhiro Suzuki
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  ABSTRACT: To determine the effects of mild hypothermia therapy (34 degrees C) for brain edema caused by hypoxic ischemic encephalopathy (HIE) or acute encephalitis/encephalopathy, we reviewed the charts and serial brain CT images in six children (males 3, average age 1.6 years) treated with mild hypothermia therapy between November 2006 and April 2009. Both of the two children with HIE after cardiopulmonary arrest did not show any deterioration of brain edema after the initiation of hypothermia therapy. However, two of four non-HIE patients (acute encephalitis/encephalopathy 3 cases and metabolic encephalopathy plus HIE 1 case) showed progressive brain edema during the cooling phase and re-warming phase, respectively. There were no differences between patients with and those without progressive brain edema with regard to the interval until initiation of mild hypothermia therapy, duration of cooling phase, duration of re-warming phase, or peak serum NSE (neuron-specific enolase) levels. However, two children with progressive brain edema showed a delayed NSE peak time (15 and 13 days after onset, respectively), compared with those without progressive brain edema (2-6 days after onset). Our study suggests that serial measurement of serum NSE might be useful marker for adjusting the methods of hypothermia therapy according to neuropathology. Further study is necessary to establish optimal hypothermia therapy especially in children with acute encephalitis/ encephalopathy.
  No to hattatsu. Brain and development 01/2011; 43(1):10-3.
• Source

  Available from: PubMed Central

  Article: Abnormal cystatin C levels in two patients with bardet-biedl syndrome.

  Makito Hirano, Mitsuru Ohishi, Toshihide Yamashita, Yasushi Ikuno, Hiromi Iwahashi, Toshiyuki Mano, Ryu Ishihara, Ichiro Tanaka, Keiko Yanagihara, Chiharu Isono, Hikaru Sakamoto, Yusaku Nakamura, Susumu Kusunoki
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  ABSTRACT: Bardet-Biedl syndrome (BBS) is an autosomal recessive disorder characterized by central obesity, mental impairment, rod-cone dystrophy, polydactyly, hypogonadism in males, and renal abnormalities. The causative genes have been identified as BBS1-14. In the Western countries, the prevalence of this disease ranges from 1/13,500 to 1/160,000, while only a few Japanese patients have been reported in the English-language literature. The incidence of renal dysfunction or anomalies in previous reports varies considerably ranging from ∼20% to universal occurrence. We here report that two Japanese patients who had BBS with normal BUN and creatinine levels had elevated levels of cystatin C, a sensitive marker of glomerular filtration rate. A urine albumin level increased only in the elder patient. Thus, cystatin C may be useful for detecting renal abnormalities in patients with an apparent normal renal function. Because this disease is diagnosed by accumulation of symptoms, such a sensitive marker might help early diagnosis of BBS.
  Clinical medicine insights. Case reports. 01/2011; 4:17-20.
• Article: Birth length is a predictor of adiponectin levels in Japanese young children.

  Masahiro Nishihara, Mina Sonoda, Katsura Matsunami, Keiko Yanagihara, Naohiro Yonemoto, Shinobu Ida, Fumihiko Namba, Iichiro Shimomura, Itaru Yanagihara, Masako Waguri
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  ABSTRACT: Adiponectin has been shown to be inversely related to birth weight in schoolchildren and adolescents. However, the available information regarding a relation between birth size and adiponectin in infants and preschoolers is limited. A longitudinal study was conducted among healthy Japanese children, and serum adiponectin levels were measured at consecutive visits. The effect of sex, gestational age, birth length, birth weight, or placental weight on adiponectin was analyzed by using a linear mixed model for repeated measures. Eighty-three children were evaluated. The age range at the first visit was 0.07-5.3 years. A total number of 227 measurements were made, 1-12 per subject (median, 3) and the duration of follow-up ranged from 1 day to 2.3 years (median, 1.0 years). The subjects were divided into four groups according to the age at the first visit: (1) 0-2 months (n = 29), (2) 3 months to 1 year (n = 17), (3) 2-3 years (n = 19) and (4) 4-5 years (n = 18). In the multivariate model, birth length (coefficient = 3.94, 95% CI, 0.23-7.65) was an independent predictor of serum adiponectin levels in subjects aged 0-2 months. Gestational age (3.53, 1.14-5.92), birth length (-2.71, -4.90 to -0.53) and placental weight (2.58, 0.86-4.29) were independent predictors of serum adiponectin levels in subjects aged 4-5 years. Birth length, not birth weight, is an independent predictor of serum adiponectin levels in Japanese young children.
  Journal of pediatric endocrinology & metabolism: JPEM 09/2010; 23(9):913-20. · 0.88 Impact Factor
• Article: Epilepsy in patients with congenital cytomegalovirus infection.

  Yasuhiro Suzuki, Yasuhisa Toribe, Yukiko Mogami, Keiko Yanagihara, Masanori Nishikawa
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  ABSTRACT: Patients with congenital cytomegalovirus (CMV) infection were at high risk for postnatal seizures, but little is known about epilepsy associated with congenital CMV infection. To define the features of epilepsy, we retrospectively reviewed the clinical, laboratory and neuroradiographic findings in 19 children (male 9) with congenital CMV infection. Seven (37%) patients had developed epilepsy (partial seizure 5 and epileptic spasms 2) at a mean age of 20 months (range 2-37 months). During the clinical course, West syndrome occurred in only three patients. The most common seizure type in our series was partial seizure. At the time of last follow-up (mean 96 months), seizures remained uncontrolled in six patients. Neonatal clinical manifestations (gestational age, gender distribution, birth asphyxia or symptoms at birth) were not predictive of the development of epilepsy. On the contrary, some neuroradiographic findings (ventricular dilatation and migration disorder) were significantly associated with the development of epilepsy.
  Brain and Development 07/2008; 30(6):420-4. · 2.12 Impact Factor
• Article: Anti-annexin A2 IgM antibody in preterm infants: its association with chorioamnionitis.

  Fumihiko Namba, Hiroyuki Kitajima, Atsushi Tabata, Masahiro Nakayama, Noriyuki Suehara, Katsura Matsunami, Keiko Yanagihara, Masahiro Nishihara, Akihiro Morita, Minoru Yamada, Akihito Kimoto, Shinya Hirano, Kiyotoshi Sekiguchi, Masanori Fujimura, Itaru Yanagihara
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  ABSTRACT: Intrauterine infection is associated with chorioamnionitis (CAM), which can lead to preterm delivery. We previously reported that the levels of IgM and the incidence of CAM were elevated in preterm infants with neonatal pulmonary emphysema. The pathogen and target of this IgM remain unclear. By using Western blot and amino acid sequences, we have determined one of the target proteins: annexin A2. Immunohistochemical analysis showed that annexin A2 was expressed at fetal chorion and amnion membranes. Among very low birth weight (VLBW) infants with hyper-IgM (> or = 30 mg/dL), 58.8% showed a high titer against annexin A2 (more than x 16), which accounted for about 20%-40% of the total IgM. Anti-annexin A2 IgM antibody inhibited plasmin generation. Furthermore, the median of anti-annexin A2 IgM titer from preterm infants who were delivered with high-grade (grade III) CAM was significantly higher than those from preterm infants without CAM (p = 0.011) and with low-grade CAM (grade I and II) (p = 0.010). Here, we indicate the fetal autoimmunoreactivity against the fetomaternal interface in preterm infants.
  Pediatric Research 01/2007; 60(6):699-704. · 2.70 Impact Factor
• Article: [Longitudinal MRI findings of glucose transporter I deficiency syndrome (Glut-1DS) in a Japanese patient - longitudinal investigation of multifocal T2 prolonged subcortical lesions, Case Report].

  Noriko Kamio, Katsumi Imai, Keiko Yanagihara, Shihoko Ohba, S Nabatame, K Shimono, Tsuyoshi Okinaga, Keiichi Ozono, Yoshinori Watanabe, Toshisaburo Nagai
  No to hattatsu. Brain and development 02/2006; 38(1):54-6.
• Article: Thermostable direct hemolysin of Vibrio parahaemolyticus is a bacterial reversible amyloid toxin.

  Takashi Fukui, Kentaro Shiraki, Daizo Hamada, Kojiro Hara, Tomoko Miyata, Shinsuke Fujiwara, Kouta Mayanagi, Keiko Yanagihara, Tetsuya Iida, Eiichiro Fukusaki, Tadayuki Imanaka, Takeshi Honda, Itaru Yanagihara
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  ABSTRACT: Thermostable direct hemolysin (TDH), a major virulence factor of Vibrio parahaemolyticus, is detoxified by heating at approximately 60-70 degrees C but is reactivated by additional heating above 80 degrees C. This paradoxical phenomenon, known as the Arrhenius effect, has remained unexplained for approximately 100 years. We now demonstrate that the effect is related to structural changes in the protein that produce fibrils. The native TDH (TDHn) is transformed into nontoxic fibrils rich in beta-strands by incubation at 60 degrees C (TDHi). The TDHi fibrils are dissociated into unfolded states by further heating above 80 degrees C (TDHu). Rapid cooling of TDHu results in refolding of the protein into toxic TDHn, whereas the protein is trapped in the TDHi structure by slow cooling of TDHu. Transmission electron microscopy indicates the fibrillar structures of TDHi. The fibrils show both the property of the nucleation-dependent elongation and the increase in its thioflavin T fluorescence. Formation of beta-rich structures of TDH was also observed in the presence of lipid vesicles containing ganglioside G(T1b), a putative TDH receptor. Congo red was found to inhibit the hemolytic activity of TDH in a dose-dependent manner. These data reveal that the mechanism of the Arrhenius effect which is tightly related to the fibrillogenicity of TDH.
  Biochemistry 08/2005; 44(29):9825-32. · 3.42 Impact Factor
• Article: Analysis of recombinant human saposin A expressed by Pichia pastoris.

  Minoru Yamada, Koji Inui, Daizo Hamada, Kumiko Nakahira, Keiko Yanagihara, Norio Sakai, Toshinori Nishigaki, Keiichi Ozono, Itaru Yanagihara
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  ABSTRACT: Saposins (SAPs) are small glycoproteins required for activation of sphingolipid hydrolysis by lysosomal enzymes. Four SAPs, SAP-A, -B, -C, and -D, are proteolytically cleaved from a single gene product termed prosaposin. The mature coding sequence of human SAP-A tagged with 6-histidine was expressed in Pichia pastoris and the recombinant protein was purified from the culture supernatant by simple purification steps with an immobilized metal ion affinity column, a Concanavalin A column, and reversed-phase HPLC. Secreted SAP-A contained both glycosylated and nonglycosylated forms. Both forms of SAP-A activated galactocerebroside and 4-methylumbelliferyl beta-d-glucoside hydrolysis by galactocerebrosidase and glucocerebrosidase. SAP-A expressed in P. pastoris should be useful for further structural and functional analysis of this protein.
  Biochemical and Biophysical Research Communications 06/2004; 318(2):588-93. · 2.48 Impact Factor
• Article: Neuromagnetic gamma-band activity in the primary and secondary somatosensory areas.

  Aya Ihara, Masayuki Hirata, Keiko Yanagihara, Hirotomo Ninomiya, Katsumi Imai, Ryouhei Ishii, Yasuhiro Osaki, Kotoe Sakihara, Hiromi Izumi, Hiroyuki Imaoka, Amami Kato, Toshiki Yoshimine, Shiro Yorifuji
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  ABSTRACT: To evaluate the gamma-band activity related to somatosensory processing, we recorded neuromagnetic signals from seven healthy subjects. The source power changes evoked by electrical stimulation of the median nerve were estimated with synthetic aperture magnetometry (SAM). Source power in the low gamma band (40 Hz) decreased in the contralateral primary somatosensory cortex (SI) for a few hundred milliseconds (i.e. middle and long latency) and then increased inversely. Source power in the high gamma band (70-90 Hz) increased simultaneously both in the contralateral SI and contra/ipsilateral secondary somatosensory cortex (SII) in 80-180 ms. These results suggest that low and high gamma oscillations work under independent mechanisms during somatosensory processing. In particular, high gamma oscillations may play an essential role in making a functional connection between SI and SII.
  Neuroreport 03/2003; 14(2):273-7. · 1.66 Impact Factor
• Article: Ictal Video‐EEG Recording of Three Partial Seizures in a Patient with the Benign Infantile Convulsions Associated with Mild Gastroenteritis

  Katsumi Imai, Kazumasa Otani, Keiko Yanagihara, Zhong-gi Li, Yasuyuki Futagi, Jiro Ono, Toshisaburo Nagai, Shintaro Okada
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  ABSTRACT: Purpose: In infants, benign convulsions can be triggered by febrile illness or mild diarrhea such as Rotavirus gastroenteritis. The triggering mechanism of these convulsions is still unknown. In spite of several reports concerning clinical features, the ictal EEG recordings were rarely analyzed by a video-EEG monitoring system. To reveal a clue for the triggering mechanism of these convulsions, we analyzed the correlation of clinical manifestations and the EEG discharges during the ictal events and compared with previous reports.Methods: The ictal EEG of a cluster of three afebrile convulsions associated with mild gastroenteritis was recorded by an EEG closed-circuit TV (EEG-CCTV) monitoring system in a 6-month-old healthy female infant.Results: All seizures began as complex partial seizures (CPSs), which exhibited a motionless stare with or without leftward deviation of both eyes, and evolved to secondarily generalized tonic-clonic seizures (SGTCSs) for −90 s. Each of three ictal discharges began from the right occipital, right centroparietotemporal, and left occipital regions, respectively.Conclusions: Although initiating sites of ictal discharges of benign infantile convulsions associated with mild gastroenteritis (BICE) were previously reported to be variable among patients, these results indicated that those differ among seizures even in a same infant.
  Epilepsia 09/1999; 40(10):1455 - 1458. · 3.96 Impact Factor