Infantile spasms: III. Prognostic implications of bitemporal hypometabolism on positron emission tomography.

abstract：OBJECTIVE:We tested the premise that cholinesterase inhibitor therapy should target butyrylcholinesterase (BuChE) in Alzheimer's disease (AD), not acetylcholinesterase (AChE) alone, because both enzymes hydrolyze acetylcholine, and BuChE is increased in AD cerebral cortex. METHODS:To examine this issue in vivo, we qua...

journal_title：Annals of neurology

authors： Kuhl DE,Koeppe RA,Snyder SE,Minoshima S,Frey KA,Kilbourn MR

更新日期：2006-01-01 00:00:00

abstract：:A total of 36 patients with Batten disease (juvenile-onset neuronal ceroid lipofuscinosis), homozygous or heterozygous for the major mutation, a 1.02-kb deletion, in the CLN3 gene, were studied to relate their genotype to their clinical phenotype. The onset of visual failure and epilepsy was highly concordant in both ...

journal_title：Annals of neurology

authors： Järvelä I,Autti T,Lamminranta S,Aberg L,Raininko R,Santavuori P

更新日期：1997-11-01 00:00:00

abstract：:To elucidate the role of D-serine in human central nervous system, we analyzed D-serine, L-serine, and glycine concentrations in cerebrospinal fluid of healthy children and children with a defective L-serine biosynthesis (3-phosphoglycerate dehydrogenase deficiency). Healthy children showed high D-serine concentration...

journal_title：Annals of neurology

authors： Fuchs SA,Dorland L,de Sain-van der Velden MG,Hendriks M,Klomp LW,Berger R,de Koning TJ

更新日期：2006-10-01 00:00:00

abstract：:To establish phenotype-genotype correlations in early-onset parkinsonism, we have compared the phenotype of a large series of 146 patients with and 250 patients without parkin mutations. Although no single sign distinguished the groups, patients with mutations had significantly earlier and more symmetrical onset, dyst...

journal_title：Annals of neurology

authors： Lohmann E,Periquet M,Bonifati V,Wood NW,De Michele G,Bonnet AM,Fraix V,Broussolle E,Horstink MW,Vidailhet M,Verpillat P,Gasser T,Nicholl D,Teive H,Raskin S,Rascol O,Destée A,Ruberg M,Gasparini F,Meco G,Agid Y,Du

更新日期：2003-08-01 00:00:00

abstract：:A case-control study was performed to determine the possible roles of various environmental factors, prior illnesses, drug use, and personal habits in the development of Alzheimer's disease. Such information was collected from 40 patients with onset of dementia prior to age 70 and from 80 community control subjects ma...

journal_title：Annals of neurology

authors： Heyman A,Wilkinson WE,Stafford JA,Helms MJ,Sigmon AH,Weinberg T

更新日期：1984-04-01 00:00:00

abstract：OBJECTIVE:Traumatic brain injury (TBI) often results in traumatic axonal injury (TAI). This can be difficult to identify using conventional imaging. Diffusion tensor imaging (DTI) offers a method of assessing axonal damage in vivo, but has previously mainly been used to investigate groups of patients. Machine learning ...

journal_title：Annals of neurology

authors： Hellyer PJ,Leech R,Ham TE,Bonnelle V,Sharp DJ

更新日期：2013-04-01 00:00:00

abstract：:Reductions in cerebral metabolism sufficient to impair cognition in normal individuals also occur in Alzheimer's disease (AD). The degree of clinical disability in AD correlates closely to the magnitude of the reduction in brain metabolism. Therefore, we tested whether impairments in tricarboxylic acid (TCA) cycle enz...

journal_title：Annals of neurology

authors： Bubber P,Haroutunian V,Fisch G,Blass JP,Gibson GE

更新日期：2005-05-01 00:00:00

abstract：OBJECTIVE:Somnambulism affects up to 4% of adults and constitutes one of the leading causes of sleep-related violence and self-injury. Diagnosing somnambulism with objective instruments is often difficult because episodes rarely occur in the laboratory. Because sleep deprivation can precipitate sleepwalking, we aimed t...

journal_title：Annals of neurology

authors： Zadra A,Pilon M,Montplaisir J

更新日期：2008-04-01 00:00:00

abstract：:Two families with a point mutation in mtDNA associated with myoclonic epilepsy and ragged-red fiber disease showed pronounced clinical heterogeneity. The mothers of the two families had adult-onset myopathy with ragged-red fibers, partial deficiency of cytochrome c oxidase, and sensory neuropathy. Members of the first...

journal_title：Annals of neurology

authors： Graf WD,Sumi SM,Copass MK,Ojemann LM,Longstreth WT Jr,Shanske S,Lombes A,DiMauro S

更新日期：1993-06-01 00:00:00

abstract：:Charcot-Marie-Tooth (CMT) neuropathies are inherited neuromuscular disorders caused by a length-dependent neurodegeneration of peripheral nerves. More than 900 mutations in 60 different genes are causative of the neuropathy. Despite significant progress in therapeutic strategies, the disease remains incurable. The inc...

journal_title：Annals of neurology

authors： Bouhy D,Timmerman V

更新日期：2013-09-01 00:00:00

abstract：:The role of mitochondrial DNA (mtDNA) mutations in the pathogenesis of Leber's hereditary optic neuropathy (LHON) has yet to be characterized. Several clinical features of the disease imply that nuclear genes might also be involved in its expression. We have confirmed the presence of a severe NADH:coenzyme Q1 reductas...

journal_title：Annals of neurology

authors： Cock HR,Tabrizi SJ,Cooper JM,Schapira AH

更新日期：1998-08-01 00:00:00

abstract：:We studied sleep patterns for three nights in 10 subjects with moderate to severe progressive supranuclear palsy and correlated the findings with disease severity using quantitative measures of motor, cognitive, and eye movement impairment. All subjects had severe insomnia, spending 2 to 6 hours awake per night; the m...

journal_title：Annals of neurology

authors： Aldrich MS,Foster NL,White RF,Bluemlein L,Prokopowicz G

更新日期：1989-06-01 00:00:00

abstract：:A sensitive enzyme-linked immunosorbent assay method was employed to measure interleukin-2 (IL-2) levels in cerebrospinal fluid (CSF) and sera from 30 patients with multiple sclerosis (MS) and 8 patients with other neurological diseases. Detectable levels of IL-2 were found in 6 sera and 9 CSF samples of 21 patients w...

journal_title：Annals of neurology

authors： Gallo P,Piccinno M,Pagni S,Tavolato B

更新日期：1988-12-01 00:00:00

abstract：:Mitochondria are cellular organelles crucial for energy supply and calcium homeostasis in neuronal cells, and their dysfunction causes seizure activity in some rare human epilepsies. To directly test whether mitochondrial respiratory chain enzymes are abnormal in the most common form of chronic epilepsy, temporal lobe...

journal_title：Annals of neurology

authors： Kunz WS,Kudin AP,Vielhaber S,Blümcke I,Zuschratter W,Schramm J,Beck H,Elger CE

更新日期：2000-11-01 00:00:00

abstract：:Alterations of nodal and paranodal axolemma of the rat sciatic nerve were investigated in antigalactocerebroside serum-induced demyelination. A ferric ion-ferrocyanide (FeFCN) stain that appears to stain the regions with a high sodium channel density in nerve fibers was applied. When acute conduction block was initiat...

journal_title：Annals of neurology

authors： Saida K,Saida T,Kayama H,Nishitani H

更新日期：1984-06-01 00:00:00

abstract：OBJECTIVE:Guillain-Barré syndrome (GBS) is an acute postinfectious immune-mediated polyneuropathy. Although preceding respiratory tract infections with Mycoplasma pneumoniae have been reported in some cases, the role of M. pneumoniae in the pathogenesis of GBS remains unclear. We here cultured, for the first time, M. p...

journal_title：Annals of neurology

authors： Meyer Sauteur PM,Huizinga R,Tio-Gillen AP,Roodbol J,Hoogenboezem T,Jacobs E,van Rijn M,van der Eijk AA,Vink C,de Wit MC,van Rossum AM,Jacobs BC

更新日期：2016-10-01 00:00:00

abstract：:Four infants had bilateral hippocampal sclerosis by magnetic resonance scans, including oblique coronal fast spin echo images of the temporal lobes; [18F]fluorodeoxyglucose-positron emission tomographic scans, done in 2 infants, showed isolated bilateral anterior temporal lobe hypometabolism. All had epilepsy with epi...

journal_title：Annals of neurology

authors： DeLong GR,Heinz ER

更新日期：1997-07-01 00:00:00

abstract：:Four guanidino compounds that have been found to be markedly increased in cerebrospinal fluid and brain tissue of uremic patients, namely, guanidine, methylguanidine, creatinine, and guanidinosuccinic acid, were applied to mouse spinal cord neurons in primary dissociated cell culture to evaluate their effects on posts...

journal_title：Annals of neurology

authors： De Deyn PP,Macdonald RL

更新日期：1990-11-01 00:00:00

abstract：:We report an immunohistochemical study of the mitochondrial alpha-ketoglutarate dehydrogenase complex (KGDHC) in the substantia nigra in Parkinson's disease. The KGDHC, the three enzyme complex catalyzing the oxidation of alpha-ketoglutarate to succinate through succinic semialdehyde, is the rate-regulating enzyme of ...

journal_title：Annals of neurology

authors： Mizuno Y,Matuda S,Yoshino H,Mori H,Hattori N,Ikebe S

更新日期：1994-02-01 00:00:00

abstract：:There is increasing interest in the potential of dopamine agonists to provide a neuroprotective effect and to alter the natural course of levodopa-treated Parkinson's disease (PD). Theoretically, such a protective effect might derive from (a) a levodopa sparing effect, (b) stimulation dopamine autoreceptors resulting ...

journal_title：Annals of neurology

authors： Olanow CW,Jenner P,Brooks D

更新日期：1998-09-01 00:00:00

abstract：:Two extended haplotypes of the tau gene (H1 and H2) have been described. The frequency of H1 haplotype is increased in progressive supranuclear palsy (PSP). PSP is associated with filamentous tau lesions in neurons and glia, which are reportedly composed exclusively of tau isoforms with four repeats in the microtubule...

journal_title：Annals of neurology

authors： Liu WK,Le TV,Adamson J,Baker M,Cookson N,Hardy J,Hutton M,Yen SH,Dickson DW

更新日期：2001-10-01 00:00:00

abstract：:Human T-cell lymphotropic virus type III (HTLV-III) has been isolated from neural tissues and cerebrospinal fluid (CSF) of patients with neurological syndromes associated with the acquired immune deficiency syndrome (AIDS) and the virus may be directly involved in the pathogenesis of the syndromes. To detect HTLV-III ...

journal_title：Annals of neurology

authors： Gabuzda DH,Ho DD,de la Monte SM,Hirsch MS,Rota TR,Sobel RA

更新日期：1986-09-01 00:00:00

abstract：OBJECTIVE:Infantile spasms are seizures associated with a severe epileptic encephalopathy presenting in the first 2 years of life, and optimal treatment continues to be debated. This study evaluates early and sustained response to initial treatments and addresses both clinical remission and electrographic resolution of...

journal_title：Annals of neurology

authors： Knupp KG,Coryell J,Nickels KC,Ryan N,Leister E,Loddenkemper T,Grinspan Z,Hartman AL,Kossoff EH,Gaillard WD,Mytinger JR,Joshi S,Shellhaas RA,Sullivan J,Dlugos D,Hamikawa L,Berg AT,Millichap J,Nordli DR Jr,Wirrell E,

更新日期：2016-03-01 00:00:00

abstract：:Noradrenergic fibers were identified in rat cervical lymph nodes by fluorescence histochemistry and confirmed by radioenzymatic determination of norepinephrine. After superior cervical ganglionectomy, lymph node norepinephrine and noradrenergic fluorescence were strikingly decreased. The alpha-adrenergic radioligand [...

journal_title：Annals of neurology

authors： Giron LT Jr,Crutcher KA,Davis JN

更新日期：1980-11-01 00:00:00

abstract：OBJECTIVE:Osteopontin (OPN), a pleiotropic extracellular matrix glycoprotein, has been reported to be protective against ischemic lesions, but effects of OPN on vascular functions have not been investigated. The aim of this study was to assess whether recombinant OPN (r-OPN) could prevent cerebral vasospasm after subar...

journal_title：Annals of neurology

authors： Suzuki H,Hasegawa Y,Chen W,Kanamaru K,Zhang JH

更新日期：2010-11-01 00:00:00

abstract：OBJECTIVE:To develop RNA splicing biomarkers of disease severity and therapeutic response in myotonic dystrophy type 1 (DM1) and type 2 (DM2). METHODS:In a discovery cohort, we used microarrays to perform global analysis of alternative splicing in DM1 and DM2. The newly identified splicing changes were combined with p...

journal_title：Annals of neurology

authors： Nakamori M,Sobczak K,Puwanant A,Welle S,Eichinger K,Pandya S,Dekdebrun J,Heatwole CR,McDermott MP,Chen T,Cline M,Tawil R,Osborne RJ,Wheeler TM,Swanson MS,Moxley RT 3rd,Thornton CA

更新日期：2013-12-01 00:00:00

abstract：OBJECTIVE:Gray matter (GM) damage and meningeal inflammation have been associated with early disease onset and a more aggressive disease course in multiple sclerosis (MS), but can these changes be identified in the patient early in the disease course? METHODS:To identify possible biomarkers linking meningeal inflammat...

journal_title：Annals of neurology

authors： Magliozzi R,Howell OW,Nicholas R,Cruciani C,Castellaro M,Romualdi C,Rossi S,Pitteri M,Benedetti MD,Gajofatto A,Pizzini FB,Montemezzi S,Rasia S,Capra R,Bertoldo A,Facchiano F,Monaco S,Reynolds R,Calabrese M

更新日期：2018-04-01 00:00:00

abstract：:Aggregated tau proteins constitute the basic matrix of neuronal inclusions specific to numerous neurodegenerative disorders. Monodimensional and two-dimensional Western blot analyses performed on cortical brain homogenates allowed discrimination between disease-specific tau protein profiles. These observations raised ...

journal_title：Annals of neurology

authors： Delacourte A,Sergeant N,Wattez A,Gauvreau D,Robitaille Y

更新日期：1998-02-01 00:00:00

abstract：:The in vivo demyelinating capacity of sera from 27 patients with Guillain-Barré syndrome (GBS) and 47 other individuals was studied by intraneural injection into rat sciatic nerves. The morphological features of the nerves in cross section taken just proximal to the site of needle insertion was assessed 48 hours after...

journal_title：Annals of neurology

authors： Saida T,Saida K,Lisak RP,Brown MJ,Silberberg DH,Asbury AK

更新日期：1982-01-01 00:00:00

abstract：:Paired-helical filaments (PHFs) are an important diagnostic criterion of the inclusion-body myositis (IBM) muscle biopsy; but, until now, their presence could be identified only by electronmicroscopy. In this report, we describe an easy immunocytochemical procedure, utilizing commercially available antibody, that enab...

journal_title：Annals of neurology

authors： Askanas V,Alvarez RB,Mirabella M,Engel WK

更新日期：1996-03-01 00:00:00