Amyotrophic lateral sclerosis: Problems and prospects.

abstract：:Excessive astrocytosis in cortical tubers in tuberous sclerosis complex (TSC) suggests that astrocytes may be important for epileptogenesis in TSC. We previously demonstrated that astrocyte-specific Tsc1 gene inactivation in mice (Tsc1 cKO mice) results in progressive epilepsy. Here, we report that glutamate transport...

journal_title：Annals of neurology

authors： Wong M,Ess KC,Uhlmann EJ,Jansen LA,Li W,Crino PB,Mennerick S,Yamada KA,Gutmann DH

更新日期：2003-08-01 00:00:00

abstract：OBJECTIVE:C9orf72 expansion is the most common genetic cause of frontotemporal dementia (FTD). We examined aging trajectories of cortical thickness (CTh) and surface area in C9orf72 expansion adult carriers compared to healthy controls to characterize preclinical cerebral changes leading to symptoms. METHODS:Data were...

journal_title：Annals of neurology

authors： Le Blanc G,Jetté Pomerleau V,McCarthy J,Borroni B,van Swieten J,Galimberti D,Sanchez-Valle R,LaForce R Jr,Moreno F,Synofzik M,Graff C,Masellis M,Tartaglia MC,Rowe JB,Vandenberghe R,Finger E,Tagliavini F,de Mendonça A,

更新日期：2020-07-01 00:00:00

abstract：OBJECTIVE:Hemodynamic impairment in one hemisphere has been shown to trigger ipsilateral motor activation in the opposite hemisphere on functional imaging. We hypothesized that reversing the hypoperfusion would normalize the motor activation pattern. METHODS:We studied four patients with high-grade stenosis and impair...

journal_title：Annals of neurology

authors： Chmayssani M,Lazar RM,Hirsch J,Marshall RS

更新日期：2009-02-01 00:00:00

abstract：OBJECTIVE:Case series with (11)C-PK11195 and positron emission tomography (PET) in stroke patients suggest that activated microglia may be detected in remote brain regions with fiber tract connections to the lesion site as an indicator of poststroke neuroinflammation. However, the specificity of these imaging findings ...

journal_title：Annals of neurology

authors： Radlinska BA,Ghinani SA,Lyon P,Jolly D,Soucy JP,Minuk J,Schirrmacher R,Thiel A

更新日期：2009-12-01 00:00:00

abstract：OBJECTIVE:The risk for symptomatic intracerebral hemorrhage (sICH) associated with thrombolytic treatment has not been evaluated in large studies using diffusion-weighted imaging (DWI). Here, we investigated the relation between pretreatment DWI lesion size and the risk for sICH after thrombolysis. METHODS:In this ret...

journal_title：Annals of neurology

authors： Singer OC,Humpich MC,Fiehler J,Albers GW,Lansberg MG,Kastrup A,Rovira A,Liebeskind DS,Gass A,Rosso C,Derex L,Kim JS,Neumann-Haefelin T,MR Stroke Study Group Investigators.

更新日期：2008-01-01 00:00:00

abstract：:Endoneurial fluid pressure (EFP) was recorded by an active, servo-null pressure system after a glass micropipette was inserted into rat sciatic nerve undergoing wallerian degeneration. The lesions were produced by crushing the left sciatic nerve of the anesthetized animal at its point of entry into the thigh. Eighty-f...

journal_title：Annals of neurology

authors： Powell HC,Myers RR,Costello ML,Lampert PW

更新日期：1979-06-01 00:00:00

abstract：:The current functional model of the basal ganglia suggests that dyskinesia results from abnormally low activity at the output of the system. This view appears incomplete. The recent literature suggests other factors. Thus, dyskinesia may result from disturbance of surround inhibition: a physiologic mechanism to select...

journal_title：Annals of neurology

authors： Filion M

更新日期：2000-04-01 00:00:00

abstract：:The characteristic pathological features of Huntington's disease (HD) are neostriatal atrophy and neuronal loss. Although neuroradiological studies often show caudate atrophy in patients with moderate HD, frequently no caudate atrophy is found early in the illness. There have been no quantitative reports to date on in...

journal_title：Annals of neurology

authors： Harris GJ,Pearlson GD,Peyser CE,Aylward EH,Roberts J,Barta PE,Chase GA,Folstein SE

更新日期：1992-01-01 00:00:00

abstract：:A subset of human immunodeficiency virus (HIV)-infected patients develop persistent CD8 hyperlymphocytosis and a Sjogren's syndrome-like syndrome associated with multivisceral CD8 T-cell infiltration, known as the diffuse infiltrative lymphocytosis syndrome (DILS). Patients with DILS tend to have higher CD4 cell count...

journal_title：Annals of neurology

authors： Moulignier A,Authier FJ,Baudrimont M,Pialoux G,Belec L,Polivka M,Clair B,Gray F,Mikol J,Gherardi RK

更新日期：1997-04-01 00:00:00

abstract：:We studied neuronal activity and its relation to the accumulation of neurofibrillary tangles in Alzheimer's disease (AD) neurons by in situ hybridization to cytochrome oxidase subunit III messenger RNA, a marker of mitochondrial energy metabolism. In AD midtemporal cortex, levels of cytochrome oxidase subunit III mess...

journal_title：Annals of neurology

authors： Hatanpää K,Brady DR,Stoll J,Rapoport SI,Chandrasekaran K

更新日期：1996-09-01 00:00:00

abstract：:The pathogenetic role of anti-GM1 in chronic acquired demyelinating polyneuropathy (CADP) is uncertain. An association between antibodies and disease activity has not yet been established. In 8 patients with CADP followed longitudinally, anti-GM1 antibodies were monitored with a standardized enzyme-linked immunosorben...

journal_title：Annals of neurology

authors： Bech E,Andersen H,Orntoft TF,Jakobsen J

更新日期：1998-01-01 00:00:00

abstract：:Parkinson's disease evolves slowly, and there is current interest in exploring the earliest stages of the disorder, because of new approaches to studying pathogenesis and developing potential neuroprotective treatment. Recognizing early Parkinson's disease is not easy. The certainty of diagnosis increases as the disea...

journal_title：Annals of neurology

authors： Calne DB,Snow BJ,Lee C

更新日期：1992-01-01 00:00:00

abstract：:The possibility of detecting acute hypoxic-ischemic brain lesions by prenatal magnetic resonance imaging or ultrasound is low. We present a case of a fetus with a vein of Galen arteriovenous malformation in whom prenatal diffusion-weighted magnetic resonance imaging at 33 weeks of gestation clearly detected cerebral a...

journal_title：Annals of neurology

authors： Baldoli C,Righini A,Parazzini C,Scotti G,Triulzi F

更新日期：2002-08-01 00:00:00

abstract：:Pallidopyramidal disease is a rare disease of young patients in which they manifest a parkinsonian syndrome and pyramidal signs. Pallidopyramidal disease has been attributed to a degeneration of the pallidum and the pyramidal tract, although only 1 patient has been studied postmortem. In the present report, [18F]fluor...

journal_title：Annals of neurology

authors： Remy P,Hosseini H,Degos JD,Samson Y,Agid Y,Warter JM,Vidailhet M

更新日期：1995-12-01 00:00:00

abstract：OBJECTIVE:Nonketotic hyperglycinemia is a neurometabolic disorder characterized by intellectual disability, seizures, and spasticity. Patients with attenuated nonketotic hyperglycinemia make variable developmental progress. Predictive factors have not been systematically assessed. METHODS:We reviewed 124 patients stra...

journal_title：Annals of neurology

authors： Swanson MA,Coughlin CR Jr,Scharer GH,Szerlong HJ,Bjoraker KJ,Spector EB,Creadon-Swindell G,Mahieu V,Matthijs G,Hennermann JB,Applegarth DA,Toone JR,Tong S,Williams K,Van Hove JL

更新日期：2015-10-01 00:00:00

abstract：:Although Alzheimer's and Parkinson's diseases predominately affect elderly adults, the proteins that play a role in the pathogenesis of these diseases are expressed throughout life. In fact, many of the proteins hypothesized to be important in the progression of neurodegeneration play direct or indirect roles in the d...

journal_title：Annals of neurology

authors： Rogers D,Schor NF

更新日期：2010-02-01 00:00:00

abstract：:We studied patients with partial and primary generalized seizures using fluorine-18-labeled 2-fluorodeoxyglucose and positron emission tomography. Interictal studies of patients with partial seizures showed regions of focal or lateralized hypometabolism in 15 of 17 patients with unilateral electroencephalographic foci...

journal_title：Annals of neurology

authors： Theodore WH,Brooks R,Sato S,Patronas N,Margolin R,Di Chiro G,Porter RJ

更新日期：1984-01-01 00:00:00

abstract：:In inclusion body myositis, T cells invade and destroy nonnecrotic muscle fibers. The mechanism by which T cells damage muscle fibers in inflammatory myopathies is not known. In this study we have investigated the expression of Fas and Fas ligand in muscle in five patients with inclusion body myositis. Reverse transcr...

journal_title：Annals of neurology

authors： Fyhr IM,Oldfors A

更新日期：1998-01-01 00:00:00

abstract：:Cerebrospinal fluid and plasma vasopressin were measured in patients with cerebral disorders associated with varying levels of elevated intracranial pressure. The mean cerebrospinal fluid vasopressin concentration was significantly increased in patients with pseudotumor cerebri (2.0 +/- 0.2 [SEM] pg/ml), intracranial ...

journal_title：Annals of neurology

authors： Sørensen PS,Gjerris F,Hammer M

更新日期：1984-05-01 00:00:00

abstract：OBJECTIVE:Our goal was to define the genetic cause of the profound hypomyelination in the taiep rat model and determine its relevance to human white matter disease. METHODS:Based on previous localization of the taiep mutation to rat chromosome 9, we tested whether the mutation resided within the Tubb4a (β-tubulin 4A) ...

journal_title：Annals of neurology

authors： Duncan ID,Bugiani M,Radcliff AB,Moran JJ,Lopez-Anido C,Duong P,August BK,Wolf NI,van der Knaap MS,Svaren J

更新日期：2017-05-01 00:00:00

abstract：:D2 dopamine receptor densities were measured in postmortem samples of the caudate nucleus and putamen from 36 parkinsonian patients. The relationship between the age of the patient, duration of the disease, and duration of L-dopa therapy versus density of brain D2 dopamine receptors was examined using [3H]spiperone. R...

journal_title：Annals of neurology

authors： Guttman M,Seeman P,Reynolds GP,Riederer P,Jellinger K,Tourtellotte WW

更新日期：1986-05-01 00:00:00

abstract：:The need for novel, efficacious, antiseizure therapies is widely acknowledged. This study investigates in humans the feasibility, safety, and efficacy of high-frequency electrical stimulation (HFES; 100-500 Hz) triggered by automated seizure detections. Eight patients were enrolled in this study, which consisted of a ...

journal_title：Annals of neurology

authors： Osorio I,Frei MG,Sunderam S,Giftakis J,Bhavaraju NC,Schaffner SF,Wilkinson SB

更新日期：2005-02-01 00:00:00

abstract：:N-acetylaspartate (NAA) contributes to the most prominent signal in proton magnetic resonance spectroscopy (1H-MRS) of the adult human brain. We report the absence of NAA in the brain of a 3-year-old child with neurodevelopmental retardation and moderately delayed myelination. Since normal concentration of NAA in body...

journal_title：Annals of neurology

authors： Martin E,Capone A,Schneider J,Hennig J,Thiel T

更新日期：2001-04-01 00:00:00

abstract：OBJECTIVE:In multiple sclerosis (MS), cerebral gray matter (GM) atrophy correlates more strongly than white matter (WM) atrophy with disability. The corresponding relationships in the spinal cord (SC) are unknown due to technical limitations in assessing SC GM atrophy. Using phase-sensitive inversion recovery (PSIR) ma...

journal_title：Annals of neurology

authors： Schlaeger R,Papinutto N,Panara V,Bevan C,Lobach IV,Bucci M,Caverzasi E,Gelfand JM,Green AJ,Jordan KM,Stern WA,von Büdingen HC,Waubant E,Zhu AH,Goodin DS,Cree BA,Hauser SL,Henry RG

更新日期：2014-10-01 00:00:00

abstract：:We have analyzed muscle biopsy specimens from 48 patients with biochemically proven phosphorylase deficiency (McArdle's disease) by sodium dodecylsulphate polyacrylamide gel electrophoresis (SDS-PAGE), immunoblotting, and immunotitration (enzyme-linked immunosorbent assay [ELISA]). Thirty-five of the 42 patients studi...

journal_title：Annals of neurology

authors： Servidei S,Shanske S,Zeviani M,Lebo R,Fletterick R,DiMauro S

更新日期：1988-12-01 00:00:00

abstract：:Mutations in DJ-1 (PARK7) have been reported in two consanguineous families with young-onset Parkinson's disease (YOPD). This study aims to confirm the presence of pathogenic DJ-1 mutations and determine their contribution in young-onset and more typical later onset Parkinson's disease (PD). The entire open reading fr...

journal_title：Annals of neurology

authors： Abou-Sleiman PM,Healy DG,Quinn N,Lees AJ,Wood NW

更新日期：2003-09-01 00:00:00

abstract：:Regional patterns of cerebral glucose utilization (rCMRglc) and blood flow (rCBF) were examined in the early recovery period following transient forebrain ischemia in order to correlate early postischemic physiological events with regionally selective patterns of ischemic neuropathology. Wistar rats were subjected to ...

journal_title：Annals of neurology

authors： Ginsberg MD,Graham DI,Busto R

更新日期：1985-10-01 00:00:00

abstract：:The unloading reflex was measured in the intrinsic muscles of the hand. By removing various fractions of the load on the muscles, we determined the size of the electromyographic response as a function of the change in the external force. This technique was applied to both hands of a patient with a pontine lesion who h...

journal_title：Annals of neurology

authors： Angel RW,Goldstein M

更新日期：1983-03-01 00:00:00

abstract：:One of the most striking manifestations of Down's syndrome is profound mental retardation. Furthermore, after 35 years of age, many patients with Down's syndrome develop clinical and pathological features of Alzheimer's disease. Since brains of patients with Alzheimer's disease show significant loss of neurons in the ...

journal_title：Annals of neurology

authors： Casanova MF,Walker LC,Whitehouse PJ,Price DL

更新日期：1985-09-01 00:00:00

abstract：:The blood-brain barrier is a natural diffusion barrier, which expresses active carriers extruding drugs on their way to the brain back into the blood against concentration gradients. Whereas these so-called adenosine triphosphate-binding cassette (ABC) transporters prevent the brain entry of toxic compounds under phys...

journal_title：Annals of neurology

authors： Hermann DM,Kilic E,Spudich A,Krämer SD,Wunderli-Allenspach H,Bassetti CL

更新日期：2006-11-01 00:00:00