Motor neuron syndrome and monoclonal IgM with antibody activity against gangliosides GM1 and GD1b.

abstract：:Neuronal injury resulting from glutamate receptor-mediated excitotoxicity has been implicated in a wide spectrum of neurological disease states, including ischemia, central nervous system trauma, and some types of neurodegenerative diseases. Excitotoxicity may interact with other pathophysiological processes to enhanc...

journal_title：Annals of neurology

authors： Dugan LL,Choi DW

更新日期：1994-01-01 00:00:00

abstract：:Eighty-three patients with epidural spinal cord compression, from metastatic cancer were treated with high-dose adrenocorticosteroids and a new radiation fractionation protocol. Only those patients were included who had complete or almost complete block on myelography and who had not received prior radiation therapy t...

journal_title：Annals of neurology

authors： Greenberg HS,Kim JH,Posner JB

更新日期：1980-10-01 00:00:00

abstract：:Recently, the mutation causing early-onset generalized torsion dystonia has been identified as a GAG deletion in the gene for an adenosine triphosphate-binding protein named torsinA. We describe a German family with 5 clinically affected individuals carrying this mutation. In at least 4 of the 5 patients, the disease ...

journal_title：Annals of neurology

authors： Gasser T,Windgassen K,Bereznai B,Kabus C,Ludolph AC

更新日期：1998-07-01 00:00:00

abstract：OBJECTIVE:A study was undertaken to describe the clinical spectrum, voltage-gated potassium channel (VGKC) complex antibody specificities, and central nervous system localization of antibody binding in 29 patients diagnosed with Morvan syndrome (MoS). METHODS:Clinical data were collected using questionnaires. Radioimm...

journal_title：Annals of neurology

authors： Irani SR,Pettingill P,Kleopa KA,Schiza N,Waters P,Mazia C,Zuliani L,Watanabe O,Lang B,Buckley C,Vincent A

更新日期：2012-08-01 00:00:00

abstract：:Multiple sclerosis (MS) is a demyelinating disease of the central nervous system (CNS) afflicting approximately 250,000 individuals in the United States. This inflammatory disease has variable clinical manifestations, ranging from a relapsing-remitting course to a chronic progressive disease. Approximately one third o...

journal_title：Annals of neurology

authors： Bansil S,Cook SD,Rohowsky-Kochan C

更新日期：1995-05-01 00:00:00

abstract：:This study reports a case of type VII mucopolysaccharidosis (beta-glucuronidase deficiency) presenting as lethal hydrops fetalis. Skin fibroblast cultures established postmortem revealed deficient beta-glucuronidase activity. Mucopolysaccharides were stored in various cells of the brain, heart, kidney, liver, and sple...

journal_title：Annals of neurology

authors： Irani D,Kim HS,El-Hibri H,Dutton RV,Beaudet A,Armstrong D

更新日期：1983-10-01 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：OBJECTIVE:To identify brain regions, cell types, or both that generate abnormal electrical discharge in tuberous sclerosis complex (TSC). Here we examined excitatory and inhibitory synaptic currents in human tissue samples obtained from a TSC patient with no discernible cortical tubers and acute neocortical brain slice...

journal_title：Annals of neurology

authors： Wang Y,Greenwood JS,Calcagnotto ME,Kirsch HE,Barbaro NM,Baraban SC

更新日期：2007-02-01 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: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：OBJECTIVES:African Americans are at greater risk for developing Alzheimer's disease (AD) dementia than non-Hispanic whites. In addition to biological considerations (eg, genetic influences and comorbid disorders), social and environmental factors may increase the risk of AD dementia. This paper (1) assesses neuroimagin...

journal_title：Annals of neurology

authors： Meeker KL,Wisch JK,Hudson D,Coble D,Xiong C,Babulal GM,Gordon BA,Schindler SE,Cruchaga C,Flores S,Dincer A,Benzinger TL,Morris JC,Ances BM

更新日期：2021-02-01 00:00:00

abstract：:In 9 patients with progressive supranuclear palsy and in 27 controls, dopamine and homovanillic acid concentrations, choline acetyltransferase (CAT) activity, and the number of [3H]spiperone and [3H]quinuclidinyl benzilate binding sites were measured post mortem in the striatum (caudate nucleus, putamen, and nucleus a...

journal_title：Annals of neurology

authors： Ruberg M,Javoy-Agid F,Hirsch E,Scatton B,LHeureux R,Hauw JJ,Duyckaerts C,Gray F,Morel-Maroger A,Rascol A

更新日期：1985-11-01 00:00:00

abstract：:The oculocerebrorenal syndrome (OCRS), Lowe's syndrome, is an X-linked, recessive disease characterized by mental retardation, congenital corneal abnormalities and cataracts, growth failure, rickets, osseous abnormalities, renal dysfunction with periodic acidosis, hypotonia, and areflexia. Ultrastructural studies of s...

journal_title：Annals of neurology

authors： Wisniewski KE,Kieras FJ,French JH,Houck GE Jr,Ramos PL

更新日期：1984-07-01 00:00:00

abstract：:A hereditary ceruloplasmin deficiency associated with severe iron deposition in visceral organ and brain tissues found on histopathological examination at autopsy is discussed. Three siblings of consanguineous Japanese parents were studied. Their clinical symptoms were progressive dementia, extrapyramidal disorders, c...

journal_title：Annals of neurology

authors： Morita H,Ikeda S,Yamamoto K,Morita S,Yoshida K,Nomoto S,Kato M,Yanagisawa N

更新日期：1995-05-01 00:00:00

abstract：:A young man presented with apparent transient ischemic attacks following a motorcycle accident in which he sustained minor injuries only. Computerized axial tomography demonstrated a small right frontal infarct, and angiography revealed an aneurysm of the right internal carotid artery in its extracranial course. This ...

journal_title：Annals of neurology

authors： Bradbury PG,Lambert CD

更新日期：1978-07-01 00:00:00

abstract：:A 26-year-old woman was in spontaneous clinical remission from myasthenia gravis (MG) for six months, yet gave birth to a full-term infant with typical neonatal MG. It is believed that transplacental transfer of anti-acetylcholine (ACh) receptor antibodies is responsible for neonatal MG; therefore, neonatal MG represe...

journal_title：Annals of neurology

authors： Elias SB,Butler I,Appel SH

更新日期：1979-07-01 00:00:00

abstract：:Multiple sclerosis (MS) is the most common idiopathic inflammatory disease of the central nervous system. The distinction between MS and other benign or fulminant inflammatory demyelinating disorders is based on quantitative, rather than qualitative, differences in chronicity and severity. Primary progressive MS may d...

journal_title：Annals of neurology

authors： Weinshenker BG

更新日期：1994-01-01 00:00:00

abstract：OBJECTIVE:Recent studies have identified a subset of outgrowth cell population with endothelial phenotype in long-term cultures of peripheral blood mononuclear cells. The concept that peripheral blood-derived cells participate in neuronal regeneration remains highly controversial, and no specific cell type has been ide...

journal_title：Annals of neurology

authors： Jung KH,Chu K,Lee ST,Song EC,Sinn DI,Kim JM,Kim SJ,Kim JH,Kang KM,Park HK,Lee SH,Kim M,Lee SK,Roh JK

更新日期：2008-03-01 00:00:00

abstract：OBJECTIVE:The Veterans Administration Cooperative Studies Program #468, a multicenter study that randomized Parkinson's disease (PD) patients to either subthalamic nucleus (STN) or globus pallidus internus (GPi) deep brain stimulation (DBS), found that stimulation at either target provided similar overall motoric benef...

journal_title：Annals of neurology

authors： Katz M,Luciano MS,Carlson K,Luo P,Marks WJ Jr,Larson PS,Starr PA,Follett KA,Weaver FM,Stern MB,Reda DJ,Ostrem JL,CSP 468 study group.

更新日期：2015-04-01 00:00:00

abstract：:A unique phenotype of Waardenburg-Hirschsprung disease (WS4) accompanied by peripheral neuropathy and central dysmyelination has been recognized recently in association with SOX10 mutations. We report an infant boy with lethal congenital hypomyelinating neuropathy and WS4 who had a heterozygous SOX10 mutation (Q250X)....

journal_title：Annals of neurology

authors： Inoue K,Shilo K,Boerkoel CF,Crowe C,Sawady J,Lupski JR,Agamanolis DP

更新日期：2002-12-01 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：OBJECTIVE:The aim of the study was to assess the distribution, frequency, and specific location of mutant huntingtin protein (mHTT) aggregates-the pathological hallmark of Huntington disease (HD)-within the various compartments of the spinal cord and their potential impact on the local vasculature and blood-spinal cord...

journal_title：Annals of neurology

authors： Sciacca G,Cicchetti F

更新日期：2017-12-01 00:00:00

abstract：:A young man developed pathological thirst and hyperdipsia, hyperphagia, disordered temperature regulation, a lowered threshold for aggressive behavior, apathy, impaired memory, and seizures following encephalitis. He had marked hyponatremia. Bouts of water drinking produced water intoxication and precipitated status e...

journal_title：Annals of neurology

authors： Howe JG,Penney MD,Currie S,Morgan D

更新日期：1983-02-01 00:00:00

abstract：OBJECTIVE:Newer magnetic resonance imaging (MRI) techniques have shown promise in capturing early Parkinson disease (PD)-related changes in the substantia nigra pars compacta (SNc), the key pathological loci. Their translational value, however, is hindered by technical complexity and inconsistent results. METHODS:A no...

journal_title：Annals of neurology

authors： Du G,Lewis MM,Sica C,Kong L,Huang X

更新日期：2019-01-01 00:00:00

abstract：OBJECTIVE:The aim of the study was to evaluate whether the visual analysis of muscle magnetic resonance imaging scans can identify specific patterns of muscle involvement. METHODS:We assessed scans from 83 patients with muscle disorders characterized by rigidity of the spine secondary to mutations in 4 different genes...

journal_title：Annals of neurology

authors： Mercuri E,Clements E,Offiah A,Pichiecchio A,Vasco G,Bianco F,Berardinelli A,Manzur A,Pane M,Messina S,Gualandi F,Ricci E,Rutherford M,Muntoni F

更新日期：2010-02-01 00:00:00

abstract：:Using two-color flow cytometric analysis, we studied peripheral blood lymphocyte subsets in 15 patients with human T-cell lymphotropic virus type I-associated myelopathy. The percentage of CD4+ 4B4+ cells (helper inducer T cell) was significantly increased in the patients with the myelopathy, compared with 16 healthy ...

journal_title：Annals of neurology

authors： Itoyama Y,Kira J,Fujii N,Goto I,Yamamoto N

更新日期：1989-08-01 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：:We sought to determine whether the putative pontine micturition center in the human dorsal pons contains corticotrophin-releasing factor (CRF) neurons, and whether these neurons are depleted in patients with multiple system atrophy and bladder dysfunction. Brains were obtained at autopsy from 4 control subjects and 4 ...

journal_title：Annals of neurology

authors： Benarroch EE,Schmeichel AM

更新日期：2001-11-01 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：:Paraneoplastic chorea is described in 16 patients: 11 with limited small-cell carcinoma, 2 with lung cancer revealed by imaging, 1 with renal cell carcinoma, and 1 with lymphoma. All had CRMP-5-IgG; 6 also had ANNA-1 (anti-Hu), including 1 without evident cancer. Chorea was the initial and most prominent symptom in 11...

journal_title：Annals of neurology

authors： Vernino S,Tuite P,Adler CH,Meschia JF,Boeve BF,Boasberg P,Parisi JE,Lennon VA

更新日期：2002-05-01 00:00:00