Alexia and left homonymous hemianopia in a non-right-hander.

abstract：:Autosomal dominant spinocerebellar ataxias (SCAs) are clinically and genetically a heterogeneous group of neurodegenerative disorders. Recently, mild CAG repeat expansion in the alpha1A voltage-dependent calcium channel gene has been found to be associated with a type of autosomal dominant SCA (SCA6). We analyzed 98 J...

journal_title：Annals of neurology

authors： Ikeuchi T,Takano H,Koide R,Horikawa Y,Honma Y,Onishi Y,Igarashi S,Tanaka H,Nakao N,Sahashi K,Tsukagoshi H,Inoue K,Takahashi H,Tsuji S

更新日期：1997-12-01 00:00:00

abstract：OBJECTIVE:Transient high-frequency oscillations (HFOs; 150-600Hz) in local field potentials generated by human hippocampal and parahippocampal areas have been related to both physiological and pathological processes. The cellular basis and effects of normal and abnormal forms of HFOs have been controversial. This lack ...

journal_title：Annals of neurology

authors： Alvarado-Rojas C,Huberfeld G,Baulac M,Clemenceau S,Charpier S,Miles R,de la Prida LM,Le Van Quyen M

更新日期：2015-02-01 00:00:00

abstract：:Chronic progressive myelopathy (CPM) is a difficult clinical problem. Many patients who present with CPM turn out to have a spinal form of multiple sclerosis (MS), but until there is clear lesion dissemination, a definite clinical diagnosis cannot be made. We have looked for MS-related abnormalities in 72 patients wit...

journal_title：Annals of neurology

authors： Paty DW,Blume WT,Brown WF,Jaatoul N,Kertesz A,McInnis W

更新日期：1979-11-01 00:00:00

abstract：:Based on earlier findings that the presence of word comprehension impairment (a deficit in the meaning of words, or lexical semantics) in acute stroke was strongly associated with the presence of hypoperfusion or infarct in Wernicke's area, we tested the hypothesis that the severity of word comprehension impairment wa...

journal_title：Annals of neurology

authors： Hillis AE,Wityk RJ,Tuffiash E,Beauchamp NJ,Jacobs MA,Barker PB,Selnes OA

更新日期：2001-11-01 00:00:00

abstract：:We report a neurological disease among Cree Indian children in a northern Quebec village. The disease manifests as severe mental retardation, cerebral atrophy with white matter changes and calcifications, and systemic immunological abnormalities. Eleven cases are known in five families. The familial incidence of cases...

journal_title：Annals of neurology

authors： Black DN,Watters GV,Andermann E,Dumont C,Kabay ME,Kaplan P,Meagher-Villemure K,Michaud J,O'Gorman G,Reece E

更新日期：1988-10-01 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：:Imaging of cerebrovascular beta-amyloid (cerebral amyloid angiopathy) is complicated by the nearly universal overlap of this pathology with Alzheimer's pathology. We performed positron emission tomographic imaging with Pittsburgh Compound B on 42-year-old man with early manifestations of Iowa-type hereditary cerebral ...

journal_title：Annals of neurology

authors： Greenberg SM,Grabowski T,Gurol ME,Skehan ME,Nandigam RN,Becker JA,Garcia-Alloza M,Prada C,Frosch MP,Rosand J,Viswanathan A,Smith EE,Johnson KA

更新日期：2008-11-01 00:00:00

abstract：:Benign familial neonatal convulsions (BFNC) is a rare dominantly inherited epileptic syndrome characterized by frequent brief seizures within the first days of life. The disease is caused by mutations in one of two recently identified voltage-gated potassium channel genes, KCNQ2 or KCNQ3. Here, we describe a four-gene...

journal_title：Annals of neurology

authors： Lerche H,Biervert C,Alekov AK,Schleithoff L,Lindner M,Klinger W,Bretschneider F,Mitrovic N,Jurkat-Rott K,Bode H,Lehmann-Horn F,Steinlein OK

更新日期：1999-09-01 00:00:00

abstract：:The accuracy of a variety of finger and color confrontation tests in identifying chiasmal and optic nerve visual field defects was assessed in patients whose field defects had been established beforehand by a conventional achromatic kinetic technique on the Goldmann perimeter. Kinetic and static finger confrontation m...

journal_title：Annals of neurology

authors： Trobe JD,Acosta PC,Krischer JP,Trick GL

更新日期：1981-07-01 00:00:00

abstract：:To investigate the potential role of drug therapy in preventing or exacerbating seizure-related brain injury in the prepubescent brain, we administered kainic acid to rats at postnatal day 35. Therapy with daily phenobarbital was started directly before or 1 day after kainic acid was administered, and was continued th...

journal_title：Annals of neurology

authors： Mikati MA,Holmes GL,Chronopoulos A,Hyde P,Thurber S,Gatt A,Liu Z,Werner S,Stafstrom CE

更新日期：1994-09-01 00:00:00

abstract：:Electroencephalographic (EEG) and evoked potential data were recorded during behavioral testing from 8 dyslexic and 10 normal boys aged 9 to 11 years. Topographic mapping of their brain electrical activity revealed four discrete regions of difference between the two groups involving both hemispheres, left more than ri...

journal_title：Annals of neurology

authors： Duffy FH,Denckla MB,Bartels PH,Sandini G

更新日期：1980-05-01 00:00:00

abstract：:A patient with generalized convulsions noted that seizures were reliably precipitated by mental arithmetic. The interictal electroencephalogram revealed only a mild, diffuse, nonspecific disturbance, but bursts of generalized epileptiform activity with no obvious clinical expression accompanied efforts at mental arith...

journal_title：Annals of neurology

authors： Wilkins AJ,Zifkin B,Andermann F,McGovern E

更新日期：1982-06-01 00:00:00

abstract：:In Machado-Joseph disease (MJD) gene, there is a C/G polymorphism immediately after the CAG repeat; the expanded CAG repeat tract is exclusively followed by C, whereas about half of wild-type alleles are followed by G. Using this C/G polymorphism, we have engineered the small interfering RNA (siRNA) which decreased th...

journal_title：Annals of neurology

authors： Li Y,Yokota T,Matsumura R,Taira K,Mizusawa H

更新日期：2004-07-01 00:00:00

abstract：:Ten patients with sporadic olivopontocerebellar atrophy and autonomic failure were studied with positron emission tomography. Subjects underwent both an [11C]diprenorphine and an [18F]fluorodopa scan. The mean caudate-occipital uptake ratio for [11C]diprenorphine was significantly reduced to 88% and the putamen-occipi...

journal_title：Annals of neurology

authors： Rinne JO,Burn DJ,Mathias CJ,Quinn NP,Marsden CD,Brooks DJ

更新日期：1995-05-01 00:00:00

abstract：:The neurodiagnostic criteria of Leigh syndrome have not yet been clearly redefined based on the expanding of molecular etiologies. We aimed to analyze 20 years of clinical, genetic, and magnetic resonance studies from our Leigh syndrome cohort to provide a detailed description of central nervous system lesions in Leig...

journal_title：Annals of neurology

authors： Alves CAPF,Teixeira SR,Martin-Saavedra JS,Guimarães Gonçalves F,Lo Russo F,Muraresku C,McCormick EM,Falk MJ,Zolkipli-Cunningham Z,Ganetzky R,Vossough A,Goldstein A,Zuccoli G

更新日期：2020-08-01 00:00:00

abstract：OBJECTIVE:Accumulation of mitochondrial DNA (mtDNA) damage has been associated with aging and abnormal oxidative metabolism. We hypothesized that in human immunodeficiency virus-associated sensory neuropathy (HIV-SN), damaged mtDNA accumulates in distal nerve segments, and that a spatial pattern of mitochondrial dysfun...

journal_title：Annals of neurology

authors： Lehmann HC,Chen W,Borzan J,Mankowski JL,Höke A

更新日期：2011-01-01 00:00:00

abstract：:To evaluate reports of abnormal levels of free amino acids (AA) in patients with amyotrophic lateral sclerosis (ALS), we studied serum, cerebrospinal fluid, and urine AA in 12 patients with ALS and 12 controls matched for age, sex, and severity of disability. ALS patients had statistically significant elevations in se...

journal_title：Annals of neurology

authors： Patten BM,Harati Y,Acosta L,Jung SS,Felmus MT

更新日期：1978-04-01 00:00:00

abstract：OBJECTIVE:Although electrophysiologic dysfunction of the subthalamic nucleus is putative, deep brain stimulation of this structure has recently been reported to improve obsessions and compulsions. In Parkinson disease, sensorimotor subthalamic neurons display high-frequency burst firing, which is considered as an elect...

journal_title：Annals of neurology

authors： Piallat B,Polosan M,Fraix V,Goetz L,David O,Fenoy A,Torres N,Quesada JL,Seigneuret E,Pollak P,Krack P,Bougerol T,Benabid AL,Chabardès S

更新日期：2011-05-01 00:00:00

abstract：:A 53-year-old man was diagnosed 8 years earlier with globoid cell leukodystrophy (GLD, Krabbe disease) by his severe deficiency of galactocerebrosidase (GALC) activity. He was found to have eight nucleotide changes on the two copies of his GALC gene, including two in the leader sequence, four considered polymorphisms,...

journal_title：Annals of neurology

authors： Luzi P,Rafi MA,Wenger DA

更新日期：1996-07-01 00:00:00

abstract：OBJECTIVE:The discovery of a posture-dependent effect on the difference between intraocular pressure (IOP) and intracranial pressure (ICP) at the level of lamina cribrosa could have important implications for understanding glaucoma and idiopathic intracranial hypertension and could help explain visual impairments in as...

journal_title：Annals of neurology

authors： Eklund A,Jóhannesson G,Johansson E,Holmlund P,Qvarlander S,Ambarki K,Wåhlin A,Koskinen LO,Malm J

更新日期：2016-08-01 00:00:00

abstract：OBJECTIVE:Intracellular amyloid β-protein (Aβ) contributes to neurodegeneration in Alzheimer disease (AD). Apomorphine (APO) is a dopamine receptor agonist for Parkinson disease and also protects against oxidative stress. Efficacy of APO for an AD mouse model and effects of APO on cell cultures are studied. METHODS:Th...

journal_title：Annals of neurology

authors： Himeno E,Ohyagi Y,Ma L,Nakamura N,Miyoshi K,Sakae N,Motomura K,Soejima N,Yamasaki R,Hashimoto T,Tabira T,LaFerla FM,Kira J

更新日期：2011-02-01 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：:Vasospasm may be one of the causes of amaurosis fugax. A patient is reported who daily experienced multiple brief episodes of amaurosis fugax. The absence of physical, laboratory, or radiographic evidence for thromboembolism, hemodynamic compromise, or vasculitis, suggested that the amaurosis might be caused by vasosp...

journal_title：Annals of neurology

authors： Winterkorn JM,Teman AJ

更新日期：1991-09-01 00:00:00

abstract：:Juvenile myoclonic epilepsy is a common type of idiopathic generalized epilepsy characterized by myoclonic, generalized tonic-clonic, and in 30% of patients, absence seizures. We studied a three-generation pedigree of 33 members, 10 of whom were clinically affected with juvenile myoclonic epilepsy or presented with su...

journal_title：Annals of neurology

authors： Serratosa JM,Delgado-Escueta AV,Medina MT,Zhang Q,Iranmanesh R,Sparkes RS

更新日期：1996-02-01 00:00:00

abstract：:Nerve fibers containing neuropeptide Y (NPY), vasoactive intestinal polypeptide (VIP), substance P (SP), and calcitonin gene-related peptide (CGRP) were seen in the adventitia or at the adventitia-media border of the human temporal artery. Pharmacological experiments on isolated temporal artery segments revealed that ...

journal_title：Annals of neurology

authors： Jansen I,Uddman R,Hocherman M,Ekman R,Jensen K,Olesen J,Stiernholm P,Edvinsson L

更新日期：1986-10-01 00:00:00

abstract：OBJECTIVE:To examine the association between odor identification deficits and future mortality in a multiethnic community cohort of older adults. METHODS:Participants were evaluated with the 40-item University of Pennsylvania Smell Identification Test (UPSIT). Follow-up occurred at 2-year intervals with information on...

journal_title：Annals of neurology

authors： Devanand DP,Lee S,Manly J,Andrews H,Schupf N,Masurkar A,Stern Y,Mayeux R,Doty RL

更新日期：2015-09-01 00:00:00

abstract：:A 37-year-old homosexual man with the acquired immune deficiency syndrome (AIDS) developed progressive, ultimately fatal, neurological deficits 12 weeks after a course of cutaneous zoster. Premortem radiological procedures and cerebrospinal fluid analyses were nondiagnostic. At postmortem examination, several opportun...

journal_title：Annals of neurology

authors： Ryder JW,Croen K,Kleinschmidt-DeMasters BK,Ostrove JM,Straus SE,Cohn DL

更新日期：1986-02-01 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：:Because the symptomatic treatments for multiple sclerosis (MS) are limited, new approaches have been sought. Anatomical studies of MS lesions show a relative preservation of axons, and clinical studies suggest that some of the neurological impairment in patients with MS is physiological. Electrophysiological studies s...

journal_title：Annals of neurology

authors： Bever CT Jr

更新日期：1994-01-01 00:00:00

abstract：:Epilepsy is a disease of recurrent seizures that can develop after a wide range of brain insults. Although surgical resection of focal regions of seizure onset can result in clinical improvement, the molecular mechanisms that produce and maintain focal hyperexcitability are not understood. Here, we demonstrate a regio...

journal_title：Annals of neurology

authors： Rakhade SN,Yao B,Ahmed S,Asano E,Beaumont TL,Shah AK,Draghici S,Krauss R,Chugani HT,Sood S,Loeb JA

更新日期：2005-11-01 00:00:00