Upregulation of Fas/Fas ligand in inclusion body myositis.

abstract：:Intellectual disability is common and is associated with significant morbidity. Until the latter half of the 20th century, there were no efficacious treatments. Following initial breakthroughs associated with newborn screening and metabolic corrections, little progress was made until recently. With improved understand...

journal_title：Annals of neurology

authors： Picker JD,Walsh CA

更新日期：2013-09-01 00:00:00

abstract：:Patterns of cerebral metabolic intercorrelations were compared in the resting state in 15 healthy young men (ages 20 to 32 years) and 15 healthy elderly men (ages 64 to 83 years). Controlling for whole-brain glucose metabolism, partial correlation coefficients were determined between pairs of regional cerebral metabol...

journal_title：Annals of neurology

authors： Horwitz B,Duara R,Rapoport SI

更新日期：1986-01-01 00:00:00

abstract：:Amyloid plaques in Alzheimer's disease contain beta-amyloid, encoded by portions of exons 16 and 17 of the amyloid precursor protein. The specific association of rare amyloid precursor protein mutations with some kindreds with early-onset familial Alzheimer's disease suggests that specific abnormalities in amyloid pre...

journal_title：Annals of neurology

authors： Peacock ML,Murman DL,Sima AA,Warren JT Jr,Roses AD,Fink JK

更新日期：1994-04-01 00:00:00

abstract：:Antibodies to Ma1 and Ma2 proteins identify a paraneoplastic disorder that affects the limbic system, brain stem, and cerebellum. Preliminary studies suggested the existence of other Ma proteins and different patterns of immune response associated with distinct neurologic symptoms and cancers. In this study, our aim w...

journal_title：Annals of neurology

authors： Rosenfeld MR,Eichen JG,Wade DF,Posner JB,Dalmau J

更新日期：2001-09-01 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：: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:To understand how monosynaptic inputs onto adult-born dentate granule cells (DGCs) are altered in experimental mesial temporal lobe epilepsy (mTLE) and whether their integration differs from early-born DGCs that are mature at the time of epileptogenesis. METHODS:A dual-virus tracing strategy combining retrov...

journal_title：Annals of neurology

authors： Du X,Zhang H,Parent JM

更新日期：2017-06-01 00:00:00

abstract：:Multiple sclerosis (MS) has been associated with HLA-DR2 for more than 20 years, and a large number of studies have addressed the relation between MS and the HLA class II genes, which are our major immune-response genes. This has produced a complex and confusing picture that is difficult to interpret. With the advent ...

journal_title：Annals of neurology

authors： Hillert J

更新日期：1994-01-01 00:00:00

abstract：:A 53-year-old man presented with a lumbosacral polyradiculoneuropathy and developed fluctuating encephalopathy suggestive of multifocal small vessel disease. Postmortem examination demonstrated multifocal vascular occlusion by undifferentiated cells confined to the intravascular space. Extravascular spread was found o...

journal_title：Annals of neurology

authors： Krieger C,Robitaille Y,Jothy S,Elleker G

更新日期：1982-11-01 00:00:00

abstract：:In a double-blinded, placebo-controlled, crossover study in seven mitochondrial myopathy patients (MM), we investigated whether lowering of lactate with dichloroacetate (DCA) can improve exercise tolerance and oxidative capacity in MM. DCA lowered plasma lactate at rest and during exercise (from 10.5 +/- 2.0 to 5.0 +/...

journal_title：Annals of neurology

authors： Vissing J,Gansted U,Quistorff B

更新日期：2001-05-01 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：:We describe a French amyotrophic lateral sclerosis (ALS) family with two distinct mutations in the Cu/Zn superoxide dismutase (SOD1) gene. The D90A mutation has been well described and clearly shown to cause recessive ALS. In this family, affected individuals are heterozygous for the D90A mutation and also carry a sin...

journal_title：Annals of neurology

authors： Hand CK,Mayeux-Portas V,Khoris J,Briolotti V,Clavelou P,Camu W,Rouleau GA

更新日期：2001-02-01 00:00:00

abstract：:The rate of local cerebral glucose utilization was determined for quantification of energy metabolism in macrostructures of the sensorimotor system during propagation of focal motor seizures in 24-month-old monkeys. The rate was measured in 4 control animals and in 4 monkeys each with seizures limited to the contralat...

journal_title：Annals of neurology

authors： Caveness WF,Kato M,Malamut BL,Hosokawa S,Wakisaka S,O'Neill RR

更新日期：1980-03-01 00:00:00

abstract：:Copper toxicity contributes to neuronal death in Wilson's disease and has been speculatively linked to the pathogenesis of Alzheimer's and prion diseases. We examined copper-induced neuronal death with the goal of developing neuroprotective strategies. Copper catalyzed an increase in hydroxyl radical generation in sol...

journal_title：Annals of neurology

authors： Sheline CT,Choi EH,Kim-Han JS,Dugan LL,Choi DW

更新日期：2002-08-01 00:00:00

abstract：:Tropical spastic paraparesis (TSP) is a common myeloneuropathy with primary and predominant involvement of the pyramidal tract and minimal sensory loss. The epidemic form of TSP is related to toxic nutritional factors, but the endemic form occurs in clusters in tropical areas, especially in India, Africa, the Seychell...

journal_title：Annals of neurology

authors： Vernant JC,Maurs L,Gessain A,Barin F,Gout O,Delaporte JM,Sanhadji K,Buisson G,de-Thé G

更新日期：1987-02-01 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 describe a 33-year-old homosexual man with a steroid-responsive, remitting and relapsing leukoencephalopathy associated with recent human immunodeficiency virus type 1 (HIV-1) seroconversion. Biopsy of a parieto-occipital lesion revealed demyelination and astrogliosis with focal necrosis. Detailed investigations de...

journal_title：Annals of neurology

authors： Berger JR,Tornatore C,Major EO,Bruce J,Shapshak P,Yoshioka M,Houff S,Sheremata W,Horton GF,Landy H

更新日期：1992-01-01 00:00:00

abstract：:Mutations in AbetaPP cause deposition of Abeta amyloid fibrils in brain parenchyma and cerebral vessels, resulting in Alzheimer's disease (AD) and/or cerebral amyloid angiopathy (CAA). We report a novel mutation (L705V) within the Abeta sequence of AbetaPP in a family with autosomal dominant, recurrent intracerebral h...

journal_title：Annals of neurology

authors： Obici L,Demarchi A,de Rosa G,Bellotti V,Marciano S,Donadei S,Arbustini E,Palladini G,Diegoli M,Genovese E,Ferrari G,Coverlizza S,Merlini G

更新日期：2005-10-01 00:00:00

abstract：OBJECTIVE:Ischemic injury of axons is a feature of periventricular leukomalacia, a pathological correlate of cerebral palsy. Recent evidence suggests that axons are damaged before they receive the first layer of compact myelin. Here we examine the cellular mechanisms underlying ischemic-type injury of premyelinated cen...

journal_title：Annals of neurology

authors： Alix JJ,Fern R

更新日期：2009-11-01 00:00:00

abstract：OBJECTIVE:To evaluate the ability of mesenchymal stem cells (MSCs), a subset of adult stem cells from bone marrow, to cure experimental autoimmune encephalomyelitis. METHODS:The outcome of the injection of MSCs, in mice immunized with the peptide 139-151 of the proteolipid protein (PLP), was studied analyzing clinical...

journal_title：Annals of neurology

authors： Gerdoni E,Gallo B,Casazza S,Musio S,Bonanni I,Pedemonte E,Mantegazza R,Frassoni F,Mancardi G,Pedotti R,Uccelli A

更新日期：2007-03-01 00:00:00

abstract：OBJECTIVE:This study was untaken to investigate the association of micro brain infarcts (MBIs) with antemortem global cognitive function (CF), and whether brain weight (BW) and Alzheimer lesions (neurofibrillary tangles [NFTs] or neuritic plaques [NPs]) mediate the association. METHODS:Subjects were 436 well-character...

journal_title：Annals of neurology

authors： Launer LJ,Hughes TM,White LR

更新日期：2011-11-01 00:00:00

abstract：:We report FK506-induced neurotoxicity in 14 of 44 consecutive patients following orthoptic liver transplantation. In 10 of these 14 patients, postural hand tremors were found in the first weeks following surgery, transient apraxia of speech in 3, and generalized tonic-clonic seizures were noted in 2 patients. Other ma...

journal_title：Annals of neurology

authors： Wijdicks EF,Wiesner RH,Dahlke LJ,Krom RA

更新日期：1994-04-01 00:00:00

abstract：:To study the distribution of myelin-associated glycoprotein (MAG) in human nervous tissue and in multiple sclerosis (MS) lesions, we used paraffin sections and our modification of the peroxidase-antiperoxidase technique. Sections of MS lesions also were treated with antiserum to basic protein (BP) and with histologica...

journal_title：Annals of neurology

authors： Itoyama Y,Sternberger NH,Webster HD,Quarles RH,Cohen SR,Richardson EP Jr

更新日期：1980-02-01 00:00:00

abstract：:Extramedullary hematopoiesis (EMH) in the spinal epidural space is a rare but treatable cause of progressive paraparesis in patients with a variety of hematological and systemic disorders. We report a case of extensive thoracic epidural EMH causing progressive spastic paraparesis in a patient with agnogenic myeloid me...

journal_title：Annals of neurology

authors： Stahl SM,Ellinger G,Baringer JR

更新日期：1979-05-01 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：:In diabetic nerves, the activation of the polyol pathway and a resulting decrease in Na(+)-K(+) ATPase activity lead to intra-axonal Na(+) accumulation and a smaller Na(+) gradient across the axolemma than normal. To investigate whether glycemic control is associated with acutely reversible changes in axonal excitabil...

journal_title：Annals of neurology

authors： Kitano Y,Kuwabara S,Misawa S,Ogawara K,Kanai K,Kikkawa Y,Yagui K,Hattori T

更新日期：2004-10-01 00:00:00

abstract：:We previously reported that the monoclonal antibody Alz-50 recognizes a protein (A68) with an apparent molecular weight of 68,000 daltons in the brains of patients who died with Alzheimer's disease, but not in brain tissue from individuals free of neurological disease. We now report that substantial quantities of this...

journal_title：Annals of neurology

authors： Wolozin B,Davies P

更新日期：1987-10-01 00:00:00

abstract：:The nucleus basalis of Meynert, which supplies diffuse cholinergic fibers to the cerebral neocortex, was investigated in two cases of parkinsonism-dementia complex of Guam (PDG). The nucleus basalis of the two PDG patients showed extensive neuron loss when compared with age-matched non-Guamanian controls, suggesting t...

journal_title：Annals of neurology

authors： Nakano I,Hirano A

更新日期：1983-01-01 00:00:00

abstract：OBJECTIVE:We examined the association of nutrient intake with microstructural white matter integrity, and the role of white matter integrity in the association between nutrient consumption and cognition. METHODS:This cross-sectional analysis included 239 elderly (age ≥ 65 years) participants of a multiethnic cohort. W...

journal_title：Annals of neurology

authors： Gu Y,Vorburger RS,Gazes Y,Habeck CG,Stern Y,Luchsinger JA,Manly JJ,Schupf N,Mayeux R,Brickman AM

更新日期：2016-06-01 00:00:00

abstract：OBJECTIVE:To assess the ability for visual search and recognition of roadside targets and safety errors during a landmark and traffic sign identification task in drivers with Parkinson's disease (PD). METHODS:Seventy-nine drivers with PD and 151 neurologically normal older adults underwent a battery of visual, cogniti...

journal_title：Annals of neurology

authors： Uc EY,Rizzo M,Anderson SW,Sparks J,Rodnitzky RL,Dawson JD

更新日期：2006-10-01 00:00:00