当前位置： SCI文献检索 > BRAIN & DEVELOPMENT期刊下所有文献 > Inappropriate intracranial hemodynamics in the natural course of MELAS.

Inappropriate intracranial hemodynamics in the natural course of MELAS.

Abstract：

:The abnormalities of intracranial hemodynamics associated with strokelike episodes in MELAS are variable depend on the time phase from the onset of strokelike episodes and on the progression of the dementia state. To clarify the regional cerebral blood flows (rCBF) in the natural course of MELAS is very important to understand the pathogenic mechanism of this disorder, either cytopathy, angiopathy or both. We analyzed the serial studies of brain statistical parametric mapping (SPM) 99 single photon emission computed tomography (SPECT) in 5 MELAS patients in maximum 10 years interval, who fulfilled the clinical, pathological and genetic criteria of MELAS, and have an A3243G mutation in the mitochondrial tRNA(Leu(UUR)) gene. SPM is a proven and effective method for the voxel-by-voxel analysis of functional images which show the advantage in its promise of fully automated neurophysiological imaging analysis throughout the whole brain using various statistical analyses. SPECT acquisition was initiated and was reconstructed by iterative algorithm and were processed and analyzed with SPM 99 for Windows software. Statistics were displayed as Z scores (threshold: P < 0.01). The inappropriate intracranial hemodynamics was found not only at the acute but at the interictal phase, and was getting worse as the disease progress. Hypoperfusion in the posterior cingulate cortex was always observed (corrected P < 0.01) in MELAS patients, which is the typical finding reported in Alzheimer's disease. The inappropriate intracranial hemodynamics is a common feature and may be related with mitochondrial angiopathy in the natural course of MELAS.

journal_name

Brain Dev

journal_title

Brain & development

authors

Nishioka J,Akita Y,Yatsuga S,Katayama K,Matsuishi T,Ishibashi M,Koga Y

doi

10.1016/j.braindev.2007.06.008

subject

Has Abstract

pub_date

2008-02-01 00:00:00

pages

100-5

issue

2

eissn

0387-7604

issn

1872-7131

pii

S0387-7604(07)00155-6

journal_volume

30

pub_type

杂志文章

相关文献

BRAIN & DEVELOPMENT文献大全
  • Ataxia with vitamin E deficiency and severe dystonia: report of a case.

    abstract：:Mutation of the gene for alpha-tocopherol transfer protein causes ataxia with isolated vitamin E deficiency, a disorder usually stabilized or improved after vitamin E supplementation. Dystonia has rarely been described in ataxia with isolated vitamin E deficiency (AVED) patients. We present the case of a young boy wit...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/s0387-7604(03)00054-8

    authors： Roubertie A,Biolsi B,Rivier F,Humbertclaude V,Cheminal R,Echenne B

    更新日期：2003-09-01 00:00:00

  • Neurochemistry evaluated by MR spectroscopy in a patient with xeroderma pigmentosum group A.

    abstract：:MRI of a female patient with xeroderma pigmentosum group A (XP-A) showed progressive cerebral atrophy, but no disease-specific lesion. MR spectroscopy with short TE sequences in the bilateral white matter revealed decreased N-acetyl aspartate (neuro-axonal marker) and increased myo-inositol (astroglial marker) with a ...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/j.braindev.2018.06.013

    authors： Yokota K,Sano K,Murofushi Y,Yoshimaru D,Takanashi JI

    更新日期：2018-11-01 00:00:00

  • Disentangling the effects of Tourette syndrome and attention deficit hyperactivity disorder on cognitive and behavioral phenotypes.

    abstract：:Eighty participants (62 males; 18 females; age range: 6-16 years) took part in the study, comprising four groups of 20 subjects each: TS-only, ADHD-only, TS+ADHD, controls. The age distributions, did not differ significantly among the four groups. The severity of symptoms, assessed by the TSGS, did not differ signific...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/j.braindev.2006.12.003

    authors： Rizzo R,Curatolo P,Gulisano M,Virzì M,Arpino C,Robertson MM

    更新日期：2007-08-01 00:00:00

  • Predictive factors of seizure frequency and duration of antiepileptic treatment in rolandic epilepsy: a retrospective study.

    abstract：:Factors useful to predict seizure frequency and duration of antiepileptic treatment of children with benign partial epilepsy and rolandic spikes were retrospectively evaluated in 72 patients seizure-free for at least 5 years and off antiepileptic drugs for at least 2 years. Three groups were considered: Group I, 11 pa...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/s0387-7604(87)80049-9

    authors： Ambrosetto G,Giovanardi Rossi P,Tassinari CA

    更新日期：1987-01-01 00:00:00

  • Ictal EEG in patients with convulsions with mild gastroenteritis.

    abstract：:The aim of this study is to reveal detailed clinical manifestations and an evolution of ictal EEG discharges of convulsions with mild gastroenteritis (CwG). We recorded ictal EEGs of six patients with CwG. Clinical manifestations included loss of responsiveness, motion arrest, cyanosis, lateral eye deviation, and hemi...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/j.braindev.2006.06.002

    authors： Maruyama K,Okumura A,Sofue A,Ishihara N,Watanabe K

    更新日期：2007-01-01 00:00:00

  • Unique astrocytic inclusion in a 2 month-old baby showing Leigh-like brain lesions with lactic acidosis.

    abstract：:An unique cytoplasmic inclusion was found in astrocytes of a 2-month-old female baby who showed Leigh-like brain lesions with lactic acidosis, hypoglycemia and hepatomegaly. Although a defective enzyme was not determined, a metabolic disorder was suggested from clinicopathological observations. Symmetrically distribut...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/s0387-7604(00)00117-0

    authors： Yamamoto T,Armstrong D,Shibata N,Kato Y,Kobayashi M

    更新日期：2000-06-01 00:00:00

  • The Rett and Rett-like syndromes: a broad concept.

    abstract：:Eight patients who satisfied the following three criteria: 1) autistic behavior, 2) mental retardation and 3) stereotypic hand movements were evaluated. Four patients fulfilled the diagnostic criteria for the Rett syndrome developed by representatives of the International Rett Syndrome Association and the Center for D...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/s0387-7604(12)80054-4

    authors： Lin MY,Wang PJ,Lin LH,Shen YZ

    更新日期：1991-07-01 00:00:00

  • Nutritional status among women whose pregnancy outcome was afflicted with neural tube defects in Tigray region of Ethiopia.

    abstract：BACKGROUND:Nutritional deficiency in pregnant women is a confirmed cause of neural tube defects (NTDs). Alongside to this background, We sought to determine the nutritional status and level of awareness on the issue of the NTDs as well as folic acid (FA) utilization among women who born infants with NTDs in Tigray regi...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/j.braindev.2018.12.005

    authors： Welderufael AL,Berihu BA,Berhe Y,Magana T,Asfaw S,Gebreselassie K,Belay E,Kebede H,Mulugeta A

    更新日期：2019-05-01 00:00:00

  • Efficacy of phenobarbital for benign convulsions with mild gastroenteritis: A randomized, placebo-controlled trial.

    abstract：OBJECTIVE:This study was performed to evaluate the efficacy and safety of intravenous phenobarbital (PB) for benign convulsions with mild gastroenteritis (CwG). METHODS:A randomized, single-blind, placebo-controlled trial involving patients with CwG was conducted at the Japanese Red Cross Society Himeji Hospital. Pati...

    journal_title：Brain & development

    pub_type： 杂志文章,随机对照试验

    doi：10.1016/j.braindev.2019.03.014

    authors： Takami Y,Nakagawa T

    更新日期：2019-08-01 00:00:00

  • Serial MRI changes in a patient with infantile Alexander disease and prolonged survival.

    abstract：:Alexander disease is a major entity of leukodystrophy; magnetic resonance imaging (MRI) studies of the brain typically show extensive changes in the cerebral white matter with frontal predominance. Heterozygous missense mutations of GFAP are thought to be sufficient for the molecular diagnosis, which has widened the A...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/j.braindev.2010.10.007

    authors： Shiihara T,Yoneda T,Mizuta I,Yoshida T,Nakagawa M,Shimizu N

    更新日期：2011-08-01 00:00:00

  • Hereditary progressive dystonia with marked diurnal fluctuation (Segawa syndrome) in Taiwan.

    abstract：:Since 1988, we have diagnosed 6 cases of hereditary progressive dystonia with marked diurnal fluctuation (HPD) in Taiwan. All cases presented with clinical features similar to those described by Segawa. They consisted of four sporadic and two familial cases. The age at onset ranged from 18 months to 8 years. There is ...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/0387-7604(94)90048-5

    authors： Wang PJ,Ko YM,Young C,Hwu WL,Shen YZ

    更新日期：1994-03-01 00:00:00

  • Pharmacokinetics of zonisamide in perinatal period.

    abstract：:Zonisamide is widely used for intractable epilepsy and the effects of this drug on fetuses and neonates through the mother taking it for epilepsy need to be clarified. We measured the zonisamide concentration in plasma and breast milk using high-performance liquid chromatography to investigate the transfer of zonisami...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/s0387-7604(01)00407-7

    authors： Kawada K,Itoh S,Kusaka T,Isobe K,Ishii M

    更新日期：2002-03-01 00:00:00

  • Hyperthermia-induced seizures with a servo system: neurophysiological roles of age, temperature elevation rate and regional GABA content in the rat.

    abstract：:A servo system including a microwave generator was applied to raise a rat's body temperature at a pre-set rate. Using this system the effects of age and the temperature elevation rate upon febrile seizures in rats were studied. The relationship between the brain GABA content and hyperthermia was also studied. From the...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/s0387-7604(12)80307-x

    authors： Morimoto T,Nagao H,Sano N,Takahashi M,Matsuda H

    更新日期：1990-01-01 00:00:00

  • Congenital muscular dystrophy with eye and brain involvement. The Turkish experience in two cases.

    abstract：:Eye and brain involvement in congenital muscular dystrophies (CMD) constitute a distinct group with a spectrum of brain malformations. We report two such CMD patients among our series of 58 cases with CMD. Despite known clinical and neuroradiological overlap, we tend to classify them into specific syndromes, though th...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/0387-7604(95)00047-f

    authors： Topaloğlu H,Cila A,Taşdemir AH,Saatçi I

    更新日期：1995-07-01 00:00:00

  • Morphometric development of the human fetal auditory system: inferior collicular nucleus.

    abstract：:The development of the human inferior collicular nucleus was studied on serial sections of the brains of 9 fetuses at 12-34 weeks of gestation and an adult of 63 years using an electronic planimeter with a computer. Morphometric analysis of the development of the inferior collicular nucleus showed that its development...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/0387-7604(95)00089-5

    authors： Nara T,Goto N,Hamano S,Okada A

    更新日期：1996-01-01 00:00:00

  • Dramatic response after functional hemispherectomy in a patient with epileptic encephalopathy carrying a de novo COL4A1 mutation.

    abstract：:We describe the first case of a successful functional hemispherectomy in a patient with epileptic encephalopathy and a de novo collagen type IV alpha 1 (COL4A1) mutation. A 4-year-old girl was COL4A1 mutation-positive and suffered from drug-resistant epilepsy, hemiplegia, and developmental delay. Magnetic resonance im...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/j.braindev.2016.11.006

    authors： Hino-Fukuyo N,Kikuchi A,Iwasaki M,Sato Y,Kubota Y,Kobayashi T,Nakayama T,Haginoya K,Arai-Ichinoi N,Niihori T,Sato R,Suzuki T,Kudo H,Funayama R,Nakayama K,Aoki Y,Kure S

    更新日期：2017-04-01 00:00:00

  • Follow-up study of children with cerebral coordination disturbance (CCD, Vojta).

    abstract：:713 children (from newborn to 12-month-old) with delayed motor development were carefully examined and classified into normal, very light cerebral coordination disturbance (CCD, Vojta), light CCD, moderate CCD, severe CCD, suspected cerebral palsy (CP) and other diseases at their first visit, and were followed up care...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/s0387-7604(83)80024-2

    authors： Imamura S,Sakuma K,Takahashi T

    更新日期：1983-01-01 00:00:00

  • Neurological presentation of Griscelli syndrome: obstructive hydrocephalus without haematological abnormalities or organomegaly.

    abstract：:Griscelli syndrome is a rare autosomal-recessive disorder characterised by partial albinism, immunodeficiency, organomegaly and accelerated phases. During accelerated phases, pancytopenia, haemophagocytosis, hypoproteinemeia occur which may be accompanied by neurological deterioration. Primary neurological presentatio...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/j.braindev.2006.09.007

    authors： Rajadhyax M,Neti G,Crow Y,Tyagi A

    更新日期：2007-05-01 00:00:00

  • Changes in BAER amplitudes after perinatal asphyxia during the neonatal period in term infants.

    abstract：:We recorded serially brainstem auditory evoked response (BAER) during the neonatal period in term infants who suffered perinatal asphyxia. The amplitudes of BAER components was analysed at 40 dB above BAER threshold of each subject who had a threshold

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/j.braindev.2006.03.004

    authors： Jiang ZD,Shao XM,Wilkinson AR

    更新日期：2006-10-01 00:00:00

  • X-linked adrenoleukodystrophy: clinical course and minimal incidence in South Brazil.

    abstract：UNLABELLED:X-linked adenoleukodystrophy is a genetic disease that affects the degradation of very long-chain fatty acids. In male patients, common pictures are the cerebral form (CALD), myeloneuropathy (AMN), and Addison-only. OBJECTIVE:To describe the clinical course of affected male patients from South Brazil betwee...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/j.braindev.2009.02.002

    authors： Jardim LB,da Silva AC,Blank D,Villanueva MM,Renck L,Costa ML,Vargas CR,Deon M,Coelho Dl,Vedolin L,de Castro CG Jr,Gregianin L,Bonfim C,Giugliani R

    更新日期：2010-03-01 00:00:00

  • Brain stem and spinal cord impairment in Rett syndrome: somatosensory and auditory evoked responses investigations.

    abstract：:Six females with Rett syndrome (RS)--all seriously motor disabled with clinical symptomatology indicating not only brain but also spinal cord impairment--were investigated using auditory and somatosensory evoked responses techniques. In all patients the responses representing the pathways through the upper spinal cord...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/s0387-7604(87)80076-1

    authors： Badr GG,Witt-Engerström I,Hagberg B

    更新日期：1987-01-01 00:00:00

  • White matter abnormalities in an adult patient with l-2-hydroxyglutaric aciduria.

    abstract：:l-2-Hydroxyglutaric aciduria (l-2-HGA) is a rare inborn error of metabolism. Mainly, patients with this disorder exhibit neurological symptoms and characteristic neuroradiological findings, such as subcortical white matter abnormalities, which are believed to be caused by the toxicity of the accumulation of l-2-hydrox...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/j.braindev.2015.04.012

    authors： Yamamoto T,Yoshioka S,Tsurusaki Y,Shino S,Shimojima K,Shigematsu Y,Takeuchi Y,Matsumoto N

    更新日期：2016-01-01 00:00:00

  • Congenital variant of Rett syndrome due to an intragenic large deletion in MECP2.

    abstract：:Rett syndrome (RTT) is a neurodevelopmental disorder that is one of the most common causes of mental retardation in females. RTT diagnosis is based on distinct clinical criteria. We describe here a female patient with severe phenotype of congenital variant RTT. The patient originally presented with severe developmenta...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/j.braindev.2011.09.014

    authors： Kobayashi Y,Ohashi T,Akasaka N,Tohyama J

    更新日期：2012-08-01 00:00:00

  • Thiamine-deficient encephalopathy due to excessive intake of isotonic drink or overstrict diet therapy in Japanese children.

    abstract：AIM:To report on two children with encephalopathy caused by dietary thiamine deficiency due to newly developing nutritional problems in contemporary Japan. SUBJECTS:A 1-year-old boy who had consumed 1l of isotonic drinks per day for 4 months after an episode of diarrhea, and presented with ocular movement disorder, dy...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/j.braindev.2009.08.004

    authors： Saeki K,Saito Y,Komaki H,Sakakibra T,Nakagawa E,Sugai K,Sakuma H,Sasaki M,Honda T,Hayashi H,Katori N,Miyahara Y

    更新日期：2010-08-01 00:00:00

  • A case of intraneural perineurioma presenting with monomelic atrophy in a child.

    abstract：:We report the case of an 11-year-old girl who developed slowly progressive atrophy of the left lower extremity. She suffered from mild dilated cardiomyopathy of unknown cause since 4years of age. When she was 7years old, her family noticed that her left extremity was thinner compared to the right one. Computed tomogra...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/j.braindev.2009.11.005

    authors： Miyahara-Katayama A,Ohya Y,Omi T,Komaki H,Nonaka I,Sato N,Sasaki M

    更新日期：2010-04-01 00:00:00

  • The changes in the topological properties of brain structural network based on diffusion tensor imaging in pediatric epilepsy patients with vagus nerve stimulators: A graph theoretical analysis.

    abstract：PURPOSE:This study aimed to analyze the topological characteristics of brain structural network in pediatric epilepsy patients with vagus nerve stimulation (VNS) by applying graph theoretical approaches. METHODS:Nine patients with generalized seizures and eight normal controls (NC) were enrolled. Based on diffusion te...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/j.braindev.2020.07.006

    authors： Zhu J,Xu C,Zhang X,Qiao L,Wang X,Zhang X,Yan X,Ni D,Yu T,Zhang G,Li Y

    更新日期：2021-01-01 00:00:00

  • tuberous sclerosis: hydroxyproline content in urine and tissues.

    abstract：:To elucidate the nature of an overabundance of collagen seen on microscopic examination in tuberous sclerous (TS), the hydroxyproline content in tissues and urine was determined. TS tissues of 5 patients were obtained on necropsy or plastic surgery. Urine was collected from 10 patients with TS and 19 controls. Tumors ...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/s0387-7604(81)80009-5

    authors： Tanaka H,Arima M

    更新日期：1981-01-01 00:00:00

  • Widespread pH abnormalities in patients with malformations of cortical development and epilepsy: a phosphorus-31 brain MR spectroscopy study.

    abstract：INTRODUCTION:Neuroimaging studies demonstrate that not only the lesions of malformations of cortical development (MCD) but also the normal-appearing parenchyma (NAP) present metabolic impairments, as revealed with (1)H-MRS. We have previously detected biochemical disturbances in MCD lesions with phosphorus-31 magnetic ...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/j.braindev.2013.12.010

    authors： Andrade CS,Otaduy MC,Valente KD,Park EJ,Kanas AF,Silva Filho MR,Tsunemi MH,Leite CC

    更新日期：2014-11-01 00:00:00

  • MicroRNA-31 regulating apoptosis by mediating the phosphatidylinositol-3 kinase/protein kinase B signaling pathway in treatment of spinal cord injury.

    abstract：:Apoptosis is a highly conservative energy demand program for non-inflammatory cell death, which is extremely significant in normal physiology and disease. There are many techniques used for studying apoptosis. MicroRNA (miRNA) is closely related to cell apoptosis, and especially microRNA-31 (miR-31) is involved in apo...

    journal_title：Brain & development

    pub_type： 杂志文章,评审

    doi：10.1016/j.braindev.2019.04.010

    authors： Wang Y,Yuan Y,Gao Y,Li X,Tian F,Liu F,Du R,Li P,Wang F,Xu S,Wu X,Wang C

    更新日期：2019-09-01 00:00:00

  • Clinicopathological differences between juvenile and late infantile metachromatic leukodystrophy.

    abstract：:The autopsy report of the juvenile type of metachromatic leukodystrophy is rare. The clinical and pathological difference between the juvenile and the late infantile type of metachromatic leukodystrophy was described. Loss of myelin sheaths was much less in the brain stem and spinal cord in the juvenile type than in t...

    journal_title：Brain & development

    pub_type： 杂志文章

    doi：10.1016/s0387-7604(81)80065-4

    authors： Takashima S,Matsui A,Fujii Y,Nakamura H

    更新日期：1981-01-01 00:00:00