Decoupling of autonomic and cognitive emotional reactions after cerebellar stroke.

abstract：:The subtype IV of Machado-Joseph disease (MJD), characterized by parkinsonism variably combined with ataxia, distal atrophy, and sensory loss, has been all but ignored in recent reports of MJD, including those describing the molecular biologic substrate of the disease. We have demonstrated expansion of the CAG trinucl...

journal_title：Annals of neurology

authors： Tuite PJ,Rogaeva EA,St George-Hyslop PH,Lang AE

更新日期：1995-10-01 00:00:00

abstract：:A prospective clinical and biochemical study on the effects of treatment with haloperidol has been performed in seven patients with Tourette syndrome. Pretreatment cerebrospinal fluid levels of homovanillic acid (CSF HVA) were significantly reduce in all patients, whereas 5-hydroxyindoleacetic acid was reduced in only...

journal_title：Annals of neurology

authors： Singer HS,Butler IJ,Tune LE,Seifert WE Jr,Coyle JT

更新日期：1982-10-01 00:00:00

abstract：OBJECTIVE: METHODS:We describe biochemically and clinically relevant aspects of mitochondrial ATP synthase, the enzyme that supplies most ATP for the cells energy demand. RESULTS:Analyzing human Rho zero cells we could identify three subcomplexes of ATP synthase: F1 catalytic domain, F1 domain with bound natural IF1 ...

journal_title：Annals of neurology

authors： Carrozzo R,Wittig I,Santorelli FM,Bertini E,Hofmann S,Brandt U,Schägger H

更新日期：2006-02-01 00:00:00

abstract：:Patients with senile dementia of the Alzheimer type frequently have difficulty performing visual tasks. These difficulties may be due, at least partially, to degenerative changes in both the primary visual pathway and the visual association areas. To determine whether retinal ganglion cell dysfunction contributes to v...

journal_title：Annals of neurology

authors： Trick GL,Barris MC,Bickler-Bluth M

更新日期：1989-08-01 00:00:00

abstract：:An in vitro preparation has been developed in which epileptogenesis in mammalian central nervous system tissue may be studied. Addition of sodium penicillin to the medium bathing slices of guinea pig hippocampus induced epileptiform activity similar to that seen in hippocampal penicillin foci in vivo. Epileptiform eve...

journal_title：Annals of neurology

authors： Schwartzkroin PA,Prince DA

更新日期：1977-05-01 00:00:00

abstract：:Recent reports showed many patients with chronic fatigue syndrome (CFS) harbor a retrovirus, xenotropic murine leukemia-related virus (XMRV), in blood; other studies could not replicate this finding. A useful next step would be to examine cerebrospinal fluid, because in some patients CFS is thought to be a brain disor...

journal_title：Annals of neurology

authors： Schutzer SE,Rounds MA,Natelson BH,Ecker DJ,Eshoo MW

更新日期：2011-04-01 00:00:00

abstract：:We assessed nigrostriatal dopaminergic function in Parkinson's disease (PD) patients undergoing a double-blind, placebo-controlled surgical trial of embryonic dopamine cell implantation. Forty PD patients underwent positron emission tomography (PET) imaging with [18F]fluorodopa (FDOPA) prior to randomization to transp...

journal_title：Annals of neurology

authors： Nakamura T,Dhawan V,Chaly T,Fukuda M,Ma Y,Breeze R,Greene P,Fahn S,Freed C,Eidelberg D

更新日期：2001-08-01 00:00:00

abstract：:Congenital amusia is a lifelong disability that prevents afflicted individuals from enjoying music as ordinary people do. The deficit is limited to music and cannot be explained by prior brain lesion, hearing loss, or any cognitive or socio-affective disturbance. Recent behavioral results suggest that this disorder is...

journal_title：Annals of neurology

authors： Peretz I,Brattico E,Tervaniemi M

更新日期：2005-09-01 00:00:00

abstract：:Intracranial abscesses are uncommon in the newborn. Observation of 2 infants and a review of the literature reveal that the offending organism was a Citrobacter in 12 of the 16 documented cases. In the Neonatal Meningitis Cooperative Study this organism was responsible for meningitis in only 4%. However, an analysis o...

journal_title：Annals of neurology

authors： Curless RG

更新日期：1980-09-01 00:00:00

abstract：:Pyruvate dehydrogenase deficiency is a major cause of primary lactic acidosis and neurological dysfunction in infancy and early childhood. Most cases are caused by mutations in the X-linked gene for the E1alpha subunit of the complex. Mutations in DLAT, the gene encoding dihydrolipoamide acetyltransferase, the E2 core...

journal_title：Annals of neurology

authors： Head RA,Brown RM,Zolkipli Z,Shahdadpuri R,King MD,Clayton PT,Brown GK

更新日期：2005-08-01 00:00:00

abstract：:Saturated very long-chain fatty acids in erythrocyte membranes, blood plasma, and mononuclear cells were studied in 4 patients with childhood-adolescent adrenoleukodystrophy and 4 patients with adult adrenoleukodystrophy and 19 normal control subjects by using high-performance liquid chromatography. Ratios of C26:0 to...

journal_title：Annals of neurology

authors： Antoku Y,Koike F,Ohtsuka Y,Sakai T,Tsukamoto K,Nagara H,Iwashita H,Goto I

更新日期：1991-07-01 00:00:00

abstract：:The regional metabolic rate for glucose (rMRglu) was studied in macaque monkeys after spinal cord transection at T10, both during spinal shock and after reflexes had returned. The rMRglu was measured in all Rexed layers in cord segments both caudal and rostral to the level of the transection utilizing the quantitative...

journal_title：Annals of neurology

authors： Schwartzman RJ,Eidelberg E,Alexander GM,Yu J

更新日期：1983-07-01 00:00:00

abstract：:A 30-year-old male drug abuser developed ophthalmoplegia, bulbar paralysis, and limb weakness responsive to edrophonium. However, potentiation of a low-amplitude evoked muscle action potential was produced with repetitive nerve stimulation at 10 Hz, and the clinical and electrophysiological data suggested the diagnosi...

journal_title：Annals of neurology

authors： Rapoport S,Watkins PB

更新日期：1984-09-01 00:00:00

abstract：:The worldwide epidemiology of Creutzfeldt-Jakob disease (CJD) is presented from an analysis of 1,435 patients. In the United States, the average annual mortality rate is at least 0.26 deaths per million. Temporal-spatial clustering of cases was not found in the United States, but reports from other countries indicate ...

journal_title：Annals of neurology

authors： Masters CL,Harris JO,Gajdusek DC,Gibbs CJ Jr,Bernoulli C,Asher DM

更新日期：1979-02-01 00:00:00

abstract：OBJECTIVE:To identify novel autoantibodies for neuropathic pain (NeP). METHODS:We screened autoantibodies that selectively bind to mouse unmyelinated C-fiber type dorsal root ganglion (DRG) neurons using tissue-based indirect immunofluorescence assays (IFA) with sera from 110 NeP patients with various inflammatory and...

journal_title：Annals of neurology

authors： Fujii T,Yamasaki R,Iinuma K,Tsuchimoto D,Hayashi Y,Saitoh BY,Matsushita T,Kido MA,Aishima S,Nakanishi H,Nakabeppu Y,Kira JI

更新日期：2018-08-01 00:00:00

abstract：:Two distinct deficits of ocular smooth pursuit could be demonstrated using two types of visual stimulus motion in a patient who had suffered bilateral occipitoparietal lobe infarction. First, in response to constant-velocity (zero acceleration) target motion, smooth pursuit gain (eye velocity/target velocity) was mild...

journal_title：Annals of neurology

authors： Leigh RJ,Tusa RJ

更新日期：1985-02-01 00:00:00

abstract：:We have recently reported homozygous mutations in the PINK1 gene in three consanguineous families with early-onset parkinsonism (EOP) linked to the PARK6 locus. To further evaluate the pathogenic role of PINK1 in EOP and to draw genotype-phenotype correlates, we performed PINK1 mutation analysis in a cohort of Italian...

journal_title：Annals of neurology

authors： Valente EM,Salvi S,Ialongo T,Marongiu R,Elia AE,Caputo V,Romito L,Albanese A,Dallapiccola B,Bentivoglio AR

更新日期：2004-09-01 00:00:00

abstract：:Classic late infantile neuronal ceroid lipofuscinosis (LINCL) is a neurodegenerative disease in which autofluorescent "curvilinear" storage bodies accumulate in tissues from affected patients. Recently, the LINCL gene (CLN2) has been found to code for a pepstatin-insensitive lysosomal protease whose activity is defici...

journal_title：Annals of neurology

authors： Berry-Kravis E,Sleat DE,Sohar I,Meyer P,Donnelly R,Lobel P

更新日期：2000-02-01 00:00:00

abstract：OBJECTIVE:Acquired epilepsy is a devastating long-term risk of various brain insults, including trauma, stroke, infections, and status epilepticus (SE). There is no preventive treatment for patients at risk. Attributable to the complex alterations involved in epileptogenesis, it is likely that multitargeted approaches ...

journal_title：Annals of neurology

authors： Bar-Klein G,Klee R,Brandt C,Bankstahl M,Bascuñana P,Töllner K,Dalipaj H,Bankstahl JP,Friedman A,Löscher W

更新日期：2016-12-01 00:00:00

abstract：:Positron emission tomography (PET) of glucose metabolism is often applied for the localization of epileptogenic brain regions, but hypometabolic areas are often larger than or can miss epileptogenic cortex in nonlesional neocortical epilepsy. The present study is a three-dimensional brain surface analysis designed to ...

journal_title：Annals of neurology

authors： Juhász C,Chugani DC,Muzik O,Watson C,Shah J,Shah A,Chugani HT

更新日期：2000-07-01 00:00:00

abstract：OBJECTIVE:Neuronal channelopathies cause brain disorders, including epilepsy, migraine, and ataxia. Despite the development of mouse models, pathophysiological mechanisms for these disorders remain uncertain. One particularly devastating channelopathy is Dravet syndrome (DS), a severe childhood epilepsy typically cause...

journal_title：Annals of neurology

authors： Liu Y,Lopez-Santiago LF,Yuan Y,Jones JM,Zhang H,O'Malley HA,Patino GA,O'Brien JE,Rusconi R,Gupta A,Thompson RC,Natowicz MR,Meisler MH,Isom LL,Parent JM

更新日期：2013-07-01 00:00:00

abstract：:A comprehensive analysis of cerebral hemodynamics and metabolism was carried out in 14 patients with pseudotumor cerebri. Tracer techniques were employed to measure cerebral blood flow (CBF) and vascular reactivity to acute changes in arterial carbon dioxide tension and blood pressure, cerebral blood volume (CBV), and...

journal_title：Annals of neurology

authors： Raichle ME,Grubb RL Jr,Phelps ME,Gado MH,Caronna JJ

更新日期：1978-08-01 00:00:00

abstract：:Two members of a family with a neuropathy resembling Charcot-Marie-Tooth disease were unable to relax their muscles after voluntary contraction. Muscle spasm often outlasted voluntary contraction by 30 seconds or more before subsiding into myokymia and fasciculations. The posture of the hand during muscle spasm resemb...

journal_title：Annals of neurology

authors： Lance JW,Burke D,Pollard J

更新日期：1979-06-01 00:00:00

abstract：:Positron emission tomography (PET) of brain glucose utilization is highly sensitive in detecting focal cortical abnormalities in patients with infantile spasms even when the computed tomographic (CT) and magnetic resonance imaging (MRI) scans are normal. Of 110 infants with spasms evaluated for potential surgical inte...

journal_title：Annals of neurology

authors： Chugani HT,Da Silva E,Chugani DC

更新日期：1996-05-01 00:00:00

abstract：:We studied rhesus monkeys with hemiparkinsonism or bilateral parkinsonism produced by unilateral or bilateral intracarotid administration of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine. Using a standardized clinical rating scale, 4 hemiparkinsonian monkeys showed a 13 to 56% (mean, 36%) spontaneous improvement during...

journal_title：Annals of neurology

authors： Kurlan R,Kim MH,Gash DM

更新日期：1991-06-01 00:00:00

abstract：:Protease-resistant prion protein, total prion protein, and glial fibrillary acidic protein were measured in various brain regions from 9 subjects with fatal familial insomnia. Six were homozygotes methionine/methionine at codon 129 (mean duration, 10.7 +/- 4 months) and 3 were heterozygotes methionine/valine (mean dur...

journal_title：Annals of neurology

authors： Parchi P,Castellani R,Cortelli P,Montagna P,Chen SG,Petersen RB,Manetto V,Vnencak-Jones CL,McLean MJ,Sheller JR

更新日期：1995-07-01 00:00:00

abstract：:We report on a new allele at the arylsulfatase A (ARSA) locus causing late-onset metachromatic leukodystrophy (MLD). In that allele arginine84, a residue that is highly conserved in the arylsulfatase gene family, is replaced by glutamine. In contrast to alleles that cause early-onset MLD, the arginine84 to glutamine s...

journal_title：Annals of neurology

authors： Kappler J,von Figura K,Gieselmann V

更新日期：1992-03-01 00:00:00

abstract：OBJECTIVE:A 12-month double-blind sham-surgery-controlled trial assessing adeno-associated virus type 2 (AAV2)-neurturin injected into the putamen bilaterally failed to meet its primary endpoint, but showed positive results for the primary endpoint in the subgroup of subjects followed for 18 months and for several seco...

journal_title：Annals of neurology

authors： Warren Olanow C,Bartus RT,Baumann TL,Factor S,Boulis N,Stacy M,Turner DA,Marks W,Larson P,Starr PA,Jankovic J,Simpson R,Watts R,Guthrie B,Poston K,Henderson JM,Stern M,Baltuch G,Goetz CG,Herzog C,Kordower JH,Alt

更新日期：2015-08-01 00:00:00

abstract：:Malignant tumors are known to have a "remote" or nonmetastatic effect on the central and peripheral nervous systems. Eight patients were seen with proximal muscle weakness in association with bronchogenic carcinoma (5), carcinoma of breast (2), and leukemia (1). Electromyography demonstrated small polyphasic motor uni...

journal_title：Annals of neurology

authors： Barron SA,Heffner RR Jr

更新日期：1978-09-01 00:00:00

abstract：:To characterize the protein composition of degenerating neurons in Alzheimer disease, enriched fractions of isolated cortical neurons from postmortem Alzheimer brain were compared by ultrastructural and biochemical techniques to neuronal isolates from aged normal controls and from patients with the nonfibrillary degen...

journal_title：Annals of neurology

authors： Selkoe DJ

更新日期：1980-11-01 00:00:00